Pheochromocytoma

A usually benign, well-encapsulated, lobular, vascular tumor of chromaffin tissue of the ADRENAL MEDULLA or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of EPINEPHRINE and NOREPINEPHRINE, is HYPERTENSION, which may be persistent or intermittent. During severe attacks, there may be HEADACHE; SWEATING, palpitation, apprehension, TREMOR; PALLOR or FLUSHING of the face, NAUSEA and VOMITING, pain in the CHEST and ABDOMEN, and paresthesias of the extremities. The incidence of malignancy is as low as 5% but the pathologic distinction between benign and malignant pheochromocytomas is not clear. (Dorland, 27th ed; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1298)

Also Known As:

Networked: 6748 relevant articles (170 outcomes, 660 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Paraganglioma (Paragangliomas)
3.	Cushing Syndrome
4.	Hypertension (High Blood Pressure)
5.	Neuroblastoma

Experts

1.	Pacak, Karel: 83 articles (12/2015 - 03/2002)
2.	Eisenhofer, Graeme: 51 articles (06/2015 - 05/2002)
3.	Tischler, Arthur S: 23 articles (06/2015 - 07/2002)
4.	Plouin, Pierre-François: 21 articles (04/2014 - 10/2002)
5.	Gimenez-Roqueplo, Anne-Paule: 20 articles (01/2016 - 10/2004)
6.	Pacak, K: 19 articles (05/2013 - 02/2001)
7.	Takekoshi, Kazuhiro: 19 articles (01/2013 - 03/2002)
8.	Kawakami, Yasushi: 18 articles (01/2013 - 03/2002)
9.	Lenders, Jacques W M: 17 articles (11/2015 - 06/2002)
10.	Linehan, W Marston: 17 articles (01/2014 - 05/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pheochromocytoma:

1.	3-Iodobenzylguanidine (Iobenguane)IBA 09/01/2013 - "I-MIBG hybrid SPECT-CT shows high sensitivity and specificity for characterizing adrenal lesions in patients with clinical or biochemical suspicion of pheochromocytoma and is superior to PS and SPECT alone. " 10/01/2005 - "Promising results have been published on single high-dosage [131I]metaiodobenzylguanidine ([131I]MIBG) treatment for malignant pheochromocytoma. " 07/01/1999 - "After surgical removal of the pheochromocytoma, cardiac MIBG uptake and the heart:mediastinum uptake ratios improved significantly (197% +/- 20%, P < 0.05) in all the patients. " 04/01/1985 - "Rapid clearance of iodine-131 MIBG from the heart and liver of patients with adrenergic dysfunction and pheochromocytoma." 04/01/2007 - "[(18)F]DA PET was found to show more promising results when compared with [(123/131)I]MIBG scintigraphy in the diagnostic localization of VHL-related adrenal pheochromocytoma, with a 100% rate of localization. "
2.	Hydrogen Peroxide (Hydroperoxide)FDA LinkGeneric 04/25/2007 - "We investigated its potential protective effects on hydrogen peroxide-induced oxidative stress and apoptotic death in cultured rat pheochromocytoma (PC12) cells. " 01/26/2005 - "The protective effects of EPS2 on peroxide hydrogen (H2O2)-induced cell lesion, level of lipid peroxidation, antioxidant enzyme activities were investigated in the rat pheochromocytoma line PC12 cells. " 09/01/2013 - "This study was designed to explore free radical scavenging potential of ICD in different in vitro systems and its protective role in hydrogen peroxide (H₂O₂)-induced oxidative stress and apoptotic death in cultured rat pheochromocytoma (PC12) cells. " 02/01/2008 - "In the present study, using a rat pheochromocytoma (PC12) cell line, the effect of DSS on hydrogen peroxide (H2O2) induced apoptosis was studied. " 06/01/2003 - "This study was designed to isolate new genes related to apoptosis in rat pheochromocytoma (PC12) cells treated with hydrogen peroxide (H2O2), and to characterize the roles of the genes using both in vitro and in vivo models of oxidative injury. "
3.	CatecholaminesIBA 08/01/1980 - "The authors describe a sensitive radioenzymatic technique for determining blood catecholamines which is very effective in localizing occult functioning pheochromocytomas. " 09/01/2009 - "Measurement of metanephrines instead of catecholamines by either technique improved the diagnostic accuracy for the diagnosis of pheochromocytomas. " 03/01/1995 - "Availability of biochemical assays for catecholamines and their metabolites has improved our ability to establish or exclude the diagnosis of pheochromocytoma in a suspected patient. " 04/01/2014 - "The abdominal and pelvic imaging study showed a 10-cm left adrenal mass, predominantly cystic, compatible with pheochromocytoma, which was confirmed after biochemical tests (increased urine metanephrines and plasma catecholamines). " 08/01/2006 - "Several studies have demonstrated that metanephrines, which are the metabolites of catecholamines, are reliable parameters for the diagnosis of pheochromocytoma. "
4.	Norepinephrine (Noradrenaline)FDA LinkGeneric 11/01/1985 - "Infusion of norepinephrine decreased both beta 1- and beta 2-receptor number, but was more effective for the beta 1-receptors; this result was somewhat at variance with that we previously reported for rats bearing transplanted pheochromocytomas. " 01/01/1989 - "The plasma concentration of free norepinephrine in peripheral veins is helpful in establishing the presence or absence of pheochromocytoma. " 01/01/2008 - "This study demonstrated a strong correlation between low frequency HRV, plasma norepinephrine, arterial blood pressure, and heart rate during pheochromocytoma surgery." 06/01/1992 - "All studies confirm that a nonstressed plasma norepinephrine of more than 2000 pg/mL is diagnostic of pheochromocytoma. " 10/20/1983 - "Endocrinological studies confirming the presence of a pheochromocytoma were as follows: 1) Plasma noradrenaline level was significantly elevated to 1750 pg/ml. 2) Urinary catecholamine and their metabolites (Metanephrines and VMA) were markedly elevated. "
5.	MetanephrineIBA 11/30/2004 - "Pheochromocytomas, with their protean clinical manifestations, may be diagnostic challenges, but assay of metanephrines, especially plasma metanephrine, can be particularly helpful in diagnosis. " 01/01/2012 - "A CT scan of her abdomen revealed an adrenal mass and serum metanephrine studies confirmed the diagnosis of pheochromocytoma. " 11/01/2010 - "Further investigation of catecholamine and metanephrine concentrations in dogs with pheochromocytomas is warranted to fully evaluate this test as a diagnostic tool; however, the findings of this study suggest that the results may be difficult to interpret in dogs with concurrent illness." 01/01/2015 - "The authors present a rare case of secondary HT due to a pheochromocytoma in a 15-year-old patient, whose metanephrine assay confirmed the diagnosis, and abdominal ultrasound and CT localised the tumour in the adrenal gland. " 11/01/2014 - "To improve the cost efficiency of the imaging evaluation of clinically suspected pheochromocytoma by using 24-hour fractionated urine metanephrine (FUM) results. "
6.	AldosteroneIBA 10/01/1961 - "[Pheochromocytoma in a young subject with paroxysmal cutaneous manifestations of allergic type and increased documentation of the histaminic equivalent of the blood and of aldosterone]." 11/01/2014 - "There were 48 patients with pheochromocytoma (PHE) and 46 patients with aldosterone-producing adenoma (APA). " 08/01/2010 - "Case report: Coexistence of pheochromocytoma and bilateral aldosterone-producing adenomas in a 36-year-old woman." 11/01/2009 - "Thirty-eight patients with confirmed CPA were compared with 18 healthy subjects as well as 48 control patients suffering from aldosterone-producing adenomas (n=13), pheochromocytomas (n=16), or nonfunctioning adenomas (n=19). " 01/01/2009 - "Coexistence of aldosterone-producing adrenocortical adenoma and pheochromocytoma in an ipsilateral adrenal gland."
7.	NormetanephrineIBA 03/01/2014 - "Seasonal variation in plasma free normetanephrine concentrations: implications for biochemical diagnosis of pheochromocytoma." 01/01/2014 - "The workup included an endocrinological examination, which revealed a moderately elevated urine normetanephrine level, suggesting the possibility of a pheochromocytoma. " 12/01/2011 - "In two patients where pheochromocytoma was excluded, plasma metanephrin or normetanephrine was above the reference level, corresponding to a specificity of 99%. " 10/01/2011 - "She was found to have elevated urinary normetanephrine levels; radiological imaging revealed bilateral pheochromocytomas with a pancreatic lesion. " 05/01/2004 - "Consistently elevated plasma levels of free normetanephrine provided the first and only biochemical evidence for a pheochromocytoma in this case. "
8.	Dopamine (Intropin)FDA LinkGeneric 07/07/2013 - "Rat pheochromocytoma (PC12) cells, a suitable model to study exocytotic dopamine release, were differentiated on IDEs functionalized with an overoxidized PSS(-)-doped PPy film. " 05/01/2013 - "The purpose of this study was to characterize methylmercury (MeHg)-induced dopamine (DA) release from undifferentiated pheochromocytoma (PC12) cells and to examine the potential role for DA synthesis in this process. " 10/09/2007 - "Thus, alterations of gene expression, dopamine (DA) content, and cell proliferation in rat pheochromocytoma PC12 cells are employed as indicators to clarify this relationship in the present study. " 12/17/2015 - "Dopamine release from a rat pheochromocytoma cell is more stimulated by a more negative voltage pulse. " 07/01/2015 - "The microchip capillary electrophoresis system was successfully applied to determine dopamine in single cultured rat pheochromocytoma cells. "
9.	Adrenergic Receptors (Adrenergic Receptor)IBA 06/01/1988 - "The purpose of the study was to investigate leukocyte beta adrenoceptors and platelet alpha 2 adrenoceptors in pheochromocytoma. " 06/01/1988 - "[Value of the study of circulating adrenergic receptors in the diagnosis and surveillance after surgery of pheochromocytoma]." 10/05/1984 - "Radioligand binding studies on the density and characteristics of alpha adrenoceptors have not revealed consistent differences between normotensive and hypertensive subjects, with the exception of pheochromocytoma, in which a consistent down regulation of alpha 2 adrenoceptors in thrombocytes has been demonstrated. " 05/01/2013 - "In addition, Eph had a significant effect on the viability of the rat pheochromocytoma PC12 cells through β-adrenergic receptors. " 07/01/2010 - "A reduction in mortality associated with resection of pheochromocytoma (from 40% to 60% to the current 0% to 6%) occurred when alpha-adrenergic receptor blockade was introduced as preoperative preparatory therapy."
10.	PhenoxybenzamineFDA Link 06/01/1990 - "This prospective clinical study evaluates the possible beneficial effects of increased phenoxybenzamine dosage in the preoperative treatment of patients with pheochromocytoma. " 05/01/2007 - "A pheochromocytoma was suspected, and phenoxybenzamine was added to her treatment regimen. " 05/01/2005 - "After recompensation and subsequent administration of phenoxybenzamine a benign pheochromocytoma was resected. " 10/01/2004 - "Chronomically interpreted monitoring revealed: 1) the persistence of a statistically significant circadian rhythm during 12-hourly phenoxybenzamine treatment; 2) days-long changes in BP MESOR, the duration of which could not be previously determined based on spotchecks; 3) changes in the circadian amplitude of BP, which can be either very small or very large, compatible with the diagnosis of intermittent circadian hyper-amplitude-tension (CHAT); and 4) a very wide range of BP and HR values, so that occasional (casual) measurements fail to convey the dynamics that may underlie this infrequently found clinical condition of an elevated catecholamine excretion compatible with a pheochromocytoma. " 10/01/2004 - "Circadian hypo- and hyper-amplitude-tension (CHAT) associated with putative pheochromocytoma and 12-hourly phenoxybenzamine treatment."

Therapies and Procedures

1.	Adrenalectomy 06/01/2007 - "Is the laparoscopic adrenalectomy for pheochromocytoma the best treatment?" 04/01/2009 - "Surgical resection remains the only curative therapy for pheochromocytomas, and advances in minimally invasive techniques have shown laparoscopic adrenalectomy to be safe and effective. " 05/01/2000 - "Laparoscopic adrenalectomy for selected patients presenting pheochromocytoma is feasible and provides good results." 09/01/2011 - "Posterior retroperitoneoscopic adrenalectomy is a safe and effective alternative to transabdominal laparoscopic adrenalectomy for pheochromocytoma." 06/01/2010 - "Laparoscopic adrenalectomy is a safe and effective treatment for large pheochromocytomas. "
2.	Drug Therapy (Chemotherapy) 06/01/1996 - "The combined chemotherapy with CVD appears to be effective for advanced malignant pheochromocytoma." 10/01/1984 - "Chemotherapy trials in malignant pheochromocytoma: report of two patients and review of the literature." 11/01/2015 - "Strategic Chemotherapy for Pheochromocytoma?" 11/01/2015 - "Metastatic pheochromocytoma continues to be an incurable disease, and treatment with conventional cytotoxic chemotherapy offers limited efficacy. " 06/01/2014 - "Preoperative treatment included primary pheochromocytoma resection, chemotherapy, alpha blockade, embolization, and radiation. "
3.	Anesthesia 12/01/2003 - "In light of surgical and anesthesia expertise, laparoscopic resection of benign pheochromocytomas is safe and effective with resultant short hospital stays. " 11/01/2014 - "Management of anesthesia in unspecified extra-adrenal pheochromocytoma patient who used beta-blocker." 07/01/2012 - "Anesthesia for a patient with pheochromocytoma is challenging; irrespective of whether it is a diagnosed case for planned surgery or an occult case, it can be a nightmare. " 12/01/2011 - "Fifty-nine cases of pheochromocytoma were confirmed by histopathology examination: 38 in group 1 and 21 in group 2. No differences were found in the preoperative demographics, comorbidities, BP and anesthesia. " 10/01/2011 - "Pheochromocytoma detected during anesthesia induction."
4.	Combination Drug Therapy (Combination Chemotherapy) 05/01/1985 - "Thus, combination chemotherapy appears to be effective for symptomatic malignant pheochromocytoma." 09/01/2003 - "Successful treatment of malignant pheochromocytoma with combination chemotherapy containing anthracycline." 01/01/1990 - "Successful combination chemotherapy of malignant pheochromocytoma]." 08/15/1988 - "Irradiation and combination chemotherapy should be evaluated further in the management of malignant pheochromocytoma." 08/15/1988 - "High-voltage irradiation and combination chemotherapy for malignant pheochromocytoma."
5.	Ligation 04/01/2011 - "In this study we present our experience for laparoscopic resection of pheochromocytomas, and evaluate the safety of delayed adrenal vein ligation. " 07/01/2013 - "Early identification and ligature of the adrenal vein, with minimal gland manipulation, are the major advantages, especially in case of pheochromocytoma. " 04/01/2011 - "Laparoscopic resection of pheochromocytomas with delayed vein ligation." 03/01/2008 - "Overexpression of ERBB-2 was more frequently detected in malignant than benign pheochromocytomas by multiplex ligation-dependent probe amplification and immunohistochemistry." 07/01/2002 - "We recommend an early ligature of the suprarenal vein in a pheochromocytoma."