Pheochromocytoma - Knowledge

965:. In the UK eligibility for NHS funded genetic testing is determined by criteria set by NHS England Genomics service. The criteria in 2023 included all patients with paraganglioma and all patients with unilateral pheochromocytoma aged under 60. The most recent data indicates that there are 25 pheochromocytoma susceptibility genes; however, just 12 are recognized as part of a well-known syndrome. Determining the genetic status of a pheochromocytoma patient is crucial – each gene is inherited in a different pattern, associated with specific disease characteristics, and may respond more favorably to certain treatment options. Furthermore, early identification can guide physicians on screening recommendations for first degree relatives of patients with pheochromocytoma. There is no current consensus for how and when 260: 3374: 2163:, and treatment planning – they are not used to discern tumor location or help the surgical team prepare for excision. For most pheochromocytoma patients, functional imaging will follow a CT or MR. If anatomic imaging only demonstrates an adrenal tumor without evidence of disease anywhere else in the body and the metanephrine levels are overtly elevated, functional imaging can be foregone in favor of prompt surgical excision. Over the last decade, there have been five functional techniques used to evaluate the pheochromocytoma patient (1) 18F- 2717: 3423: 2104:(MR) scans. These imaging modalities serve to initially locate the tumor and provide detailed information about size, morphology, and structural relation to adjacent internal structures. Traditionally, a patient presents to their physician for symptoms concerning for a pheochromocytoma, which prompts a biochemical evaluation. If the results are positive, the patient is referred for anatomic imaging with a CT or MR scan. However, as anatomic imaging becomes more readily available, patients are referred to an 3035: 1987: 2312:(SDHx) mutations. While FDOPA inconsistently detected metastatic disease, Ga-DOTA analogues have demonstrated superior localization of metastatic pheochromocytoma. When directly compared in one head-to-head study in 2019, Ga-DOTA analogues outperformed FDOPA, particularly in the detection of metastatic bone lesions. An additional benefit of the DOTA analogues is the ability for treatment with peptide receptor radionuclide therapy, which will be discussed in the treatment section below. 603: 2861:) oral supplementation may be required to ensure they do not develop adrenal insufficiency. When the body is stressed (during surgery), the adrenal glands naturally produce more steroids; however, if the glands have been removed, they are unable to do so. Therefore, "stress-dosing" steroids are required and should be started intraopertively to mimic the natural physiology of the adrenal glands. The typical regimen when post-operative adrenal insufficiency is thought to be likely: 1936: 615: 2187: 526:(fast heart rate), sweating, and headaches. Some PGLs may secrete little to no catecholamines, or only secrete paroxysmally (episodically), and other than secretions, PGLs can still become clinically relevant through other secretions or mass effect (most common with head and neck PGL). PGLs of the head and neck are typically parasympathetic and their sympathetic counterparts are predominantly located in the abdomen and pelvis, particularly concentrated at the 1899:, and generalized weakness) limit compliance and have vastly diminished prescriptions. While the adverse side-effects with clonidine are inconvenient, the most dangerous aspect of clonidine is withdrawal rebound hypertension – that is, when the medicine is abruptly discontinued, blood pressure may rapidly return or surpass the original value. However, a one-time, weight-based dose can be utilized in limited settings to help determine disease status. After 3213: 1871:. In comparison to the specificity of elevated catecholamines in the pheochromocytoma patient, chromogranin A is a non-specific polypeptide that is high in a variety of neuroendocrine tumors. However, a 2006 report from Italy found that over 90% of studied pheochromocytoma patients demonstrated elevated chromogranin A levels. If metanephrine values are equivocal, chromogranin A can be used as an adjunct marker to predict the presence of a tumor. 2240: 120: 25: 212: 1826:. As the majority of these medications are commonly prescribed for psychiatric conditions, a conversation with the prescriber may be necessary to facilitate alternative therapeutic options while the patient is undergoing evaluation for a pheochromocytoma. After any possible prescription medications have been held, it is important to review any over-the-counter medications/supplements as well as the commonly used 2036: 829:. As with the other cardiovascular-related complications, excess catecholamines are responsible for the increased myocardial burden and significant physiologic stress. Current literature indicates that most of the catecholamine-induced damage is reversible, thereby strengthening the argument for early and accurate diagnosis in order to allow for cardiac remodeling and prevent further destruction. 3533: 66: 752:: Pheochromocytoma-related hypertensive emergencies are one of the most feared clinical manifestations. Attacks are random and may occur secondary to a trigger (see Signs and Symptoms above) or spontaneously after a catecholamine surge. The prevailing symptom is elevated systolic blood pressure (> 200 mmHg) that is unresponsive to traditional treatment regimens and threatens 969:(individual who has a genetic variant associated with pheochromocytoma, but no current evidence of disease) should be evaluated. Conversations should occur at an individual level with the patient and their provider to develop a personalized screening plan that alternates between a biochemical (blood work) evaluation and whole-body imaging to monitor disease progression. 1802:, and illicit substances can interfere with the proper collection of plasma metanephrines and lead to false-positive results. Providers should review a patient's medication list in-detail and have a discussion if temporarily discontinuing any of the interfering medications is possible. The most reported medications to result in falsely elevated metanephrines include: 2224:. Furthermore, if a patient was found to be positive on an MIBG scan, they were eligible for MIBG treatment, offering additional avenues for those with widespread metastatic disease. However, further investigation revealed that while MIBG excelled with adrenal lesions, it was far less superior in patients with extra-adrenal paragangliomas, particularly with specific 1923:. Paragangliomas of the head and neck commonly secrete dopamine, but are referred to as "biochemically silent" because they do not cause the characteristic symptoms associated with a pheochromocytoma. However, methoxytyramine can be utilized to detect the tumors of the head and neck. Further research indicates that the biomarker is also a useful indicator of 1792:(flat on their back) for 30 minutes before collection. Specific supine reference values should be used in this scenario. Ensuring these conditions is difficult and may be cost-prohibitive at most institutions. In these cases, a rested, supine draw can be repeated following a positive result in a seated position to eliminate false-positive results. 941:> 40 degrees Celsius, neurologic manifestations, and cardiovascular instability resulting in either hypo or hypertension. In contrast to a hypertensive crisis, pheochromocytoma-associated MODS may not respond to traditional alpha-receptor agents and may require emergent surgical excision if clinical stability is not achieved. 2262:(Image Left). In 2012, over 200 patients participated in a trial that compared the current gold standard of the time (MIBG/CT/MRI) to the novel FDG PET. Compared to its functional counterpart, FDG outperformed MIBG in detecting soft-tissue and bone metastases with higher specificity in patients with biochemically active tumors. 3243:(no progression) in the remaining 20 patients. Overall toxicity was low, with no high-grade haematological (blood) or kidney damage reported. At the end of that same year, a systemic review looked at all published articles (12) where metastatic pheochromocytoma patients underwent PRRT and found that treatment-related 3616:

and his team are tasked with diagnosing and treating an inmate on death row. Although the patient has a violent history of homicide, Dr. House suspects that his episodic rage and aggression may be caused by an adrenaline secreting tumor. Dr. House is able to locate the tumor and diagnoses the patient

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In the medical community, students are often taught "when you hear hoofbeats, think horses, not zebras." In other words, common diagnoses are common, so healthcare professionals should first rule out what is most expected (the horses) before diving into the rare etiologies that are far less likely to

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related disease, which has a higher chance of bilateral pheochromocytomas. The risk of leaving adrenal tissue is recurrent disease (tumor comes back). A 2019 cohort study reported that despite a 13% recurrent rate in patients who underwent a cortical-sparing adrenalectomy for pheochromocytoma, there

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As with most lab work, the patient should refrain from eating (fasting) after midnight the night prior to their collection. However, there are further recommendations specific to a metanephrines collection, including abstaining from nicotine, alcohol, and exercise for at least 12 hours prior to their

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vessels. Patients with pheochromocytoma present with myocardial infarctions despite an overall lack of plaque build-up, indicating a different mechanism for the myocardial infarction. Current research hypothesizes that the tumor secretes massive amounts of catecholamines, which directly interact with

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and discovered a six-inch oblong tumor composed of an unknown "yellow-ish coloured substance" coming from the capsula renalis (what is now known as the adrenal gland). This would become the first known clinical description of a pheochromocytoma, but as no features of the tumor itself were described,

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first demonstrated success in patients with undifferentiated neuroendocrine tumors and then trials began with metastatic pheochromocytoma patients. In 2019, Vyakaranam et al. published favourable results for their 22 patients who underwent PRRT, with partial response in 2 patients and stable disease

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for (3) hours and a repeat blood draw will be taken. A positive result (indicating a pheochromocytoma) will occur if the plasma metanephrine levels remain elevated after clonidine is given. If the results are the same or fall, the test is negative and the patient does not have a pheochromocytoma. It

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Headaches are one of the core clinical manifestations of a pheochromocytoma and can result in debilitating pain. The majority of studied patients report their pain began and ended abruptly without warning and described the pain as a severe, bilateral throbbing (although the scale of severity was not

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was met with mixed opinions from the rare disease community. The executive director of the VHL Alliance was happy with the portrayal of a VHL patient in mainstream media, but pointed out that of the four scripts she knew of with a VHL patient, three involved a pheochromocytoma, which occurs in less

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and found no difference in complications between the two groups. The following year, a group from France published a similar article with a warning against waiting an entire week to begin alpha-blockade. The French researchers called for immediate surgical intervention and consideration of steps to

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is not a preferred imaging modality and should be avoided in the pheochromocytoma patient. However, in specific patient populations where avoid ionizing radiation is the top priority (children, pregnant women), ultrasound can be used as an adjunct method when MR may be unavailable or the patient is

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In contrast to an operation for non-metastatic disease, an open procedure may be preferred over a minimally invasive technique in order to circumvent potential tumor spread. This also aids surgical visualization and offers the best opportunity to identify and remove metastatic lymph nodes. Reports

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population point to the advent of modern imaging evaluation and the ability to detect these tumors prior to death. While each of the above studies reported varying incidence and prevalence values, all have indicated that the average age at initial diagnosis is between the third to fifth decade of

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Recent literature has detailed several factors that predict accelerated progression of disease and higher mortality rates, including patients who choose to forego surgical resection of their primary tumor, larger tumors at initial presentation, older age at initial diagnosis, and a shortened time

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As was mentioned in the functional imaging section above, MIBG is not only useful in locating the presence of metastatic disease, but also as an available treatment modality. In 2019, a multi-center phase 2 trial looked at the safety and efficacy of MIBG therapy in metastatic or unresectable (not

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Borderline elevated metanephrines present a diagnostic challenge to the physician – the first step is to repeat the labs, taking extra precautions to follow the gold standard diagnosis described above, including the conditions of collection, pharmaceutical interference, and any potential diet and

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is defined as the likely outcome of a disease OR, the chance of recovery or a recurrence. This is an extremely difficult question when it comes to pheochromocytoma, and the answer depends on the patients genetic status, presence of metastatic disease, and the location of their primary tumor. An

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between patients whose tumors shrunk versus those without a response (no reduction in tumor burden via imaging), even in non-responders, patients reported feeling better, blood pressure was lower, and some patients were even able to undergo surgery following disease stabilization with CVD. When

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was 36.7 months and 92% of patients had at least a partial positive response (tumor shrinkage) or stable disease without progression within the first year of the study. Furthermore, over a fourth of the patients were able to decrease their anti-hypertensive medications and reported symptomatic

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While the pre-operative alpha and beta blockade discussed above is overwhelmingly recognized as the standard of care, particularly in the United States, there has been discussion at the international level if alpha-blockade is necessary. In 2017, a team of researchers from Germany published an

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All patients with phaeochromocytomas are currently considered to have a lifelong risk of metastases and therefore conceptually they are all considered 'malignant'. The risk of metastasis ranges from ~5 to 15%. There is no single histological finding or biomarker to reliably predict metastatic

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appears to reduce late morbidity, time to oral fluid or food intake and time to ambulation when compared to laparoscopic transperitoneal adrenalectomy, however there is uncertainty about these effects due to very low-quality evidence. For outcomes such as all-cause mortality, early morbidity,

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Following the development of FDG-PET, neuroendocrine-specific PET scans began to emerge. One of the first favorable imaging modalities was F-FDOPA, which demonstrated a high sensitivity in detecting head and neck paragangliomas as well as non-metastatic disease outside of the head and neck.

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nature of catecholamine synthesis and release, patients may experience "attacks" or "spells" where they are suddenly overwhelmed with signs and symptoms of their tumor. Attacks can occur spontaneously (without warning) or may be triggered by a variety of pharmaceutical agents (including

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for a pheochromocytoma ranged from 30 to 45%. Retrospective series have postulated that these alarmingly high death rates were due to the lack of a pre-operative blockade with alpha and beta-adrenoceptor antagonist and the need for modern anesthesia practices. From this point forward,

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Unlike many routine laboratory tests that can be drawn at a moments notice, there are several recommendations that should be followed to ensure the ideal conditions and an accurate sample. Current research indicates that blood work should only be drawn after a patient has been resting

2684:(having the patient take in plenty of fluids prior to surgery). However, after reviewing their patient data, over 60% of the same physicians failed to discuss salt-loading and adequate hydration. When the patients were stratified by age, those that were younger received the advice to 2151:

unable to complete the scan. Furthermore, if an acute adrenal hemorrhage is suspected in a pheochromocytoma patient, ultrasound is a quick, painless, radiation-less, and cheap modality for a "first-pass" before the above imaging modalities or surgery is used to confirm the diagnosis.

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reviewed 54 pheochromocytoma cases between 1928–1977 and discovered that just 24% of the patients were correctly diagnosed prior to their death. Outside of the United States, several countries have documented their own epidemiological studies and compared them to what is known in

706:(episodic/sporadic), patients may not immediately seek treatment as the problem "disappears on its own." Furthermore, when pictured in the ideal clinical scenario (an older woman in her mid-50s), the spontaneous attacks of flushing, sweating, and a racing heart may be mistaken for 3251:

DOTATATE with the traditionally studied Lu analog and the various possibilities and novel treatment options these combinations will bring to the field. While the overall reported side-effects have been promising, it is important to note that a collaborative effort between the

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patients are studied by various categories, research has suggested that females are less likely to have extended survival with CVD chemotherapy compared to their male counterparts. Genetic status has been shown to greatly impact response to CVD. A team of researchers from the

2770:: There are several reasons a patient may have low blood pressure in the post-operative period. First and foremost, the tumor (and its abundance of catecholamines causing high blood pressure) has been removed. Furthermore, the patient may still experience the effects of their 1526:

The typical primary symptom is hypertension, which may be either episodic or continual. A diagnosis of pheochromocytoma should be suspected when the patient simultaneously presents with hypertension and the classic triad of heart palpitations, headaches, and profuse sweating.

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is a United States–based advocacy parent organization with the goal of promoting awareness and research opportunities to cure rare diseases. Groups such as these encourage patients to become their own advocates and change agents in their healthcare decision-making processes.

2947:. There have been several studied risk factors associated with the development of metastatic disease – while the patients genetic background plays an important role, the initial age of presentation and size of the tumor lead to negative outcomes. Of all the genetic variants, 3061:

from 1973 to 2015 and reported that 94% of patients acknowledged symptomatic improvement and over 80% of patients showed no evidence of recurrent disease five years post-therapy. Another report from the same institution looked at almost two decades of patients who underwent

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associated with male sex and synchronous metastases. Metastases are divided into synchronous and metachronous; those that are synchronous have developed within several months of the primary tumor, while metachronous metastases do not appear for a significant period of time.

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Whitelaw BC, Prague JK, Mustafa OG, Schulte KM, Hopkins PA, Gilbert JA, McGregor AM, Aylwin SJ. Phaeochromocytoma crisis. Clin Endocrinol (Oxf). 2014 Jan;80(1):13-22. doi: 10.1111/cen.12324. Epub 2013 Oct 17. Erratum in: Clin Endocrinol (Oxf). 2013 Mar;80(3):468. PMID:

3289:(no known genetic mutation), which commonly have low malignant potential. In 2019, a consortium of almost twenty European medical centers looked at the prognosis of malignant pheochromocytoma and the data starkly varies from the report of sporadic, single tumors, with a 3752:

Oyasu R, Yang XJ, Yoshida O, eds. (2008). "What is the difference between pheochromocytoma and paraganglioma? What are the familial syndromes that have pheochromocytoma as a component? What are the pathologic features of pheochromocytoma indicating malignancy?".

3140:(TMZ) and had progression free survival of 13 and 27 months, indicating that TMZ can be considered as an alternative treatment regimen in those who have progressed on CVD. Several studies have since reported successful responses with TMZ, particularly in the 869:

or strokes in patients with a pheochromocytoma. In a study of 130 patients with pheochromocytoma, 7 patients were diagnosed with a transient ischemic attack (the neurologic deficit completely resolved) and 3 patients experienced a stroke with persistent

3503:) became the first physician to successfully excise a pheochromocytoma. However, Mayo was likely unaware of the diagnosis prior to the operation. Not until 1929 was a pheochromocytoma recognized preoperatively. Throughout the early 1900s, the operative 2764:, with additional 5.0 mg dose every ten minutes until the blood pressure falls within an acceptable range. If the blood pressure is only minimally elevated, the patient can resume their alpha and beta-adrenoceptor antagonist from prior to surgery. 936:

Caused by an elevated inflammatory response, multiple organ dysfunction is a severe, life-threatening emergency with increasing mortality based on the number of systems involved. Pheochromocytoma-related MODS is associated with multiple organ failure,

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also conducted a study using a nationwide registry and reported incidence results of 0.57 per 100,000 person-years from 2011 to 2015, which was a significant increase from their 0.37 cases per 100,000 person-years reported from 1995 to 1999. Current

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imaging has become a major player in the diagnosis of patients with pheochromocytoma, with current estimates that 10–49% of all cases diagnosed after imaging was obtained for another reason. When an adrenal nodule (potential tumor) is discovered on

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procedure (traditional surgical technique) is currently preferred for extra-adrenal disease, unless the tumor is small, non-invasive, and in an easy to maneuver location. While previous data indicated the need for a minimally invasive approach with

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Roman-Gonzalez A, Zhou S, Ayala-Ramirez M, Shen C, Waguespack SG, Habra MA, et al. (July 2018). "Impact of Surgical Resection of the Primary Tumor on Overall Survival in Patients With Metastatic Pheochromocytoma or Sympathetic Paraganglioma".

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Brunaud L, Boutami M, Nguyen-Thi PL, Finnerty B, Germain A, Weryha G, et al. (December 2014). "Both preoperative alpha and calcium channel blockade impact intraoperative hemodynamic stability similarly in the management of pheochromocytoma".

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Lentschener C, Baillard C, Dousset B, Gaujoux S (February 2019). "Dogma is Made to be Broken. Why Are We Postponing Curative Surgery to Administer Ineffective Alpha Adrenoreceptor Blockade in Most Patients Undergoing Pheochromocytoma Removal?".

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physician (Charles Sugrue) penned a case report to the London Medical and Physical Journal describing the peculiar case of an 8-year-old male patient who had had seemingly random fits of pain concentrated in the abdomen accompanied by "a hectic

2559:) combined alpha-beta-adrenoceptor antagonists. These agents should be avoided whenever possible as there is upwards of seven times more beta-adrenoceptor antagonism than alpha, which can worsen hypertension and lead to a catecholamine crisis. 697:

While the symptoms of a pheochromocytoma are quite common, the disease has been referred to as "the great mimic". It is estimated that approximately 0.1% of patients with hypertension have a pheochromocytoma, and it is often misdiagnosed as

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should not be given alone in a pheochromocytoma patient – this can lead to severe consequences. In 1995, a team of physicians from London described the death of a person who had been recently diagnosed pheochromocytoma after initiation of

740:, often perscribed for hypertension, can lead to unopposed alpha in the context of pheochromocytoma. Most mortality associated with diagnosed pheochromocytoma came from surgery and hypertensive crisis, but mortality has greatly improved. 3293:

of 6.7 years. Overall survival improved if the patient had (1) disease of the head and neck compared to abdomen, (2) less than 40 years of age, (3) and if their biochemistry was less than five times the upper reference limit of normal.

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of unstratified pheochromocytoma patients who underwent CVD therapy showed that 37% of patients had a significant reduction in tumor volume, while 40% of patients experienced lower catecholamine burden. While there was no difference in

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type two receptor imaging with Ga-DOTA analogues. Over the last decade, further research continues to indicate the superiority of this functional imaging modality in a wide range of clinical scenarios, even surpassing anatomic imaging

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in the undiagnosed pheochromocytoma patient as their primary presenting symptom. Kidney failure is brought about by catecholamine-induced muscle injury. Norepinephrine causes vessels to narrow, thereby limiting blood flow and inducing

2741:: In the pheochromocytoma patient, postoperative hypertension could indicate incomplete tumor resection or another tumor of unknown location. However, the traditional, non-specific causes of postoperative hypertension including pain, 2143:) cannot often tolerate an MR as the machine is close-ended compared to the open-ended design of a CT. When patients become anxious and begin to move in the machine, this causes motion artifact, which occurs less in CT-based images. 1857:

While the above (3) conditions are likely to contribute to false-positive results if not controlled for, any value greater than 3 to 4 times the upper reference limit of normal should be considered diagnostic for a pheochromocytoma.

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Lentschener C, Gaujoux S, Thillois JM, Duboc D, Bertherat J, Ozier Y, Dousset B (April 2009). "Increased arterial pressure is not predictive of haemodynamic instability in patients undergoing adrenalectomy for phaeochromocytoma".

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Chruscinski A, Brede ME, Meinel L, Lohse MJ, Kobilka BK, Hein L (November 2001). "Differential distribution of beta-adrenergic receptor subtypes in blood vessels of knockout mice lacking beta(1)- or beta(2)-adrenergic receptors".

6127:"The triad of paragangliomas, gastric stromal tumours and pulmonary chondromas (Carney triad), and the dyad of paragangliomas and gastric stromal sarcomas (Carney-Stratakis syndrome): molecular genetics and clinical implications" 5550:

Lahlou-Laforêt K, Consoli SM, Jeunemaitre X, Gimenez-Roqueplo AP (May 2012). "Presymptomatic genetic testing in minors at risk of paraganglioma and pheochromocytoma: our experience of oncogenetic multidisciplinary consultation".

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Repeat administration of 25–50 mg intravenous hydrocortisone every eight hours for a maximum of 72 hours (3 days) after the operation. Convert to oral replacement therapy as soon as the patient is able to take medication by

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disease, current research indicates a successful operation is feasible and results in a shorter hospital stay. Literature within the last decade has also demonstrated that the robotic technique may be successfully utilized for

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or radionuclide therapy, or simply decrease the size of the tumor. Unfortunately, the intended relief from the procedure is often short-lived, especially if the patient has disease outside the abdomen. A 2013 study from the

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Ebbehoj AL, Sondergaard E, Trolle C, Stochholm K, Poulsen PL (2017-05-03). "The epidemiology of pheochromocytoma: increasing incidence and changing clinical presentation. A population-based retrospective study 1977–2015".

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Pacak K, Eisenhofer G, Ahlman H, Bornstein SR, Gimenez-Roqueplo AP, Grossman AB, et al. (February 2007). "Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005".

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Several additional gene variants have been described, but the provided information is inconsistent and a consensus has not been reached in the community if these mutations are truly pheochromocytoma susceptibility genes.

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reported two unfortunate cases of rapid disease progression following a remarkable, almost complete response to PRRT. While the etiology of their recurrence is unknown, the team speculated that an elevated tumor marker

2805:(sugar). Blood glucose levels should be checked frequently in the perioperative period and insulin should be given as needed if levels are elevated. Following resection, tumor-related hyperglycemia is likely to resolve. 3347:

National Health Insurance Service data, the prevalence of a pheochromocytoma was reported at 2.13 per 100,000 persons with an incidence of 0.18 per 100,000 person-years. This is lower than the occurrence reported from

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Averbuch SD, Steakley CS, Young RC, Gelmann EP, Goldstein DS, Stull R, Keiser HR (August 1988). "Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine, and dacarbazine".

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It is important to note that larger tumors even for those larger than 6.0 cm can be attempted with a minimally invasive approach, but the team should be prepared to convert to an open procedure if necessary. An

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PET – the tumor is appreciated as the dark structure in the patient's left chest. Darkened structure at head of patient is the brain, posterior to the abdomen are the kidneys, in the pelvis is the bladder. These are

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When plasma metanephrine levels were elevated to greater than 15% of the combined levels of normetanephrine and metanephrine, an adrenal tumor or a recurrence of an adrenal tumor that had already been excised can be

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Twenty-four hour urinary metanephrines are an acceptable alternative if the plasma test is unavailable. Other additional biomarkers can be helpful to aid in the diagnosis of pheochromocytoma as well, most notable is

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during the operation. Therefore, a high-sodium diet with adequate fluid intake should be encouraged prior to surgery. Some institutions in the United States will even admit patients the night prior to surgery for

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Goffredo P, Adam MA, Thomas SM, Scheri RP, Sosa JA, Roman SA (August 2015). "Patterns of Use and Short-Term Outcomes of Minimally Invasive Surgery for Malignant Pheochromocytoma: A Population-Level Study".

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Williams MD. Paragangliomas of the Head and Neck: An Overview from Diagnosis to Genetics. Head Neck Pathol. 2017 Sep;11(3):278-287. doi: 10.1007/s12105-017-0803-4. Epub 2017 Mar 20. PMID: 28321772; PMCID:

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Patients should be transitioned to a normal maintenance (regular, daily) dose of steroids prior to discharge and referred to endocrinology for proper titration and management. Depending on the patient's

7674:"European Association of Nuclear Medicine Practice Guideline/Society of Nuclear Medicine and Molecular Imaging Procedure Standard 2019 for radionuclide imaging of phaeochromocytoma and paraganglioma" 3322:

of pheochromocytoma is between 1:2,500 and 1:6,500, meaning that for every 2,500–6,500 people, there is (on average) one person with pheochromocytoma. In the United States, this equates to an annual

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reported that a majority of patients with recurrent biochemical evidence of disease within one year of the operation and less than 30% continued to be biochemically free of disease after five years.

2931:) where they are not normally found. Patients with a paraganglioma are more likely to develop metastases than those with a pheochromocytoma. The most common extra-adrenal sites of metastases are the 7621:

Taïeb D, Tessonnier L, Sebag F, Niccoli-Sire P, Morange I, Colavolpe C, et al. (2008). "The role of 18F-FDOPA and 18F-FDG-PET in the management of malignant and multifocal phaeochromocytomas".

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Prejbisz A, Lenders JW, Eisenhofer G, Januszewicz A. Mortality associated with phaeochromocytoma. Horm Metab Res. 2013 Feb;45(2):154-8. doi: 10.1055/s-0032-1331217. Epub 2013 Jan 15. PMID: 23322516.

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Satapathy S, Mittal BR, Bhansali A (December 2019). "Peptide receptor radionuclide therapy in the management of advanced pheochromocytoma and paraganglioma: A systematic review and meta-analysis".

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imaging, there is a 5–10% chance the lesion is a pheochromocytoma. The incidence of adrenal tumors is found in the infographic above, with pheochromocytoma noted in yellow in the top right corner.

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Despite all of the below potential treatment options, recent literature highlights that (for most patients) metastatic pheochromocytoma is slow-growing. In patients with minimal disease burden, a "

2700:) where excess fluid is dangerous. While there is still no recognized consensus or gold standard, providers should individualize the decision based on the patient's perceived nutritional standing, 2539:

is then prescribed to control the heart rate. Just as with the alpha antagonists, there are selective (beta-1) and non-selective (beta-1 and beta-2) adrenoceptor antagonists. The selective agents (

2471:. These effects will decrease with time, but providers can try to avoid them by starting at a low-dose and slowly increasing until they reach their desired amount. In patient's with uncontrolled 1853:

lab draw. Patients should also avoid catecholamine-containing foods (fruits, fruit drinks, chocolate, caffeine, tomatoes, beans, nuts, and potatoes) for a minimum of 24 hours prior to collection.

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Gimenez-Roqueplo AP, Robledo M, Dahia PLM. Update on the genetics of paragangliomas. Endocr Relat Cancer. 2023 Mar 8;30(4):e220373. doi: 10.1530/ERC-22-0373. PMID: 36748842; PMCID: PMC10029328.

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Typically, complete or total adrenalectomy is performed; however, a technique referred to as "cortical-sparing" can leave a remnant (piece) of the adrenal gland in hopes of avoiding life-long

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Grossrubatscher E, Dalino P, Vignati F, Gambacorta M, Pugliese R, Boniardi M, et al. (September 2006). "The role of chromogranin A in the management of patients with phaeochromocytoma".

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and direct tumor manipulation, making surgery one of the most dangerous times for a pheochromocytoma patient if not properly prepared. In order to help circumvent a catecholamine-crisis, the

11200:"The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer" 2083:

Across both an adrenergic and a noradrenergic phenotype, the greater the sum of plasma or urinary concentrations of metanephrine and normetanephrine, the larger the expected tumor diameter.

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for metastatic pheochromocytoma and reported that local control was achieved in over 85% of targeted lesions and that 92% of procedures were associated with reduced pain and/or symptoms of

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with pheochromocytoma. Dr. Foreman, one of the doctors, attempts to appeal the inmate's death penalty on the basis that he was unable to control his actions due to his tumor. This kind of

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have been described as a dormant volcano, ready to erupt at any time, wreaking catastrophic havoc on the body. While an eruption can occur at any time, two of the most common triggers are

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results in the correct clinical scenario. When interpreting a biochemical analysis for pheochromocytoma, the provider must pay close attention to the (1) conditions of the collection, (2)

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is found on a scan ordered for another reason. For example, "Patient M" presents to his local emergency room for abdominal pain and a CT is ordered to rule-out appendicitis; however, the

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Jiang M, Ding H, Liang Y, Tang J, Lin Y, Xiang K, et al. (March 2018). "Preoperative risk factors for haemodynamic instability during pheochromocytoma surgery in Chinese patients".

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mitigate any intraoperative catecholamine crisis. These articles resulted in rebuttals from research teams in the United States, but an international consensus has not yet been reached.

9885:

Jimenez C, Rohren E, Habra MA, Rich T, Jimenez P, Ayala-Ramirez M, Baudin E (August 2013). "Current and future treatments for malignant pheochromocytoma and sympathetic paraganglioma".

9701:

Ferrer-Inaebnit E, Segura-Sampedro JJ, Alfonso-García M, González-Argente X, Morales-Soriano R (January 2021). "Cytoreductive surgery in functioning peritoneal pheochromocytomatosis".

2479:) should be utilized. Unfortunately, compared to the selective agents listed above, phenoxybenzamine is much more expensive and may not be readily available to some patients. Common 2431:

blockade a minimum of seven days prior to surgery. There are several medication options depending on the clinical scenario, each with their own associated strengths and weaknesses.

3426:

Professor Ludwig Pick, the German physician who first coined the term "pheochromocytoma" in 1912 after recognizing the color-change associated with the addition of chromium salts

8267:"Comparison of Pheochromocytoma-Specific Morbidity and Mortality Among Adults With Bilateral Pheochromocytomas Undergoing Total Adrenalectomy vs Cortical-Sparing Adrenalectomy" 3014:

procedure is performed (removing as much of the cancerous tissue as possible) in order to reduce patient symptoms by removing the source of catecholamines, improve response to

5136:

Takabatake T, Kawabata M, Ohta H, Yamamoto Y, Ishida Y, Hara H, Hattori N (July 1985). "Acute renal failure and transient, massive proteinuria in a case of pheochromocytoma".

7780:"PET/CT comparing (68)Ga-DOTATATE and other radiopharmaceuticals and in comparison with CT/MRI for the localization of sporadic metastatic pheochromocytoma and paraganglioma" 2640:

that called into question the current recommendations for alpha-blockade. The study examined the intraoperative maximal systolic arterial pressure in people with and without

12007: 4758:

Cohen JK, Cisco RM, Scholten A, Mitmaker E, Duh QY (April 2014). "Pheochromocytoma crisis resulting in acute heart failure and cardioembolic stroke in a 37-year-old man".

3487:" after they discovered extra-adrenal tissue that reacted to chromium salts, which mimicked the reaction of the adrenal medulla. Just four years later, German pathologist 4901:

Luiz HV, da Silva TN, Pereira BD, Santos JG, Gonçalves D, Manita I, Portugal J (December 2013). "Malignant paraganglioma presenting with hemorrhagic stroke in a child".

2599:. Despite attempts at resuscitation, the person died several days later. This complication is related to the impact that alpha and beta-adrenoceptor antagonists have on 3398:

Despite the prominence in many respected textbooks, these guidelines have since been established as inaccurate and are not used in current epidemiological discussions.

2139:

damage and should therefore be avoided in patients with pre-existing damage. However, patients who struggle with being in confined spaces for extended periods of time (

2911:) following tumor resection. However, the above are more likely to be encountered, which is why their management has been specifically outlined here in this article. 2315:

Also, HED-PET has shown to be an accurate tool to diagnose and rule out pheochromocytoma in complex clinical scenarios and to characterise equivocal adrenal tumours.

5093:

Celik H, Celik O, Guldiken S, Inal V, Puyan FO, Tugrul A (February 2014). "Pheochromocytoma presenting with rhabdomyolysis and acute renal failure: a case report".

2127:

on if CT or MR is the preferred imaging modality in pheochromocytoma, each method has its associated strengths and weaknesses. As CT expose the patient to ionizing

1530:

If a patient has the characteristic signs and symptoms of a pheochromocytoma and the decision is made to pursue additional biochemical (blood work) evaluation, the

10247:"Treatment of malignant pheochromocytoma/paraganglioma with cyclophosphamide, vincristine, and dacarbazine: recommendation from a 22-year follow-up of 18 patients" 2193:– the pheochromocytoma is appreciated in the left panel on the right side of the screen (right panel; left side of the screen) as the darkened circle towards the 1834:

cause false elevations in metanephrine levels. Finally, it is important to have open, non-judgemental discussions about the patient's recreational substance use.

7731:"18F-FDG and anatomic imaging in the detection of succinate dehydrogenase mutation (SDHx)–related pheochromocytoma and paraganglioma in the pediatric population" 714:. Unmanaged pheochromocytoma is dangerous and can lead to serious, potentially fatal, complications, including stroke and hypertension-induced organ damage. The 3536:

The zebra has become a powerful symbol in the pheochromocytoma advocacy community and represents the rare medical cases that are more likely to be misdiagnosed.

1875:

lifestyle habits that could alter the results. If the offending medications cannot be discontinued or repeated labs remained the same, consider administering a

9928:

Pappachan JM, Raskauskiene D, Sriraman R, Edavalath M, Hanna FW (July 2014). "Diagnosis and management of pheochromocytoma: a practical guide to clinicians".

8614:"Are patients with hormonally functional phaeochromocytoma and paraganglioma initially receiving a proper adrenoceptor blockade? A retrospective cohort study" 4971:

Anderson NE, Chung K, Willoughby E, Croxson MS (April 2013). "Neurological manifestations of phaeochromocytomas and secretory paragangliomas: a reappraisal".

3265:) could be an indication of a poor response to PRRT and called for pre-PRRT assessments to include Ki-67 values to help individualize patient treatment plans. 3247:

are minimal, with only 5 out of 102 patients choosing to voluntarily initiate treatment discontinuation. Newer reports have detailed the utility of combining

12252: 11624:"Ein Fall von doppelseitigem, völlig latent verlaufenen Nebennierentumor und gleichzeitiger Nephritis mit Veränderungen am Circulationsapparat und Retinitis" 3524:

that works to connect patients with rare diseases and allows them to meet other individuals who are experiencing similar diagnoses and treatment strategies.

2183:),(5) 11C-Hydroxy ephedrine(HED-PET). From this point forward, these imaging modalities will be referred to in their abbreviated names found in parentheses. 5241:

Newell KA, Prinz RA, Pickleman J, Braithwaite S, Brooks M, Karson TH, Glisson S (August 1988). "Pheochromocytoma multisystem crisis. A surgical emergency".

3100:, collectively known as CVD. Response to therapy is measured by a reduction in total tumor volume as well as symptomatic relief, reported by the patient. A 2499:. While uncommon, patients may have a hormonally-active pheochromocytoma and a normal blood pressure. One comparison from 2014 found that a small dose of a 5328: 12387: 10345:"Continued Tumor Reduction of Metastatic Pheochromocytoma/Paraganglioma Harboring Succinate Dehydrogenase Subunit B Mutations with Cyclical Chemotherapy" 3456:

Felix Fraenkel, who provided a clinical and morphologic picture of this tumor. While various physicians were recognizing symptoms and treating patients,

3326:(new cases per year) of 500 to 1,600 cases. However, approximations in the early 2000s reported that upwards of 50% of pheochromocytoma diagnoses are at 2277:

mutations, F-FDOPA fell inferior to the traditional FDG-PET. However, for patients with genetic variants in other pheochromocytoma-susceptibility genes (

808:(a catecholamine) is hypothesized to result in damaged cardiac tissue by inhibiting coronary blood flow and depriving cells of oxygen, thus resulting in 5435: 5424: 3132:

However, CVD is not the only proven chemotherapeutic regimen in the pheochromocytoma patient. A 2018 report demonstrated the remarkable response of two

2344:

intraoperatively. Without frequent and adequate communication between all of the above-mentioned teams, a favorable outcome is much more difficult. The

10162:"Chemotherapy with cyclophosphamide, vincristine and dacarbazine for malignant paraganglioma and pheochromocytoma: systematic review and meta-analysis" 8410:"Why Take the Risk? We Only Live Once: The Dangers Associated with Neglecting a Pre-Operative Alpha Adrenoceptor Blockade in Pheochromocytoma Patients" 11950: 11069:"Prognosis of Malignant Pheochromocytoma and Paraganglioma (MAPP-Prono Study): A European Network for the Study of Adrenal Tumors Retrospective Study" 10304:"Survival of patients with metastatic malignant pheochromocytoma and efficacy of combined cyclophosphamide, vincristine, and dacarbazine chemotherapy" 3038:

Patient receiving radiation therapy to the region of the head and neck. Full facial mold is in place to protect areas where they do not want exposure.

2364:

socioeconomic effects, and operative and postoperative parameter, the evidence is uncertain about the effects of either interventions over the other.

841:

is the most common abnormal heart rhythm associated with a pheochromocytoma and is experienced by patients as the feeling of a "fluttering heart" or

130: 8036:

Arezzo A, Bullano A, Cochetti G, Cirocchi R, Randolph J, Mearini E, et al. (Cochrane Metabolic and Endocrine Disorders Group) (December 2018).

7480:

Rufini V, Treglia G, Perotti G, Giordano A (January 2013). "The evolution in the use of MIBG scintigraphy in pheochromocytomas and paragangliomas".

10443:"Temozolomide Is a Potential Therapeutic Tool for Patients With Metastatic Pheochromocytoma/Paraganglioma-Case Report and Review of the Literature" 3285:

article about prognosis published in 2000 reported a 91% 5-year survival rate in their patient population; however, over 86% of their patients had

3166:

improvement. There are several patients who are not eligible for MIBG treatment, including pregnant women (exposure to radiation is harmful to the

2843:

found that some beta blocker use may cause people to more prone to hypoglycemia and not experience these symptoms, which could delay the diagnosis.

3508:

physician-scientists have been recognizing patterns in patients with pheochromocytoma and identifying genetic associations and various syndromes.

1943:

While diagnostic, laboratory values can also provide physician's with important information about the type, location, size, and associated tumor

1819: 11982: 4528:

Zhang R, Gupta D, Albert SG (December 2017). "Pheochromocytoma as a reversible cause of cardiomyopathy: Analysis and review of the literature".

3679:

Beard CM, Sheps SG, Kurland LT, Carney JA, Lie JT (December 1983). "Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979".

1003:

A negative result may be upsetting to a child if their sibling was positive; an opportunity to ask questions and process results may be helpful.

1000:

Testing one pediatric sibling at a time allows the family to narrow their focus when results are returned and support each sibling individually.

11461:"Pheochromocytoma/paraganglioma: recent updates in genetics, biochemistry, immunohistochemistry, metabolomics, imaging and therapeutic options" 9971:

Buhl T, Mortensen J, Kjaer A (March 2002). "I-123 MIBG imaging and intraoperative localization of metastatic pheochromocytoma: a case report".

3031:(I-MIBG) prior to surgery and then scanning the patient intraoperatively with a probe to detect disease that may be missed with the naked eye. 1903:

overnight, patient's will present to their testing site for a baseline metanephrines blood draw and clonidine administration. They will remain

7394:

Leung K, Stamm M, Raja A, Low G (February 2013). "Pheochromocytoma: the range of appearances on ultrasound, CT, MRI, and functional imaging".

12057: 977:

Additional practices may help maintain the emotional and psychological well-being of the minor. Screening includes a multidisciplinary team (

5165:"Multiple organ failure and coma as initial presentation of pheochromocytoma in a patient with multiple endocrine neoplasia (MEN) type II A" 997:

A positive result from testing during family-observed days of celebration may mask the happiness associated with these events in the future.

10014:

De Filpo G, Maggi M, Mannelli M, Canu L (June 2020). "Management and outcome of metastatic pheochromocytomas/paragangliomas: an overview".

9838:"Response after surgical resection of metastatic pheochromocytoma and paraganglioma: can postoperative biochemical remission be predicted?" 11276:"Epidemiology and Prognosis of Pheochromocytoma/Paraganglioma in Korea: A Nationwide Study Based on the National Health Insurance Service" 12490: 11247:

Sutton MG, Sheps SG, Lie JT (June 1981). "Prevalence of clinically unsuspected pheochromocytoma. Review of a 50-year autopsy series".

10998: 5645:

Favier J, Amar L, Gimenez-Roqueplo AP (February 2015). "Paraganglioma and phaeochromocytoma: from genetics to personalized medicine".

10645:

Carrasquillo JA, Pandit-Taskar N, Chen CC (May 2016). "I-131 Metaiodobenzylguanidine Therapy of Pheochromocytoma and Paraganglioma".

3545:

has become increasingly powerful to the rare disease community and resulted in several organizations, societies, and special events (

3495:. Many surgeons attempted to remove these tumors over the next decade, but their patients died intraoperatively from shock. In 1926, 2972:" approach with frequent imaging to monitor disease is favorable, withholding treatment until evidence of progression is visualized. 1675: 5017:

Shemin D, Cohn PS, Zipin SB (November 1990). "Pheochromocytoma presenting as rhabdomyolysis and acute myoglobinuric renal failure".

3565:

In July 2012, an actual pheochromocytoma patient, Tannis Brown, former vice-president of the PheoPara Troopers, was featured on the

3516:

While a rare disease, there have been several references to pheochromocytoma in popular culture and the media, specifically medical

12158: 8227:

Berber E, Mitchell J, Milas M, Siperstein A (August 2010). "Robotic posterior retroperitoneal adrenalectomy: operative technique".

3553: 812:

tissue. Fortunately, following tumor excision and the subsequent quelling of catecholamines, the damage has been proven reversible.

514:(PGL) family of tumors, being defined as an intra-adrenal PGL . These neuroendocrine tumors can be sympathetic, where they release 12033: 2811:: After the tumor is removed, insulin is no longer inhibited, which can bring the blood glucose dangerously low. Symptoms include 12380: 3637: 2965:

approach to the original disease, especially in big tumors, has been appointed as an important risk factor for tumoral seeding.

1947:. There are (3) major, well-recognized biochemical phenotypes that can be used by health care providers to direct patient care. 3366:

life. When younger patients are diagnosed with a pheochromocytoma, there should be a high suspicion for hereditary disease, as

3010:; leave no remnant of disease. However, with widespread metastatic disease, this is not always feasible. Therefore, a surgical 2883:, the total typical daily dose of hydrocortisone is between 15 and 25 mg daily (divided into morning and afternoon pills). 2401: 2283: 1082: 191: 12149: 11686: 9077:

Wong C, Yu R (July 2010). "Preoperative preparation for pheochromocytoma resection: physician survey and clinical practice".

6101: 5225: 3927: 3770: 3228: 880:

published). While 71% of the studied patients reported headaches, just over 20% of the affected patients endorsed associated

163: 7883:"Peptide Receptor Radionuclide Therapy as a Novel Treatment for Metastatic and Invasive Phaeochromocytoma and Paraganglioma" 6622:

d'Herbomez M, Do Cao C, Vezzosi D, Borzon-Chasot F, Baudin E (September 2010). "Chromogranin A assay in clinical practice".

4087:

Manger WM (August 2006). "An overview of pheochromocytoma: history, current concepts, vagaries, and diagnostic challenges".

2355:(minimally invasive technique) for most adrenal tumors, unless they are invasive or are larger than 6.0 centimeters. A 2018 12721: 10494:"SDHB mutations are associated with response to temozolomide in patients with metastatic pheochromocytoma or paraganglioma" 9649:"Synchronous and metachronous liver metastases in patients with colorectal cancer-towards a clinically relevant definition" 4617:

Van YH, Wang HS, Lai CH, Lin JN, Lo FS (November 2002). "Pheochromocytoma presenting as stroke in two Taiwanese children".

4284:

Prejbisz A, Lenders JW, Eisenhofer G, Januszewicz A (November 2011). "Cardiovascular manifestations of phaeochromocytoma".

11325:"Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A nationwide study and systematic review" 7031:"Precision Medicine: An Update on Genotype/Biochemical Phenotype Relationships in Pheochromocytoma/Paraganglioma Patients" 5788:"First report of bilateral pheochromocytoma in the clinical spectrum of HIF2A-related polycythemia-paraganglioma syndrome" 3045:– With regard to pheochromocytoma, radiation techniques are primarily used for pain control, specifically with regards to 2607:. The normal blood vessel is open, allowing for adequate blood flow. When catecholamines activate the alpha receptor, the 2258:

is the most common and readily available functional imaging technique at most hospital systems, but the least-specific to

961:

recommends that all patients diagnosed with a pheochromocytoma undergo an evaluation with a genetic counselor to consider

12716: 12711: 12263: 10108:"Efficacy and Safety of Ablative Therapy in the Treatment of Patients with Metastatic Pheochromocytoma and Paraganglioma" 7085:"Pheochromocytoma catecholamine phenotypes and prediction of tumor size and location by use of plasma free metanephrines" 2395: 1773: 1045: 929: 12136: 8134:

Zhang, Liang; Chen, Danlei; Pang, Yingxian; Guan, Xiao; Xu, Xiaowen; Wang, Cikui; Xiao, Qiao; Liu, Longfei (July 2022).

3573:

The show highlighted her personal struggle with misdiagnosed disease as many physicians felt her episodic headaches and

2507:) may be used pre-operatively in some people. This will not drastically lower the patients blood pressure and make them 170: 12373: 10394:"Successful Second-Line Metronomic Temozolomide in Metastatic Paraganglioma: Case Reports and Review of the Literature" 5596:"65 YEARS OF THE DOUBLE HELIX: Genetics informs precision practice in the diagnosis and management of pheochromocytoma" 3186:) are started prior to treatment and need to be continued for at least three weeks after therapy concludes. Associated 1012:

The following table(s) detail the clinical characteristics of the well-known hereditary pheochromocytoma gene variants

6470:"Dietary influences on plasma and urinary metanephrines: implications for diagnosis of catecholamine-producing tumors" 3377:

Likelihood of diagnosis when an adrenal-nodule is identified; pheochromocytoma is in yellow near the top-right corner.

3298:

from primary tumor to presence of metastases. The actual location of the metastases can also indicate prognosis, with

9744:

Corssmit EP, Snel M, Kapiteijn E (January 2020). "Malignant pheochromocytoma and paraganglioma: management options".

5436:

https://www.england.nhs.uk/wp-content/uploads/2018/08/Rare-and-inherited-disease-eligibility-criteria-version-5.2.pdf

5425:

https://www.england.nhs.uk/wp-content/uploads/2018/08/Rare-and-inherited-disease-eligibility-criteria-version-5.2.pdf

3468:

system, which would later become crucial to the diagnosis of these tumors. Furthermore, he also introduced the term "

3257: 226: 101: 52: 38: 11108:

Hamidi O (June 2019). "Metastatic pheochromocytoma and paraganglioma: recent advances in prognosis and management".

2415:

Arguably, the most important part of a pheochromocytoma surgical plan is an adequate pre-operative blockade. Excess

149: 12742: 12186: 11720:

Kiernan CM, Solórzano CC (January 2016). "Pheochromocytoma and Paraganglioma: Diagnosis, Genetics, and Treatment".

4376:

Liao WB, Liu CF, Chiang CW, Kung CT, Lee CW (September 2000). "Cardiovascular manifestations of pheochromocytoma".

2439:

If the patient's blood pressure is moderately elevated, a selective, short-acting alpha-1 adrenoceptor antagonist (

11323:

Berends AM, Buitenwerf E, de Krijger RR, Veeger NJ, van der Horst-Schrivers AN, Links TP, Kerstens MN (May 2018).

6285:"Evaluating the optimum rest period prior to blood collection for fractionated plasma free metanephrines analysis" 2849:

Following a bilateral adrenalectomy (left and right), the patient is no longer capable of secreting the necessary

177: 12570: 12417: 9557:"Outcomes of patients with metastatic phaeochromocytoma and paraganglioma: A systematic review and meta-analysis" 6921:"Plasma methoxytyramine: clinical utility with metanephrines for diagnosis of pheochromocytoma and paraganglioma" 4043:

Hosseinnezhad A, Black RM, Aeddula NR, Adhikari D, Trivedi N (2011). "Glucagon-induced pheochromocytoma crisis".

3125:

mutations are not only more likely to initially respond to CVD, but that they also experienced over 30 months of

2669:

starting at midnight until the time of the operation. However, a small trial from 2009 reported no difference in

2619:(gets larger) and allows for increased blood flow, reducing the blood pressure. If a pheochromocytoma patient is 1102: 10682:"Efficacy of Peptide Receptor Radionuclide Therapy for Functional Metastatic Paraganglioma and Pheochromocytoma" 6033:"Recurrent Mutations of Chromatin-Remodeling Genes and Kinase Receptors in Pheochromocytomas and Paragangliomas" 2692:

that the providers chose to forego volume repletion in the older patient population for fear of their potential

1414:

There have been several published case reports of other, rare pheochromocytoma-associated susceptibility genes:

11803:"Anaesthetic management of a case of adrenal and extra-adrenal phaeochromocytoma for preoperative embolisation" 3491:

coined the term "pheochromocytoma" after he observed the consistent color change in tumors associated with the

3239: 3071: 2828: 1501: 1485: 1229: 821:

Pheochromocytomas have been implicated in various types of cardiomyopathy, including (myocarditis, see above),

4944:

Potapova G, Chazova I, Kuznetsov N, Sitina V, Popov E, Gavrilov I (June 2011). "Pheochromocytoma and Stroke".

2451:) is the preferred agent. However, the patient should be warned about the potential side-effect known as "the 2393:

if the left and right adrenal glands need to be removed. The issue is particularly important in patients with

5837:"Novel HIF2A mutations disrupt oxygen sensing, leading to polycythemia, paragangliomas, and somatostatinomas" 3588: 3253: 3115: 3020: 3000:. There are several treatment options available to patients depending on the amount and location of disease: 2908: 2022: 159: 7835:

Kroiss AS, Uprimny C, Shulkin BL, Gruber L, Frech A, Jazbec T, et al. (March 2019). "18F-DOPA PET/CT".

7525:"(131)I-MIBG therapy for malignant paraganglioma and phaeochromocytoma: systematic review and meta-analysis" 538:

1920s: from phaeochrome (another term for chromaffin), from Greek phaios 'dusky' + khrōma 'color', + -cyte.

12747: 12703: 8893:"A Necessity, Not a Second Thought: Pre-Operative Alpha-Adrenoceptor Blockade in Pheochromocytoma Patients" 2328:

Surgical resection is the only curative option for pheochromocytoma as of 2019. A successful excision is a

2255: 2233: 2168: 756:. Patients require immediate, life-saving treatment to prevent further damage to other organs and/or death. 10680:

Kong G, Grozinsky-Glasberg S, Hofman MS, Callahan J, Meirovitz A, Maimon O, et al. (September 2017).

4236:

Riester A, Weismann D, Quinkler M, Lichtenauer UD, Sommerey S, Halbritter R, et al. (December 2015).

12145: 9270: 6743:

Kosman ME (July 1975). "Evaluation of clonidine hydrochloride (Catapres). A new antihypertensive agent".

3136:

patients who failed CVD chemotherapy (disease progressed despite medication), but were then treated with

2701: 2677: 1815: 1769: 1765: 1714: 588: 10059:"External beam radiation therapy for advanced/unresectable malignant paraganglioma and pheochromocytoma" 9313:"Effect of beta blocker use and type on hypoglycemia risk among hospitalized insulin requiring patients" 4330:

Young WF (December 2007). "Adrenal causes of hypertension: pheochromocytoma and primary aldosteronism".

2954:

mutations have the highest rates of developing metastatic disease. Another study has reported increased

1764:

is considered the gold standard diagnosis for pheochromocytoma. Over 10 studies have confirmed that the

7221:"Current and future anatomical and functional imaging approaches to pheochromocytoma and paraganglioma" 3411: 3402: 3058: 2993: 2305: 2109: 2101: 547: 10492:

Hadoux J, Favier J, Scoazec JY, Leboulleux S, Al Ghuzlan A, Caramella C, et al. (December 2014).

7355:"15 YEARS OF PARAGANGLIOMA: Imaging and imaging-based treatment of pheochromocytoma and paraganglioma" 6429:"Elevated urinary free and deconjugated catecholamines after consumption of a catecholamine-rich diet" 6244:"Biochemical diagnosis of pheochromocytoma: how to distinguish true- from false-positive test results" 5984:"Vascular Changes in the Retina and Choroid of Patients With EPAS1 Gain-of-Function Mutation Syndrome" 5289:

Fishbein L (February 2016). "Pheochromocytoma and Paraganglioma: Genetics, Diagnosis, and Treatment".

2774:, which causes lower blood pressure. First-line treatment for postoperative hypotension is aggressive 2427:

recommends that all patients with functional (hormonally active) tumors be started on a pre-operative

1879:

suppression test. In the 1970s, the drug clonidine hydrocloride swept the market as a novel agent for

12560: 12537: 12140: 11510:

Conzo G, Pasquali D, Colantuoni V, Circelli L, Tartaglia E, Gambardella C, et al. (2014-05-01).

11067:

Hescot S, Curras-Freixes M, Deutschbein T, van Berkel A, Vezzosi D, Amar L, et al. (June 2019).

8315: 7268:

McCullough PA, Choi JP, Feghali GA, Schussler JM, Stoler RM, Vallabahn RC, Mehta A (September 2016).

3566: 3330:; therefore, the above estimations may be lower than expected. In a 50-year autopsy case series, the 3277: 3126: 2124: 1418: 866: 138: 76: 10830:

Vyakaranam AR, Crona J, Norlén O, Granberg D, Garske-Román U, Sandström M, et al. (June 2019).

6700:

Därr R, Lenders JW, Stange K, Kindel B, Hofbauer LC, Bornstein SR, Eisenhofer G (January 2013). "".

5384:

Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SK, Murad MH, et al. (June 2014).

4502: 3792: 3381:

Classically, the pheochromocytoma "rules of 10" have been taught, particularly to medical students:

2778:, which is why ensuring the patient is well-hydrated (see above) prior to surgery is so imperative. 733:, mainly due to hypertensive crisis in the mother and intrauterine growth restriction in the fetus. 12613: 12553: 12532: 12396: 12274: 7083:

Eisenhofer G, Lenders JW, Goldstein DS, Mannelli M, Csako G, Walther MM, et al. (April 2005).

3323: 3178:, and those are who not expected to live longer than three months. As MIBG therapy can destroy the 2243: 2010:

Patients are more likely to present with continuous, persistent pheochromocytoma-related symptoms (

1811: 1667: 1459: 1066: 949:

Current estimates predict that upwards of 40% of all pheochromocytomas are related to an inherited

826: 8085:

Aggeli C, Nixon AM, Parianos C, Vletsis G, Papanastasiou L, Markou A, et al. (October 2017).

6174: 2055:

Patients are more likely to be asymptomatic; however, they may present with non-specific signs of

259: 12523: 8038:"Transperitoneal versus retroperitoneal laparoscopic adrenalectomy for adrenal tumours in adults" 7173:

Neumann HP, Young WF, Eng C (August 2019). Longo DL (ed.). "Pheochromocytoma and Paraganglioma".

3367: 3203: 3154: 3119: 3063: 3050: 3028: 2948: 2880: 2500: 2496: 2468: 2309: 2296: 2229: 2172: 2074: 2026: 1916:

following clonidine. Patients should not depend on themselves for transport following this test.

1835: 770: 661: 394: 267:

Normal remnant adrenal gland (left) with a pheochromocytoma (right) involving the adrenal medulla

134: 10973: 9403:

MacKenzie CR, Goodman SM (July 2016). "Stress Dose Steroids: Myths and Perioperative Medicine".

6332:

Lenders JW, Willemsen JJ, Eisenhofer G, Ross HA, Pacak K, Timmers HJ, Sweep CG (February 2007).

5503:"SDHB-related pheochromocytoma and paraganglioma penetrance and genotype–phenotype correlations" 12608: 10106:

Kohlenberg J, Welch B, Hamidi O, Callstrom M, Morris J, Sprung J, et al. (February 2019).

6242:

Eisenhofer G, Goldstein DS, Walther MM, Friberg P, Lenders JW, Keiser HR, Pacak K (June 2003).

2746: 2068: 1627: 1531: 822: 778:(heart) tissue and exert negative effects including oxygen deprivation, leading to accelerated 699: 12325: 9364:"Selective use of steroid replacement after adrenalectomy: lessons from 331 consecutive cases" 8800:"Perioperative α-receptor blockade in phaeochromocytoma surgery: an observational case series" 6919:

Rao D, Peitzsch M, Prejbisz A, Hanus K, Fassnacht M, Beuschlein F, et al. (August 2017).

3811:

Lenders JW, Pacak K, Walther MM, Linehan WM, Mannelli M, Friberg P, et al. (March 2002).

184: 12752: 12665: 12086:"Does this patient have pheochromocytoma? A systematic review of clinical signs and symptoms" 11023:

Noshiro T, Shimizu K, Watanabe T, Akama H, Shibukawa S, Miura W, et al. (January 2000).

10729:

Strosberg J, Wolin E, Chasen B, Kulke M, Bushnell D, Caplin M, et al. (September 2018).

9836:

Ellis RJ, Patel D, Prodanov T, Sadowski S, Nilubol N, Adams K, et al. (September 2013).

9501:

Zelinka T, Musil Z, Dušková J, Burton D, Merino MJ, Milosevic D, et al. (October 2011).

9127:

Mamilla D, Araque KA, Brofferio A, Gonzales MK, Sullivan JN, Nilubol N, Pacak K (July 2019).

8136:"Surgical treatment of large pheochromocytoma (>6 cm): A 10-year single-center experience" 5982:

Dmitriev PM, Wang H, Rosenblum JS, Prodanov T, Cui J, Pappo AS, et al. (December 2019).

3436: 3373: 3007: 2846: 2779: 2588: 2492: 2452: 2164: 1799: 953:

susceptibility mutation. Of the remaining 60% of tumors, more than 30% are associated with a

797: 760: 44: 10778:

Strosberg J, El-Haddad G, Wolin E, Hendifar A, Yao J, Chasen B, et al. (January 2017).

10245:

Huang H, Abraham J, Hung E, Averbuch S, Merino M, Steinberg SM, et al. (October 2008).

9700: 9183:

Naranjo J, Dodd S, Martin YN (August 2017). "Perioperative Management of Pheochromocytoma".

5594:

Neumann HP, Young WF, Krauss T, Bayley JP, Schiavi F, Opocher G, et al. (August 2018).

5501:

Jochmanova I, Wolf KI, King KS, Nambuba J, Wesley R, Martucci V, et al. (August 2017).

12655: 12485: 12154: 8942:"Is there an optimal preoperative management strategy for phaeochromocytoma/paraganglioma?" 7672:

Taïeb D, Hicks RJ, Hindié E, Guillet BA, Avram A, Ghedini P, et al. (September 2019).

4660:

Abourazzak S, Atmani S, Arqam LE, Chaouki S, Labib S, Harrandou M, et al. (May 2010).

4096: 3349: 2259: 2221: 1364: 1212: 1098: 966: 527: 458: 6590: 1134: 145: 83: 8: 12660: 12548: 12233: 5254: 5030: 3496: 2865: 2754: 2750: 2663: 2584: 2580: 2329: 1658: 1070: 917: 907: 801: 749: 730: 399: 11025:"Changes in clinical features and long-term prognosis in patients with pheochromocytoma" 6791: 5933:

Zhuang Z, Yang C, Lorenzo F, Merino M, Fojo T, Kebebew E, et al. (September 2012).

5478: 5451: 4100: 3865: 3709:

Lenders JW, Eisenhofer G, Mannelli M, Pacak K (20–26 August 2005). "Phaeochromocytoma".

2727:

The most common post-operative complications, likely causes, and treatment options are:

2673:

in patients treated with preoperative intravenous fluids compared to those who did not.

622:

Other clinical manifestations that have been reported include (in no particular order):

12468: 12257: 12167: 12112: 12085: 11878: 11853: 11829: 11802: 11783: 11697: 11651: 11599: 11574: 11487: 11460: 11436: 11411: 11355: 11300: 11275: 11224: 11199: 11177: 11133: 10950: 10925: 10906: 10858: 10831: 10804: 10779: 10755: 10730: 10711: 10622: 10597: 10570: 10545: 10523: 10469: 10442: 10418: 10393: 10369: 10344: 10284: 10271: 10246: 10191: 10134: 10107: 10083: 10058: 10057:

Breen W, Bancos I, Young WF, Bible KC, Laack NN, Foote RL, Hallemeier CL (2017-11-22).

10039: 9996: 9953: 9910: 9862: 9837: 9813: 9769: 9726: 9675: 9648: 9581: 9556: 9555:

Hamidi O, Young WF, Gruber L, Smestad J, Yan Q, Ponce OJ, et al. (November 2017).

9527: 9502: 9475: 9450: 9428: 9339: 9312: 9246: 9219: 9155: 9128: 9102: 9056: 9012: 8969: 8917: 8892: 8873: 8829: 8736: 8727: 8711: 8687: 8662: 8638: 8613: 8483: 8458: 8434: 8409: 8291: 8266: 8209: 8160: 8135: 8116: 8062: 8037: 8010: 7985: 7958: 7931: 7912: 7860: 7817: 7804: 7779: 7755: 7730: 7711: 7698: 7673: 7654: 7603: 7554: 7505: 7457: 7430: 7245: 7220: 7198: 7150: 7125: 7055: 7030: 7003: 6978: 6945: 6920: 6896: 6871: 6847: 6838: 6822: 6803: 6725: 6682: 6599: 6574: 6550: 6523: 6404: 6379: 6309: 6284: 6213: 6151: 6126: 6057: 6032: 6008: 5983: 5959: 5934: 5910: 5885: 5861: 5836: 5812: 5787: 5763: 5738: 5719: 5670: 5576: 5527: 5502: 5359: 5189: 5164: 5118: 4996: 4926: 4883: 4870: 4843: 4819: 4794: 4740: 4686: 4661: 4642: 4594: 4567: 4478: 4453: 4355: 4309: 4213: 4188: 4164: 4139: 4120: 4020: 3995: 3976: 3881: 3780: 3734: 3603: 2731: 2287: 2236:

scans were developed, MIBG has slowly lost its favor for the pheochromocytoma patient.

2159:

The imaging modalities discussed below are for tumor characterization, confirmation of

1597: 1216: 1055: 715: 366: 12246: 12242: 12238: 11678: 11041: 11024: 10343:

Jawed I, Velarde M, Därr R, Wolf KI, Adams K, Venkatesan AM, et al. (July 2018).

9853: 8941: 8519: 8331: 8282: 8265:

Neumann HP, Tsoy U, Bancos I, Amodru V, Walz MK, Tirosh A, et al. (August 2019).

5884:

Pacak K, Jochmanova I, Prodanov T, Yang C, Merino MJ, Fojo T, et al. (May 2013).

5690:"Pheochromocytoma and paraganglioma pathogenesis: learning from genetic heterogeneity" 5386:"Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline" 3722: 3641: 3549:) to draw attention to the least common option sometimes being the correct diagnosis. 3027:

have also indicated the utility of administering a radionuclide agent like iodine-123

1540:

than a pheochromocytoma given the relative frequency of 0.8 per 100,000 person-years.

12647: 12495: 12476: 12412: 12285: 12117: 12008:"VHL Family Alliance Applauds Grey's Anatomy for Featuring von Hippel-Lindau Disease" 11958: 11883: 11834: 11775: 11737: 11702: 11682: 11643: 11604: 11533: 11492: 11441: 11387: 11359: 11347: 11305: 11256: 11229: 11169: 11125: 11090: 11046: 10955: 10910: 10898: 10863: 10809: 10760: 10703: 10662: 10658: 10627: 10575: 10515: 10474: 10423: 10374: 10325: 10302:

Nomura K, Kimura H, Shimizu S, Kodama H, Okamoto T, Obara T, Takano K (August 2009).

10288: 10276: 10227: 10183: 10139: 10088: 10043: 10031: 9988: 9984: 9945: 9902: 9867: 9805: 9773: 9761: 9730: 9718: 9680: 9629: 9621: 9617: 9604:"Metastatic catecholamine-secreting paraganglioma (extra-adrenal pheochromocytoma)". 9586: 9532: 9518: 9480: 9420: 9385: 9344: 9293: 9251: 9200: 9160: 9106: 9094: 9048: 9044: 9004: 8961: 8922: 8865: 8821: 8777: 8741: 8692: 8643: 8594: 8559: 8523: 8488: 8439: 8387: 8335: 8296: 8244: 8201: 8165: 8108: 8067: 8015: 7963: 7904: 7864: 7852: 7809: 7778:

Janssen I, Chen CC, Millo CM, Ling A, Taieb D, Lin FI, et al. (September 2016).

7760: 7715: 7703: 7658: 7646: 7638: 7634: 7607: 7595: 7546: 7497: 7462: 7411: 7376: 7332: 7291: 7250: 7202: 7190: 7155: 7106: 7060: 7008: 6950: 6901: 6852: 6795: 6760: 6756: 6729: 6717: 6674: 6670: 6639: 6604: 6555: 6524:"Update of Pheochromocytoma Syndromes: Genetics, Biochemical Evaluation, and Imaging" 6491: 6450: 6409: 6355: 6314: 6265: 6217: 6205: 6156: 6142: 6107: 6097: 6062: 6013: 5999: 5964: 5915: 5866: 5835:

Yang C, Sun MG, Matro J, Huynh TT, Rahimpour S, Prchal JT, et al. (March 2013).

5817: 5768: 5711: 5662: 5627: 5568: 5532: 5483: 5407: 5351: 5306: 5258: 5221: 5194: 5145: 5110: 5075: 5034: 5000: 4988: 4957: 4918: 4875: 4860: 4824: 4806: 4775: 4732: 4727: 4710: 4691: 4634: 4599: 4545: 4483: 4434: 4393: 4347: 4301: 4259: 4218: 4169: 4112: 4060: 4025: 3980: 3968: 3933: 3923: 3885: 3834: 3766: 3738: 3726: 3688: 3582: 3517: 3352:(0.8 per 100,000 person-years), in a study conducted from 1950 to 1979. However, the 3101: 2887: 2802: 2775: 2716: 2681: 2666: 2424: 2356: 2345: 2337: 1977: 1480: 958: 950: 838: 703: 571: 414: 350: 11787: 11655: 11181: 11137: 10924:

Wolf KI, Jha A, van Berkel A, Wild D, Janssen I, Millo CM, et al. (June 2019).

10544:

Pryma DA, Chin BB, Noto RB, Dillon JS, Perkins S, Solnes L, et al. (May 2019).

10527: 10392:

Tena I, Gupta G, Tajahuerce M, Benavent M, Cifrián M, Falcon A, et al. (2018).

10000: 9957: 9817: 9432: 9060: 9016: 8877: 8612:

Luiz HV, Tanchee MJ, Pavlatou MG, Yu R, Nambuba J, Wolf K, et al. (July 2016).

7916: 7821: 7558: 6807: 6686: 6575:"Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment" 5723: 5674: 5580: 5363: 5054:"A case of pheochromocytoma complicated with acute renal failure and cardiomyopathy" 4887: 4744: 4646: 4313: 4124: 2067:, and weight loss secondary to the stimulation of dopamine receptors throughout the 12565: 12510: 12336: 12163: 12107: 12097: 11873: 11865: 11824: 11814: 11767: 11729: 11692: 11674: 11635: 11594: 11586: 11523: 11482: 11472: 11431: 11423: 11379: 11339: 11295: 11287: 11219: 11211: 11161: 11117: 11080: 11036: 10945: 10937: 10890: 10853: 10843: 10799: 10791: 10750: 10742: 10715: 10693: 10654: 10617: 10609: 10565: 10557: 10505: 10464: 10454: 10413: 10405: 10364: 10356: 10315: 10266: 10258: 10219: 10195: 10173: 10160:

Niemeijer ND, Alblas G, van Hulsteijn LT, Dekkers OM, Corssmit EP (November 2014).

10129: 10119: 10078: 10070: 10023: 9980: 9937: 9914: 9894: 9857: 9849: 9797: 9753: 9710: 9670: 9660: 9613: 9576: 9568: 9522: 9514: 9470: 9462: 9412: 9375: 9334: 9324: 9285: 9241: 9231: 9192: 9150: 9140: 9086: 9040: 8996: 8973: 8953: 8912: 8904: 8857: 8833: 8811: 8798:

Groeben H, Nottebaum BJ, Alesina PF, Traut A, Neumann HP, Walz MK (February 2017).

8769: 8731: 8723: 8682: 8674: 8633: 8625: 8586: 8551: 8515: 8478: 8470: 8429: 8421: 8377: 8327: 8286: 8278: 8236: 8213: 8193: 8155: 8147: 8120: 8098: 8057: 8053: 8049: 8005: 7997: 7953: 7943: 7932:"C-hydroxy-ephedrine-PET/CT in the Diagnosis of Pheochromocytoma and Paraganglioma" 7894: 7844: 7799: 7791: 7750: 7742: 7693: 7685: 7630: 7585: 7536: 7509: 7489: 7452: 7442: 7403: 7366: 7322: 7281: 7240: 7232: 7182: 7145: 7137: 7096: 7050: 7042: 6998: 6990: 6940: 6932: 6891: 6883: 6842: 6834: 6787: 6752: 6709: 6666: 6631: 6594: 6586: 6545: 6535: 6481: 6468:

de Jong WH, Eisenhofer G, Post WJ, Muskiet FA, de Vries EG, Kema IP (August 2009).

6440: 6399: 6391: 6345: 6304: 6296: 6255: 6197: 6146: 6138: 6089: 6052: 6044: 6003: 5995: 5954: 5946: 5905: 5897: 5856: 5848: 5807: 5799: 5758: 5750: 5701: 5654: 5617: 5607: 5560: 5522: 5514: 5473: 5463: 5397: 5343: 5298: 5250: 5184: 5176: 5122: 5102: 5065: 5026: 4980: 4953: 4930: 4910: 4865: 4855: 4844:"Left ventricular thrombus and embolic stroke caused by a functional paraganglioma" 4814: 4767: 4722: 4681: 4673: 4626: 4589: 4579: 4566:

Agrawal S, Shirani J, Garg L, Singh A, Longo S, Longo A, et al. (March 2017).

4537: 4473: 4465: 4424: 4385: 4359: 4339: 4293: 4249: 4208: 4200: 4159: 4151: 4104: 4052: 4015: 4007: 3960: 3877: 3824: 3758: 3718: 3618: 3546: 3542: 3183: 3089: 2969: 2787: 2757: 2608: 2488: 2476: 2467:, particularly when transferring from a seated to standing position due to a rapid 2455:." When patients are initially exposed to one of the above agents, they may become 2209:

The first functional imaging technique utilized in pheochromocytoma patients was I-

1807: 1106: 954: 846: 779: 753: 631: 280: 9466: 7124:

Histed SN, Lindenberg ML, Mena E, Turkbey B, Choyke PL, Kurdziel KA (April 2012).

6048: 12688: 12620: 12578: 12444: 12279: 11215: 11121: 10598:"MIBG (metaiodobenzylguanidine) theranostics in pediatric and adult malignancies" 9801: 9757: 9714: 8457:

Graham RM, Thornell IR, Gain JM, Bagnoli C, Oates HF, Stokes GS (November 1976).

7141: 7101: 7084: 6350: 6333: 6188:

Neumann HP, Young WF, Eng C (August 2019). "Pheochromocytoma and Paraganglioma".

5852: 5754: 5106: 4541: 4297: 3762: 3492: 3469: 3262: 3248: 3191: 3046: 2928: 2891: 2886:

Those who have lost both their adrenal glands will also require another steroid (

2858: 2456: 2279: 2202: 2001: 1904: 1831: 1789: 1740: 1570: 1438: 1114: 962: 921: 766: 607: 507: 503: 253:

Phaeochromocytoma, adrenal medullary tumor, Chromaffin Cell Tumors, Paraganglioma

12353: 12339: 12176: 10223: 6300: 5886:"New syndrome of paraganglioma and somatostatinoma associated with polycythemia" 5549: 4630: 3951:

Tevosian SG, Ghayee HK (December 2019). "Pheochromocytomas and Paragangliomas".

3370:(earlier disease onset with each generation) is associated with some mutations. 3161:

conducive to surgery) pheochromocytoma patients and the results were promising.

2680:

in Los Angeles, California, nearly all indicated the importance of preoperative

1927:

disease – which is the only current biochemical evidence of metastases to date.

618:

There is an adrenal gland, highlighted in yellow, on top of each of the kidneys.

570:). However, patients are unlikely to experience continuous symptoms. Due to the 12603: 12518: 12342: 11983:"Neuroendocrine Cancer Survivor Featured on Discovery Fit & Health TV Show" 11528: 11511: 11343: 10941: 10546:"131I-MIBG Therapy in Patients with Advanced Pheochromocytoma or Paraganglioma" 10074: 10027: 9380: 9363: 9289: 9196: 8908: 8861: 8590: 8555: 8151: 7848: 7689: 7286: 7269: 6635: 5468: 4771: 4469: 3521: 3504: 3457: 3448: 3206:, are common, but often minimal, and can be mitigated with slow, steady dosing. 3175: 3034: 2955: 2900: 2868: 2854: 2836: 2720: 2670: 2341: 2217: 2140: 2132: 1997: 1868: 1612: 913: 816: 805: 793: 775: 726: 580: 428: 424: 12365: 12290: 12223: 12219: 12102: 11733: 11291: 10561: 10360: 9941: 9898: 9665: 9416: 9329: 9129:"Postoperative Management in Patients with Pheochromocytoma and Paraganglioma" 8577:

Clark BK (May 1992). "Beta-adrenergic blocking agents: their current status".

8240: 8197: 7795: 7746: 6994: 6031:

Toledo RA, Qin Y, Cheng ZM, Gao Q, Iwata S, Silva GM, et al. (May 2016).

5786:

Taïeb D, Yang C, Delenne B, Zhuang Z, Barlier A, Sebag F, Pacak K (May 2013).

5518: 5302: 5213: 4842:

Buchbinder NA, Yu R, Rosenbloom BE, Sherman CT, Silberman AW (December 2009).

4795:"Pheochromocytoma Underlying Hypertension, Stroke, and Dilated Cardiomyopathy" 4343: 4204: 4011: 3964: 3937: 3422: 2407:

was no decreased survival compared to their total adrenalectomy counterparts.

1772:

of this test is 97% and 93% respectively; however, there is still concern for

1318:

Pheochromocytoma, head and neck paraganglioma, gastrointestinal stromal tumor

1297:

Head and neck paraganglioma, pheochromocytoma, gastrointestinal stromal tumor

1276:

Pheochromocytoma, head and neck paraganglioma, gastrointestinal stromal tumor

12736: 12637: 12630: 12595: 12583: 11962: 11819: 11647: 11427: 11391: 11383: 10746: 10459: 10409: 9625: 8678: 8316:"Cortical-sparing adrenalectomy for patients with bilateral pheochromocytoma" 7642: 7447: 6540: 5935:"Somatic HIF2A gain-of-function mutations in paraganglioma with polycythemia" 5901: 4984: 4810: 4677: 3889: 3829: 3812: 3613: 3484: 3473: 3336: 3290: 3244: 3171: 3162: 3110: 3105: 3075: 2997: 2980:

Metastatic pheochromocytoma is best managed with a multidisciplinary team of

2904: 2794: 2771: 2697: 2641: 2604: 2592: 2532: 2428: 2416: 2383: 2360: 2352: 2333: 2113: 2105: 2018:) compared to those that are classically episode with an adrenergic phenotype 1966: 1761: 1575: 978: 685: 567: 515: 511: 470: 420: 355: 11412:"Pheochromocytoma and Paraganglioma: From Epidemiology to Clinical Findings" 11198:

Chen H, Sippel RS, O'Dorisio MS, Vinik AI, Lloyd RV, Pacak K (August 2010).

10848: 10124: 9236: 9145: 8474: 8103: 8087:"Surgery for pheochromocytoma: A 20-year experience of a single institution" 8086: 7948: 6887: 5658: 4155: 4108: 3238:

analogues mentioned above in the functional imaging section. Treatment with

12675: 12541: 12480: 12121: 11887: 11869: 11838: 11771: 11741: 11706: 11628:

Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin

11608: 11573:

Bausch B, Tischler AS, Schmid KW, Leijon H, Eng C, Neumann HP (July 2017).

11537: 11496: 11477: 11445: 11351: 11309: 11233: 11173: 11129: 11094: 11050: 10959: 10902: 10867: 10813: 10764: 10707: 10666: 10631: 10579: 10519: 10478: 10427: 10378: 10329: 10280: 10187: 10143: 10092: 10035: 9992: 9949: 9906: 9871: 9809: 9765: 9722: 9684: 9590: 9536: 9484: 9424: 9389: 9348: 9297: 9255: 9204: 9164: 9098: 9090: 9052: 9008: 8965: 8926: 8869: 8825: 8781: 8647: 8563: 8443: 8391: 8300: 8248: 8205: 8169: 8112: 8071: 8019: 7967: 7908: 7856: 7813: 7764: 7707: 7650: 7599: 7550: 7501: 7466: 7415: 7380: 7336: 7295: 7254: 7236: 7194: 7159: 7110: 7064: 7012: 6954: 6905: 6721: 6713: 6678: 6643: 6608: 6559: 6495: 6454: 6413: 6380:"Drugs and pheochromocytoma—don't be fooled by every elevated metanephrine" 6359: 6318: 6269: 6260: 6243: 6209: 6160: 6111: 6066: 6017: 5968: 5919: 5870: 5821: 5772: 5715: 5666: 5631: 5572: 5564: 5536: 5487: 5411: 5355: 5310: 5114: 4992: 4922: 4914: 4879: 4828: 4779: 4736: 4695: 4638: 4603: 4549: 4487: 4438: 4397: 4389: 4351: 4305: 4263: 4222: 4173: 4116: 4064: 4029: 3972: 3838: 3730: 3574: 3465: 3440: 3388:

10% of patients have bilateral (both left and right adrenal glands) disease

3362: 3137: 3085: 3067: 3015: 2840: 2824: 2820: 2808: 2761: 2742: 2738: 2654: 2624: 2616: 2612: 2600: 2576: 2567: 2536: 2528: 2512: 2472: 2369: 2213: 2011: 1986: 1959: 1896: 1880: 1803: 1622: 1432: 1138: 1130: 986: 938: 842: 737: 674: 656: 563: 555: 519: 450: 371: 11779: 11590: 11260: 11165: 11085: 11068: 10795: 10698: 10681: 10231: 9647:

Engstrand J, Strömberg C, Nilsson H, Freedman J, Jonas E (December 2019).

8745: 8696: 8598: 8339: 7729:

Jha A, Ling A, Millo C, Gupta G, Viana B, Lin FI, et al. (May 2018).

7186: 6764: 6334:"Is supine rest necessary before blood sampling for plasma metanephrines?" 6201: 5950: 5262: 5198: 5149: 5079: 5038: 4584: 3692: 3483:, Henri Alezais and Felix Peyron, introduced the scientific community to " 2723:

on the resected tumor confirms the diagnosis, by typical features as shown

614: 12693: 12500: 12426: 12320: 12182: 12172: 11066: 10613: 10596:

Agrawal A, Rangarajan V, Shah S, Puranik A, Purandare N (November 2018).

10320: 10303: 9633: 8816: 8799: 8773: 8527: 8492: 8425: 8382: 8365: 6856: 6799: 6486: 6469: 6445: 6428: 6395: 5803: 5622: 5402: 5385: 5347: 3500: 3488: 3461: 3353: 3331: 3223:

Same patient. Purple lesions are metastatic disease detected with FDG PET

3187: 3097: 3093: 3054: 2962: 2890:

replacement). The typical daily dose is between 50 and 200 micrograms of

2767: 2705: 2693: 2658: 2637: 2596: 2572: 2548: 2524: 2508: 2480: 2349: 2252: 2220:(secreted by pheochromocytomas), MIBG was well-suited for uptake by most 2015: 1913: 1827: 1261: 957:. Given the high association with genetic inheritance, the United States 889: 885: 787: 642: 602: 559: 523: 375: 11758:

Welbourn RB (July 1987). "Early surgical history of phaeochromocytoma".

11673:. Progress in Brain Research. Vol. 182. Elsevier. pp. 343–73. 11459:

Antonio K, Valdez MM, Mercado-Asis L, Taïeb D, Pacak K (February 2020).

8663:"The dangers of unopposed beta-adrenergic blockade in phaeochromocytoma" 8001: 7407: 7371: 7354: 7046: 6936: 6621: 5612: 5595: 4429: 4412: 4254: 4237: 3439:

distinctly marked on each cheek" with a "constant profuse and universal

2623:

started on a beta-adrenoceptor antagonist, this reverses the protective

2186: 1935: 12453: 12440: 12435: 12422: 12348: 11639: 10262: 9496: 9494: 7493: 6823:"Clonidine withdrawal. Mechanism and frequency of rebound hypertension" 5180: 4568:"Pheochromocytoma and stress cardiomyopathy: Insight into pathogenesis" 4056: 3358: 3319: 2932: 2924: 2689: 2556: 2544: 2504: 2484: 2420: 2390: 2378: 2374: 2267: 2210: 2190: 2176: 2160: 2147: 1955: 1924: 1892: 1823: 1653: 1602: 1358: 1346: 1341: 1191: 1186: 1034: 1029: 990: 833: 596: 465: 148:

if you can. Unsourced or poorly sourced material may be challenged and

12202: 10894: 10510: 10493: 10178: 10161: 9927: 9572: 9000: 8957: 8629: 7899: 7882: 7590: 7573: 7541: 7524: 7523:

van Hulsteijn LT, Niemeijer ND, Dekkers OM, Corssmit EP (April 2014).

6872:"Hypertensive urgency: an important aetiology of rebound hypertension" 6656: 6093: 5070: 5053: 3219:

Purple lesions are metastatic disease detected with DOTATATE imaging.

3212: 993:, parent, and child) where the primary focus is supporting the child. 12683: 12431: 12331: 12296: 11951:"Why Did the Young Mother Have Searing Head Pain and a Racing Heart?" 11669:

Kantorovich V, Pacak K (2010). "Pheochromocytoma and paraganglioma".

9278:

Best Practice & Research. Clinical Endocrinology & Metabolism

7522: 7327: 7310: 5379: 5377: 5375: 5373: 4711:"Cerebrovascular event, dilated cardiomyopathy, and pheochromocytoma" 3595: 3480: 3281: 3235: 3011: 2989: 2832: 2552: 2460: 2448: 2440: 2180: 2128: 2117: 1876: 1718: 1644: 1590: 1074: 722: 711: 707: 576: 518:

into the bloodstream which cause the most common symptoms, including

479: 473: 454: 11623: 10679: 9491: 6427:

de Jong WH, Post WJ, Kerstens MN, de Vries EG, Kema IP (June 2010).

5706: 5689: 5383: 4283: 2270:

disease, particularly related to succinate dehydrogenase subunit B (

2239: 1919:

Plasma methoxytyramine is a breakdown product of the catecholamine,

765:

A heart attack is often caused by a significant build-up of plaque (

12404: 10159: 9786: 7930:

Vyakaranam AR, Crona J, Norlén O, Hellman P, Sundin A (June 2019).

6979:"Update on Modern Management of Pheochromocytoma and Paraganglioma" 3608: 3339:. In the first national, epidemiological population-based study in 3199: 2981: 2540: 2511:, but it will assist the surgical and anesthesia teams if there is 2444: 2225: 2201:, while the darkened structure in the pelvis of the patient is the 2131:, MR is preferred in children and pregnant women. Furthermore, the 2064: 2060: 2052:

More likely to indicate an extra-adrenal tumor of the head and neck

2046: 1944: 1920: 1888: 1839: 1632: 1580: 982: 893: 874: 809: 651: 592: 584: 551: 476: 436: 383: 379: 359: 12301: 11901: 8314:

Lee JE, Curley SA, Gagel RF, Evans DB, Hickey RC (December 1996).

7082: 5370: 4235: 3638:"PHAEOCHROMOCYTOMA | Meaning & Definition for UK English" 3318:

According to the North American Neuroendocrine Tumor Society, the

2348:

2014 Clinical Practice Guideline for pheochromocytoma recommend a

12034:"Mischaracterizations by the popular media of medical conditions" 11372: 11322: 9646: 8846: 7353:

Castinetti F, Kroiss A, Kumar R, Pacak K, Taieb D (August 2015).

6241: 4189:"Hypertension in pheochromocytoma: characteristics and treatment" 4042: 3996:"Hypertension in pheochromocytoma: characteristics and treatment" 3472:," allowing pathologists to recognize tumors that arose from the 3453: 3444: 3431: 3407: 3327: 3179: 2985: 2850: 2816: 2798: 2685: 2301: 2198: 2194: 2097: 1900: 1843: 1585: 1447: 1443: 1381: 670: 664:– becoming light-headed or dizzy after swiftly changing positions 446: 12268: 12058:"'Grey's Anatomy' Features Rare Disease on Three-Episode Series" 8540: 7620: 5327:

Mercado-Asis LB, Wolf KI, Jochmanova I, Taïeb D (January 2018).

4709:

Dagartzikas MI, Sprague K, Carter G, Tobias JD (February 2002).

3813:"Biochemical diagnosis of pheochromocytoma: which test is best?" 1544:

disease, and multiparameter scoring systems have been proposed

12458: 12449: 12214: 11509: 10777: 9029: 5326: 5240: 4140:"Phaeochromocytoma--"the great mimic": an unusual presentation" 3286: 3195: 2812: 2782:

may be needed if the blood pressure does not respond to fluids.

2464: 2136: 2056: 1884: 1607: 1240: 881: 860: 678: 647: 626: 387: 11458: 11150: 10105: 9126: 7267: 6331: 6283:

Griffin TP, Casey R, Wall D, Bell M, O'Shea PM (August 2016).

5452:"The clinical genetics of phaeochromocytoma and paraganglioma" 5135: 4970: 4943: 4708: 3708: 2676:

In a 2010 survey of 40 endocrinologists by researchers at the

546:

The symptoms of a sympathetic pheochromocytoma are related to

289: 10829: 10644: 10491: 8940:

Challis BG, Casey RT, Simpson HL, Gurnell M (February 2017).

8226: 8035: 7431:"Anatomical and functional imaging in endocrine hypertension" 5981: 4841: 3532: 3344: 3307: 3234:

The newest of the treatment options, PRRT utilizes the 68-Ga

3167: 3057:

retrospectively reviewed all of their patients who underwent

2940: 2615:. However, when catecholamines active the beta receptor, the 2216:. Given the compounds similar structure to the catecholamine 1467: 1425: 499: 12137:

General Information About Pheochromocytoma and Paraganglioma

11925: 11197: 10974:"NCI Dictionary of Cancer Terms – National Cancer Institute" 10595: 10208: 9503:"Metastatic pheochromocytoma: does the size and age matter?" 8797: 7986:"Precision Surgery for Pheochromocytomas and Paragangliomas" 7929: 7479: 6467: 4659: 3361:

for why the incidence of pheochromocytoma is growing in the

2927:

pheochromocytoma is defined as the presence of tumor cells (

2495:, all of which do not cease with time and may limit patient 2292:

F-FDOPA has become the preferred radiopharmaceutical agent.

16:

Tumor of the adrenal medulla which secretes adrenal hormones

11022: 10391: 9362:

Shen WT, Lee J, Kebebew E, Clark OH, Duh QY (August 2006).

8758: 8709: 7123: 6426: 5883: 5449: 5445: 5443: 4238:"Life-threatening events in patients with pheochromocytoma" 3443:." Following his death, a group of physicians performed an 3340: 3303: 3299: 3129:(time until tumor returned) with continued administration. 2944: 2936: 2475:, the non-selective alpha-1 and 2 adrenoceptor antagonist ( 2272: 2197:. The darkened structure at the head of the patient is the 2035: 1306: 1285: 1206: 334: 328: 310: 304: 292: 12083: 11572: 9835: 8939: 8084: 7834: 7784:

European Journal of Nuclear Medicine and Molecular Imaging

7735:

European Journal of Nuclear Medicine and Molecular Imaging

7678:

European Journal of Nuclear Medicine and Molecular Imaging

7352: 7309:

Caraiani C, Dong Y, Rudd AG, Dietrich CF (December 2018).

5644: 4757: 4662:"Cerebral ischaemic stroke and bilateral pheochromocytoma" 3810: 3391:

10% of patients have extra-adrenal (paraganglioma) disease

566:(excessive sweating, particularly at night, also known as 11671:

Neuroendocrinology - Pathological Situations and Diseases

10728: 10301: 10013: 9500: 8456: 8366:"Preoperative management of the pheochromocytoma patient" 7311:"Reasons for inadequate or incomplete imaging techniques" 6778:

England JF (May 1977). "Clonidine rebound hypertension".

5739:"Genomic Landscape of Pheochromocytoma and Paraganglioma" 5500: 4900: 432: 319: 316: 11154:

Nature Clinical Practice. Endocrinology & Metabolism

10244: 9554: 9311:

Dungan K, Merrill J, Long C, Binkley P (November 2019).

9310: 8182: 7308: 6918: 5593: 5440: 4413:"Pheochromocytoma and pregnancy: a deceptive connection" 2171:), commonly referred to as the PET scan, (2) iodine-123 796:

damage, excessive catecholamines can result in abnormal

10880: 8264: 7837:

Revista Espanola de Medicina Nuclear e Imagen Molecular

7671: 6820: 6699: 6237: 6235: 6233: 6231: 6229: 6227: 5932: 5218:

Surgical Treatment: Evidence-Based and Problem-Oriented

4565: 3678: 3660: 3658: 1780:

medications the patient is taking, and (3) their diet.

11800: 11110:

Current Opinion in Endocrinology, Diabetes and Obesity

9884: 9079:

Experimental and Clinical Endocrinology & Diabetes

7574:"Role of (18) F-FDOPA PET/CT imaging in endocrinology" 5785: 5092: 3594:

mutation that has resulted in a pheochromocytoma. The

1976:

Patients are more likely to present with the classic,

595:(cheese and wine), intraoperative tumor manipulation, 10923: 10155: 10153: 10056: 9743: 8611: 7126:"Review of functional/anatomical imaging in oncology" 7078: 7076: 7074: 6521: 4193:

Endocrinology and Metabolism Clinics of North America

4186: 4000:

Endocrinology and Metabolism Clinics of North America

3993: 3953:

Endocrinology and Metabolism Clinics of North America

3302:

lesions (bone) faring better than their soft-tissue (

1548:

Differential Diagnosis of Pheochromocytoma by System

313: 298: 295: 286: 283: 12192: 11854:""If You Hear Hoof Beats, Think Horses, Not Zebras"" 11073: