Intrarenal pheochromocytoma
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Neurofibromatosis-1 (NF-1) is a genetic neuro-cutaneous disorder that is associated with an increased prevalence of pheochromocytoma (PHEO). However, this association may not be commonly anticipated by physicians, as patients may be normotensive. In addition, NF-1 patients can be asymptomatic and/or normotensive. These factors can result in a delayed or missed diagnosis of pheochromocytoma leading to catastrophic complications. Currently, it is recommended to perform annual blood pressure monitoring in patients with NF-1 and to test for pheochromocytoma only if found to be hypertensive. However, recent studies show that this practice may lead to underdiagnosis of pheochromocytoma. Therefore, suggesting routine biochemical testing for pheochromocytoma in all patients with NF-1.In this case report, we discuss the factors which can lead to a delayed diagnosis of pheochromocytoma in a patient with known NF-1 and hypertension.
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Pheochromocytoma is a rare tumor derived from chromaffin cells, which produces catecholamins. The presence of this tumor is considered a cause of secondary hypertension, arrhythmias, sweating and also, but very rarely, mental disorders. Update diagnostic methods of pheochromocytoma are summarized in this article. Pheochromocytoma also coexists with endocrinological syndroms, e.g. multiple endocrine neoplasia type 2 (MEN 2). Studies confirm genetic background of pheochromocytoma occurrence.
Chromaffin cell
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Purpose:To evaluate the diagnostic and treatment procedures for adrenal pheochromocytoma.Methods:220 cases of adrenal pheochromocytoma surgical treated between 1958～1998 were reviewed, including 19 cases of static pheochromocytoma. 15 cases of bilateral adrenal pheochromocytoma,4 cases of familial pheochromocytoma, 2 cases of MEN Ⅱ,2 cases of Von Hippel lindau diseases,7 cases of malignant pheochromocytoma.Results:Open surgery with ablation of the tumors were undertaken in all the 220 patients, of which 2 cases died from surgery.Conclusions:Definitive preoperative diagnosis is useful to increase surgical safety of static pheochromocytoma. Sufficient preoperative preparation reduces complication of pheochromocytoma cardiomyopathy. Imaging procedures determine surgical pathway.
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Three patients with a surgically confirmed and 1 with suspected pheochromocytoma as part of the syndrome of multiple endocrine neoplasia (MEN) type II are presented. Pheochromocytoma and medullary thyroid carcinoma (MTC) as well as other tumors are part of this heritable entity. Three cases were members of a MEN IIa family and 1 patient has suffered from a sporadic form of MEN IIb. Pheochromocytoma was diagnosed by screening procedures (catecholamine screening, computerized tomography, sonography or selective venous catheterization). In 2 of the patients there was no clinical manifestation of the pheochromocytoma. On three occasions MTC was diagnosed first, the thyroid tumor occurred prior to the pheochromocytoma by 7, 6, and 1/2 years. Pheochromocytoma occurred bilaterally in 2 patients, in 1 it is suspected in both adrenals.
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Pheochromocytoma is a frequent indicator of multiple endocrine neoplasia type 2A (MEN 2A); in the 35 French MEN 2A families in which a pheochromocytoma occurred first in some affected members, 30% of the patients had a pheochromocytoma as the first manifestation constituting 45% of all patients with pheochromocytomas. The finding of a pheochromocytoma is a strong indication for a search for medullary thyroid carcinoma and for initiating family screening.
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Nonfunctional pheochromocytoma is a particular type of pheochromocytoma without typical clinical features of catecholaminism. It is difficult to diagnose or differentially diagnose. This type of pheochromocytoma is rarely reported in the literature. From January 1, 1990 to June 1, 2004, we performed operations for 197 patients with pheochromocytomas, including 26 patients with nonfunctional pheochromocytoma. We analyzed the clinical data of the 26 patients.
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