ABSTRACT
Pheochromocytoma is a rare chromaffin cell tumor that may be associated with a genetic predisposition, such as von Hippel-Lindau (VHL) disease. VHL is an autosomal dominant disorder that is characterized by a predisposition to multiple tumors, including retinal and central nervous system hemangioblastomas, renal cell carcinoma, and pheochromocytomas. The classic presentation of pheochromocytoma is episodic hypertension, headaches, palpitations, and diaphoresis. In the pediatric population, 40% of pheochromocytomas have a hereditary basis. We present a case of metastatic pheochromocytoma in a child with VHL and discuss the relevant current medical literature.
Subject(s)
Adrenal Gland Neoplasms/pathology , Lung Neoplasms/secondary , Pheochromocytoma/secondary , von Hippel-Lindau Disease , Adrenal Gland Neoplasms/etiology , Adrenal Gland Neoplasms/surgery , Asymptomatic Diseases , Child , Humans , Lung Neoplasms/etiology , Lung Neoplasms/surgery , Male , Pheochromocytoma/etiology , Pheochromocytoma/surgery , von Hippel-Lindau Disease/complicationsABSTRACT
An eleven year old boy presented with headaches and dizziness associated to micturition. On radiologic imaging, he was found with a bladder mass. The biochemical work up was suggestive of pheochromocytoma. An OctreoScan (111In-pentreotide) was used to rule out metastatic extension or other extra-adrenal locations of the pheochromocytoma. OctreoScan data correlated well with other radiologic studies, operative findings and with the final diagnosis, validating its use on pediatric patients.
Subject(s)
Adrenal Gland Neoplasms/pathology , Indium Radioisotopes , Pheochromocytoma/diagnostic imaging , Pheochromocytoma/secondary , Somatostatin/analogs & derivatives , Urinary Bladder Neoplasms/diagnostic imaging , Urinary Bladder Neoplasms/secondary , Child , Humans , Male , Radionuclide ImagingABSTRACT
An eleven year old boy presented with headaches and dizziness associated to micturition. On radiologic imaging, he was found with a bladder mass. The biochemical work up was suggestive of pheochromocytoma. An OctreoScan (111In-pentreotide) was used to rule out metastatic extension or other extra-adrenal locations of the pheochromocytoma. OctreoScan data correlated well with other radiologic studies, operative findings and with the final diagnosis, validating its use on pediatric patients.
Subject(s)
Child , Humans , Male , Adrenal Gland Neoplasms , Pheochromocytoma , Pheochromocytoma/secondary , Urinary Bladder Neoplasms , Urinary Bladder Neoplasms/secondary , Indium Radioisotopes , Somatostatin/analogs & derivatives , SomatostatinABSTRACT
Presentamos el caso de un paciente varón de 29 años con sospecha clínica de feocromocitoma previamente diagnosticado de una Neoplasia Endocrina Múltiple (MEN) tipo 2b. Se realizó una gammagrafía con 123I-metaiodobelcilguanidina (MIBG) que mostró captación patológica en ambas suprarrenales, sugestiva de feocromocitoma bilateral. El estudio de resonancia magnética (RMN) determinó la localización anatómica. El paciente fue sometido a suprarrenalectomía bilateral confirmándose el diagnóstico de feocromocitoma bilateral en anatomía patológica.
We report a case of 29 years old man who was referred with the clinical symptoms of pheochromocytoma in a Multiple Endocrine Neoplasia (MEN) type 2b. 123I- metaiodobenzylguanidine (MIBG) scan revelead pathologic adrenal bilateral uptake described in bilateral pheochromocytoma. Anatomical localization was achieved by Magnetic Resonance (MRI). The patient was submitted to surgery for bilateral adrenal removal. Surgical pathology confirmed the diagnostic of pheochromocytoma in both adrenal glands.
Subject(s)
Male , Adult , Humans , Pheochromocytoma , Pheochromocytoma/secondary , Adrenal Gland Neoplasms , Pheochromocytoma/surgery , Pheochromocytoma/diagnosis , Magnetic Resonance Imaging , /pathology , Adrenal Gland Neoplasms/surgery , Adrenal Gland Neoplasms/diagnosis , RadiopharmaceuticalsABSTRACT
Pheochromocytoma is a rare tumor originating from neuroectodermic cells. Only 10% of these tumors are malignant. There are many familial forms of this tumor, including multiple endocrine neoplasia type II, Von Hippel-Lindau syndrome, and neurofibromatosis type I. Skin manifestations of pheochromocytoma are rare, and cutaneous metastasis in patients with multiple endocrine neoplasia IIB has never been described. The case of a patient with multiple endocrine neoplasia IIB who presented malignant pheochromocytoma with multiple cutaneous metastasis is described.
Subject(s)
Adrenal Gland Neoplasms/pathology , Multiple Endocrine Neoplasia Type 2b/pathology , Pheochromocytoma/secondary , Skin Neoplasms/secondary , Adrenal Gland Neoplasms/chemistry , Adult , Humans , Immunohistochemistry , Male , Multiple Endocrine Neoplasia Type 2b/chemistry , Pheochromocytoma/chemistry , Skin Neoplasms/chemistryABSTRACT
We report a 28 years old woman who consulted for diarrhea of two years and a thyroid nodule. A medullary thyroid carcinoma was diagnosed and a thyroidectomy performed. There was a local relapse two months later and distant metastases were found five months later. A MIBG-1131 scintigraphic image of the adrenals lead to the suspicion of a bilateral pheochromocytoma. The surgical resection of the adrenals confirmed the diagnosis. There was no response to chemotherapy and the patient continued with severe hypercalcemia, repeated infections, persistent diarrhea and cachexia, dying one year after the diagnosis. There was no family history of the disease. We conclude that this is a particularly aggressive presentation of a multiple endocrine neoplasia type 2A.