1/101. Posttraumatic hypertension secondary to adrenal hemorrhage mimicking pheochromocytoma: case report.We report the case of a 68-year-old man who presented with a mass 3 x 4 cm in size located in the right adrenal gland together with extreme hypertension, tripled urine levels for normetanephrine, and normal plasmatic levels of metanephrines. The patient had suffered a fall from a height of 2.5 meters before hospitalization. [123I]MIBG-scan was repeatedly positive in the area of the right adrenal gland. At laparotomy under alpha-adrenergic blocking agents, the suspected pheochromocytoma was histologically confirmed as hematoma. After resection of the adrenal gland, blood pressure returned to normal without drug therapy as did metanephrine levels in urine. Although adrenal insufficiency after distension of the gland caused by hemorrhage has been reported, there are no data available regarding the mimicking of a hormonally active pheochromocytoma. We conclude that intra-adrenal pressure rise caused by hematoma may cause partial ischemic necrosis to the gland but may also induce reactive hyperplasia with periodic excessive secretion of catecholamines. This interpretation is consistent with the finding that plasma levels of catecholamines were normal in contrast to the urinary normetanephrines in the presented case. It might be worthwhile to investigate patients with intra-adrenal hemorrhage immediately after sustaining multiple injuries and in the posttraumatic course of several months up to 1 or more years together with verification of abnormal urinary excretion of metanephrines as a sign of impaired adrenal function.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
2/101. Extra-adrenal phechromocytoma and renal cell carcinoma--an unusual case of tumour synchronism.We report an unusual case of tumour synchronism combining an extra-adrenal pheochromocytoma and a renal cell carcinoma in a 48-year-old male. The most usual forms of presentation are described in accordance with their clinical significance. We emphasize the diagnostic importance of specific biochemical studies and the accurate anatomical localization of extra-adrenal chromaffin tissue by magnetic resonance imaging (MRI) and scintigraphy with 131 iodine-methyliodobenzylguanidine (131I-MIBG). Surgical excision was the treatment of choice after the institution of adequate preoperative cardiovascular management. Pertinent features of the natural history of these tumours are described in accordance with their different embryological origin.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
3/101. Malignant pheochromocytoma with multiple hepatic metastases treated by chemotherapy and transcatheter arterial embolization.A 62-year-old Japanese male developed multiple hepatic metastases two years after resection of pheochromocytoma of the right adrenal gland. Transcatheter arterial embolization (TAE) was performed for the purpose of the treatment of hepatic metastases resistant to 27 cycles of combined chemotherapy consisting of cyclophosphamide, vincristine, and dacarbazine. After TAE, the hepatic metastatic lesions decreased in size and hypertension passed its crisis. The present case suggests the utility of TAE for multiple hepatic metastases under careful blood pressure monitoring.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
4/101. A patient with neurofibromatosis type 1 and watery diarrhoea syndrome due to a VIP-producing adrenal phaeochromocytoma.A patient with neurofibromatosis type 1 and watery diarrhoea syndrome due to a VIP-producing adrenal phaeochromocytoma (Case Report). J Intern Med 1999; 246: 231-234. A 43-year-old patient with neurofibromatosis type 1 suffered from watery diarrhoea syndrome induced by excessive production of vasoactive intestinal polypeptide (VIP) in an adrenal phaeochromocytoma. This case report emphasizes that patients with neurofibromatosis are prone to develop more than one disease induced by tumours originating from the neural crest. Since excessive VIP production in a phaeochromocytoma may mask the symptoms of catecholamine overproduction, and in view of the therapeutic consequences, neurofibromatosis patients with hyperVIP-aemia must be checked for the presence of a phaeochromocytoma.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
5/101. Laparoscopic intraoperative ultrasonic localization and resection of an extra-adrenal pheochromocytoma.With advances in minimally invasive techniques, the safety and efficacy of laparoscopic adrenalectomy is well documented. Compared with open surgery, the laparoscopic approach results in shorter hospital stay, reduced morbidity, and more rapid recovery. In the setting of pheochromocytoma, tumor resection is feasible although somewhat more difficult because of catecholamine liberation and frequent large lesion size. We report the successful laparoscopic resection of a para-aortic, extra-adrenal paraganglioma using ultrasound guidance.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
6/101. Clinicopathological study of pheochromocytoma of the urinary bladder: immunohistochemical, flow cytometric and ultrastructural findings with review of the literature.Pheochromocytomas usually arise from the adrenal medulla but may also arise from the carotid body, the retroperitoneum, the urinary bladder, and other locations. We report three cases of pheochromocytoma of the urinary bladder with clinicopathological, immunohistochemical, flow cytometric, and ultrastructural findings. Case 1, a 13-year-old boy presented with hematuria. He underwent partial cystectomy, 31 years later he presented with a tumor in the thoracic vertebra. Case 2, a 35-year-old woman presented with headache, nausea, vomiting, palpitations, and diaphoresis on evacuation. She underwent total cystectomy and regional lymph adenectomy. She survived for 10 years without recurrence or metastasis; however, she died from another disease. Case 3, a 31-year-old man presented with dysuria. He underwent total cystectomy and regional lymph adenectomy. The tumor metastasized to the lymph nodes, and the patient died after 4 years. The urinary bladder tumors in these three cases protruded into the lumen and invaded deeper than the middle of the muscle layer. The tumor of the urinary bladder, metastatic lymph nodes, and thoracic vertebra showed alveolar and trabecular patterns, and tumor cells were surrounded by capillaries. The tumor cells were moderate in size with ovoid nuclei and abundant eosinophilic cytoplasm that contained acidophilic granules reactive to Grimelius stain. Vascular invasion was observed in cases 1 and 2. Immunohistochemically, tumor cells showed reactivity for chromogranin, Leu 7, and S-100 protein. In each of the three cases, the dna ploidy pattern on flow cytometry was aneuploid. Ultrastructural examination revealed several neurosecretory granules, rough endoplasmic reticulum, and a few mitochondria within the cytoplasm. It is difficult to determine whether pheochromocytoma of the urinary bladder is malignant on the basis of histological, immunohistochemical, and flow cytometric findings. Long-term follow up is necessary.- - - - - - - - - - ranking = 2keywords = size (Clic here for more details about this article) |
7/101. multiple endocrine neoplasia type 2a. Study of a family.INTRODUCTION: Pheochromocytomas (Pheo) can occur sporadically, isolated or in association with other neuroendocrine lesions. In multiple endocrine neoplasia type 2a (men-2A), Pheo is associated to medullary thyroid carcinoma (MTC) or its precursor, C-cell hyperplasia (CCH) and parathyroid hyperplasia. Genetic screening provides early diagnosis and preventive treatment. In order to validate dna analysis as a reliable method of early identification of gene carriers, we compared the results of genetic screening with clinical, biochemical, imaging and pathological findings in the members of an affected family. population AND methods: The diagnosis of a bilateral necrotic Pheo in a female patient led to the study of a family with four generations, aged 3 to 78 years (mean = 30.3 yrs). The study included a clinical examination; basal and pentagastrin stimulated calcitonin values; urinary catecholamines and their metabolites; serum calcium and a genetic study (direct sequence of PCR products from genomic dna isolated from leucocytes using specific primers in exon 11 of the RET protooncogene of chromosome 10). The radiologic study, gammagraphic study (131I-MIBG) and magnetic resonance study were performed in members with clinical suspicion of Pheo. RESULTS: Seven out of nine patients had a mutation on codon 634 of exon 11 of RET (TGC-CGC), leading to cysteine arginine substitution in the codified protein; all gene carriers had biochemical markers of MTC/CCH and four of Pheo. The Pheo patients underwent adrenalectomy (bilateral in three) and all the gene carriers underwent prophylactic thyroidectomy. The pathologic findings were: MTC in four (metastasized in one); CCH in three and parathyroid hyperplasia in one. CONCLUSIONS: Phenotypic penetration of RET mutation was 100% for MTC/CCH, but only 57% of the gene carriers had Pheo. Genetic screening allowed early prophylactic treatment in four out of seven patients; pathologic findings revealed several evolutionary stages of the disease. patients not yet showing Pheo are under close clinical and laboratory surveillance.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
8/101. Management approaches to adrenal incidentalomas (adrenalomas). A view from Athens, greece.We believe the management of adrenalomas should include the following: 1. A detailed history and physical examination to detect subtle evidence of hormonal hypersecretion or the possibility of metastatic carcinoma 2. Hormonal studies, such as The short dexamethasone suppression test (2 mg of dexamethasone) followed by a high-dose dexamethasone suppression test (8 mg), CRH assay, and analysis of the diurnal cortisol rhythm if serum cortisol is greater than 3 micrograms/dL 24-Hour (or spot) urinary catecholamine metabolites (metanephrine and normetanephrine) plasma aldosterone level and renin activity in the hypertensive or normotensive patient with serum potassium less than 3.9 nmol/L 3. Additional imaging studies, such as MR imaging 131I-iodocholesterol (NP59) scanning to detect a subclinically functioning adenoma or carcinoma (MIBG scanning is rarely indicated) The role of FNA is limited. This modality may be helpful only in the patient with coexistent extradrenal carcinoma to confirm adrenal metastasis. Although genetic and molecular biology studies do not have wide clinical application, they should be encouraged and supported. Once all of these data are collected, the clinician should perform the following two steps: 1. All clearly nonfunctioning adrenalomas that are not suspicious for malignancy should be observed for several years, mainly with hormonal studies, until their secretory and benign nature is confirmed. 2. All patients with adrenalomas with evidence of subclinical function or potential for malignancy (using size, imaging, FNA, and molecular biology criteria) should undergo laparoscopic adrenalectomy for definitive diagnosis and therapy. The age, the overall medical condition, and the anxiety of the patient should be considered in the decision to operate on a patient with an adrenaloma.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
9/101. pheochromocytoma in an incidentally discovered asymptomatic cystic adrenal mass.Cystic adrenal lesions can be either cortical or medullary, and distinguishing between these 2 types of lesions may be important in patient management. Pheochromocytomas, which are adrenal medullary neoplasms, typically manifest with hypertension, headaches, palpitations, tachycardia, sweating, and anxiety symptoms; however, 10% to 17% of patients with pheochromocytomas are asymptomatic. We describe a 67-year-old woman with lifelong headaches and recent persistent cough in whom a left cystic adrenal mass was incidentally discovered by computed tomography of the chest. A moderate increase in normetanephrine and total metanephrine values in two 24-hour urine samples suggested a pheochromocytoma. Computed tomography with use of contrast medium revealed ring enhancement of the cyst wall, a finding consistent with an adrenal medullary tumor. This report demonstrates the importance of repeated 24-hour urine samples to determine the metanephrine values together with contrast-enhanced computed tomography in a patient with nonspecific symptoms.- - - - - - - - - - ranking = 1.8586093498718keywords = sample (Clic here for more details about this article) |
10/101. Simultaneously occurring liver metastases of pheochromocytoma and medullary thyroid carcinoma--a diagnostic pitfall with clinical implications for patients with multiple endocrine neoplasia type 2a.Malignant pheochromocytoma is an exceptional complication in patients with multiple endocrine neoplasia type 2a (MEN2a). In this paper, we report on a 53-year-old male patient with an evident RET gene germline mutation, who simultaneously developed hepatic metastases of medullary thyroid carcinoma (MTC) and pheochromocytoma. Comprehensive immunohistochemical investigations were performed to elaborate markers which could be useful for differentiating between MTC metastases and pheochromocytoma, respectively. calcitonin and CEA, in particular cytokeratins and trefoil factor family 1 (TFF1), were detectable exclusively in MTC, whereas all the other markers revealed a comparable expression in both MTC and pheochromocytoma. The only clues that could indicate a potential malignant course were size, a lack of sustentacular cells and hyaline globules, and a focal spindle cell pattern in pheochromocytoma. Owing to a wide agreement in cellular differentiation and a lack of specific, routinely applicable markers for pheochromocytomas, a comprehensive and goal-directed immunohistochemistry is required to rule out pheochromocytoma metastasis in patients with MEN2a. A misinterpretation could lead to harmful clinical complications, as shown in the present case.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
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