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Current Treatment Options in Oncology Aug 2003Pheochromocytoma is a rare tumor, but it represents a potentially curable form of hypertension. In patients with inherited pheochromocytoma, benign and bilateral tumors...
Pheochromocytoma is a rare tumor, but it represents a potentially curable form of hypertension. In patients with inherited pheochromocytoma, benign and bilateral tumors are more common. The diagnosis of pheochromocytoma rests in biochemical confirmation of catecholamine excess. Plasma-free metanephrine levels are arguably the most sensitive and specific test for the biochemical diagnosis of pheochromocytoma in high-risk patient populations. A timed 24-hour urine collection for total catecholamines and metabolic products (eg, vanillylmandelic acid and metanephrines) is the favored confirmatory test. Localization is most commonly accomplished with high-resolution computed tomography imaging, but magnetic resonance imaging can also be used. If both of these imaging modalities are nonlocalizing or equivocal, then radiolabeled meta-iodobenzylguanidine or somatostatin can be used to identify an adrenal or extra-adrenal tumor (paraganglioma). These imaging modalities can be used in the evaluation of patients with suspected or confirmed recurrent or metastatic disease. Systemic therapies for the treatment of patients with recurrent or metastatic disease have been disappointing. Radiation therapy is best applied for palliative relief of pain associated with bony metastases. In the absence of radiographic evidence for local tumor invasion, laparoscopic resection of small- to medium-sized (< 6 cm) pheochromocytomas is indicated. Abundant evidence indicates that this approach is safe and well tolerated and results in more rapid recovery and less long-term wound morbidity compared to open anterior or posterior adrenalectomy. Open anterior adrenalectomy is appropriate for patients with large or recurrent tumors, suspected or documented locoregional invasion, or for those patients in whom a laparoscopic approach is technically contraindicated. For selected patients with pheochromocytoma in the von Hipple-Lindau syndrome or multiple endocrine neoplasia type 2 setting in which the cumulative incidence of clinical bilateral tumors is high, a cortical-sparing approach may minimize the risk of Addisonian complications.
Topics: Humans; Pheochromocytoma
PubMed: 12943613
DOI: 10.1007/s11864-003-0008-9 -
Archives de Pediatrie : Organe Officiel... Dec 1997Pheochromocytoma is a rare tumor in children which explains, together with its miscellaneous symptomatology, why the diagnosis may be delayed. The localization of the... (Review)
Review
Pheochromocytoma is a rare tumor in children which explains, together with its miscellaneous symptomatology, why the diagnosis may be delayed. The localization of the tumor(s) rests mainly on MIBG scintigraphy, and CT scan and/or magnetic resonance imaging. Thanks to a systematic preoperative treatment of hypertension and major progress in anesthesia, the operative mortality of pheochromocytomas in children is nowadays very low. Nevertheless two major problems remain: 1) the difficulty of diagnosing and treating the malignant forms, 2) the high frequency of recurrences, sometimes many years after the removal of the primary tumor. A regular long term supervision is therefore necessary after the surgical treatment.
Topics: Adrenal Gland Neoplasms; Age Factors; Anesthesia, General; Child; Humans; Male; Pheochromocytoma; Preoperative Care; Prognosis; Recurrence
PubMed: 9538427
DOI: 10.1016/s0929-693x(97)82613-9 -
AJR. American Journal of Roentgenology Feb 2013Pheochromocytomas are relatively rare neuroendocrine tumors of the adrenal medulla. Their variable clinical presentation and biologic behavior often make accurate... (Review)
Review
OBJECTIVE
Pheochromocytomas are relatively rare neuroendocrine tumors of the adrenal medulla. Their variable clinical presentation and biologic behavior often make accurate diagnosis challenging. A variable spectrum of imaging appearances--some of which may also mimic other diseases--has been recognized. This article reviews the epidemiology; associations; and clinical, biochemical, pathologic, and multimodality imaging features of pheochromocytomas including diagnostic pearls and pitfalls.
CONCLUSION
Pheochromocytomas are often considered the great mimicker of other adrenal tumors. Because of their varied clinical, imaging, and pathologic appearances, accurate diagnosis can be challenging. The various imaging appearances on ultrasound, CT, MRI, and functional imaging can be complementary and have features that are useful for differentiating pheochromocytoma from other lesions of the adrenal.
Topics: Adrenal Gland Neoplasms; Contrast Media; Diagnosis, Differential; Diagnostic Imaging; Humans; Neoplasm Staging; Pheochromocytoma; Prevalence
PubMed: 23345359
DOI: 10.2214/AJR.12.9126 -
Hormone and Metabolic Research =... Sep 2009The treacherous and deceptive nature of pheochromocytoma makes it crucial to detect and treat it promptly; otherwise it will almost certainly be fatal from... (Review)
Review
The treacherous and deceptive nature of pheochromocytoma makes it crucial to detect and treat it promptly; otherwise it will almost certainly be fatal from cardiovascular complications or metastases. Hypertension occurring in patients with pheochromocytomas is sustained in about 50% and paroxysmal in the remainder; however, many patients remain normotensive. Hypertension attacks may be precipitated by physical activity, postural changes, anxiety, certain foods or wine, some drugs, operative procedures, etc. Cardinal manifestations are paroxysmal hypertension, headache, palpitations +/- tachycardia, inappropriate sweating; anxiety, tremulousness, pallor (rarely flushing), chest and abdominal pains; nausea and vomiting often occur. Hypercatecholaminemia manifestations are more common and pronounced when paroxysmal hypertension occurs, but persons with familial pheochromocytoma may be asymptomatic. Protean manifestations of pheochromocytoma may simulate many conditions, some of which may have elevated plasma and urine catecholamines and their metabolites. Baro-reflex failure, postural tachycardia syndrome, sleep apnea, carcinoid, renal failure, and pseudopheochromocytoma may be diagnostic challenges. The history, physical examination, biochemical testing (after eliminating interfering drugs, when possible) for plasma and urinary metanephrines can usually establish or exclude presence of pheochromocytomas. Occasionally a clonidine suppression test is needed to differentiate neurogenic from pheochromocytic hypertension. Manifestations suggesting hypercatecholaminemia without hypertension are highly atypical of pheochromocytoma. Pheochromocytoma may present as panic attacks, pre-eclampsia, cardiomyopathy, infection with fever and leucocytosis, diabetes, migraine, shock, Cushing's syndrome, multiple organ failure with lactic acidosis, neurological manifestations, transitory electrocardiogram abnormalities, constipation, intestinal obstruction, visual impairment, convulsions, etc. The key to diagnosis is always to think of pheochromocytoma in the differential diagnosis of hypertension.
Topics: Adrenal Gland Neoplasms; Humans; Pheochromocytoma
PubMed: 19242899
DOI: 10.1055/s-0028-1128139 -
Orvosi Hetilap Oct 2003
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Medicina (Kaunas, Lithuania) Jun 2020Pheochromocytomas are rare neuroendocrine tumors that can affect people of all ages and are commonly diagnosed in the 4th and 5th decades of life. Familial...
Pheochromocytomas are rare neuroendocrine tumors that can affect people of all ages and are commonly diagnosed in the 4th and 5th decades of life. Familial pheochromocytomas are diagnosed mostly between the 2nd and 3rd decades of life. They can be benign or metastatic and often present as isolated tumors or along with other neuroendocrine syndromes. We present a case of an elderly man who underwent laparoscopic adrenalectomy for pheochromocytoma at the age of 60 years but developed recurrence of metastatic pheochromocytoma after ten years. We also conducted a literature review to understand the epidemiology and presentation of the tumor and to emphasize that there should be a low threshold of suspicion for timely diagnosis and management of recurrent pheochromocytoma.
Topics: Aged; Humans; Intensive Care Units; Male; Pancreatic Neoplasms; Pheochromocytoma; Stroke
PubMed: 32604789
DOI: 10.3390/medicina56060316 -
The Nursing Clinics of North America Mar 2007Pheochromocytomas are catecholamine-secreting tumors arising from chromaffin cells of the sympathoadrenal system, which includes the adrenal medulla and sympathetic... (Review)
Review
Pheochromocytomas are catecholamine-secreting tumors arising from chromaffin cells of the sympathoadrenal system, which includes the adrenal medulla and sympathetic ganglionic tissue. The effects of catecholamine excess cause potentially fatal symptomologies and end-organ damage if not diagnosed and treated. If diagnosed and removed surgically, most of these patients can be cures. Pheochromocytomas are rare and affect from two to eight per million people. There are 800 deaths in the United States annually as a result of complications. Of patients who have pheochromocytomas diagnosed at autopsy, 75% died suddenly from myocardial infarction or cerebral vascular catastrophe. Challenges in diagnosis, tumor location, and treatment are considerable.
Topics: Adrenal Gland Neoplasms; Adult; Humans; Male; Nursing Process; Pheochromocytoma
PubMed: 17270594
DOI: 10.1016/j.cnur.2006.11.005 -
Southern Medical Journal Oct 2004Pheochromocytomas are rare chromaffin cell tumors, 90% of which arise from the adrenal glands. Pheochromocytomas presenting with true myocardial infarction are even more... (Review)
Review
Pheochromocytomas are rare chromaffin cell tumors, 90% of which arise from the adrenal glands. Pheochromocytomas presenting with true myocardial infarction are even more rare. We report a 76-year-old man who had a previously undiagnosed pheochromocytoma, and presented with the uncommon complication of myocardial infarction. Our high-risk patient was managed with the combination of simultaneous coronary artery bypass grafting and adrenalectomy.
Topics: Adrenalectomy; Aged; Coronary Artery Bypass; Humans; Male; Myocardial Infarction; Pheochromocytoma; Treatment Outcome
PubMed: 15558925
DOI: 10.1097/01.SMJ.0000141310.27872.84 -
Urology Jul 2005In adults, 10% of pheochromocytomas are extra-adrenal. Most extra-adrenal pheochromocytomas occur in the organ of Zuckerkandl. Occasionally, pheochromocytomas can arise...
In adults, 10% of pheochromocytomas are extra-adrenal. Most extra-adrenal pheochromocytomas occur in the organ of Zuckerkandl. Occasionally, pheochromocytomas can arise from paraganglion chromaffin cells in other locations. We present the case of a 63-year-old hypertensive man with a periprostatic pheochromocytoma. Our patient underwent surgical extirpation of the pheochromocytoma, while sparing the prostate from resection. His blood pressure has remained well controlled postoperatively without the addition of antihypertensive medications.
Topics: Humans; Male; Middle Aged; Pheochromocytoma; Prostatic Neoplasms
PubMed: 15961143
DOI: 10.1016/j.urology.2005.01.012 -
Endocrine Practice : Official Journal... Apr 2015Pheochromocytomas are complex tumors that require a comprehensive and systematic management plan orchestrated by a multidisciplinary team. (Review)
Review
OBJECTIVE
Pheochromocytomas are complex tumors that require a comprehensive and systematic management plan orchestrated by a multidisciplinary team.
METHODS
To achieve these ends, The Mount Sinai Adrenal Center hosted an interdisciplinary retreat where experts in adrenal disorders assembled with the aim of developing a clinical pathway for the management of pheochromocytomas.
RESULTS
The result was a consensus for the diagnosis, perioperative management, and postoperative management of pheochromocytomas, with specific recommendations from our team of adrenal experts, as well as a review of the current literature.
CONCLUSION
Our clinical pathway can be applied by other institutions directly or may serve as a guide for institution-specific management.
Topics: Adrenal Gland Neoplasms; Critical Pathways; Humans; Pheochromocytoma
PubMed: 25297659
DOI: 10.4158/EP14036.RA