Cutaneous metastasis of pheochromocytoma in multiple endocrine neoplasia IIB.

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.

Response of atrial natriuretic factor to acute extracellular fluid volume in patients with pheochromocytoma.

BACKGROUND: Patients with pheochromocytoma have been reported to show high plasmatic atrial natriuretic factor (ANF) levels. Its source may not be the atrium because blood volume, the most important physiological stimulus for ANF release, is usually reduced in these patients. METHODS: To evaluate ANF secretion functional integrity, we studied three patients with pheochromocytoma before and after surgical removal of the tumor. Extracellular fluid (ECF) volume, plasmatic ANF levels, and plasmatic renin activity (PRA) were measured. ANF was measured before and after an acute saline load of 1.5L in 90 min. RESULTS: Before surgery, ECF volume was normal or reduced, and PRA was normal but decreased after the saline load. By contrast, ANF was elevated and did not change after the saline load. After surgery ANF decreased, ECF volume rose, and the saline load induced a significant increase of plasma ANF and reduction of PRA. ANF was present in significant amounts in tumoral tissue homogenates. CONCLUSIONS: These data suggest that the tumor was the source of ANF in these patients with pheochromocytoma because high levels of ANF, despite reduced or normal ECF volume, as well as unresponsiveness to acute saline infusion, were found before surgery with subsequent normalization after tumor removal.

Serum catecholamine levels determined by high performance liquid chromatography coupled with electrochemical detection

Este trabalho apresenta um método rápido para a quantificaçäo de catecolaminas utilizando a técnica de cromatografia líquida de fase reversa acoplada à detecçäo eletroquímica. Separaçäo isocrática rápida foi obtida empregando como fase móvel a soluçäo: 0,02M de fosfato de sódio dibásico, 0,02M de ácido nítrico, pH 2,64, metanol a 10 por cento, 0,12mM de EDTA sódico e 556 mg/L de ácido heptanosulfônico. Delineou-se o procedimento de preparaçäo das amostras com extraçäo das monoaminas em alumina, para melhorar a recuperaçäo e diminuir fatores de diluiçäo. O tempo total de análise é de 15 minutos, com boa separaçäo dos picos de monoaminas. O limite de detecçäo obtido para as monoaminas séricas é de 40 a 50 pg/mL, com uma taxa de recuperaçäo de 70-75 por cento.

[Catecholamine-producing tumors]. / Tumores productores de catecolaminas.

We report 93 patients with catecholamine producing tumors that were analyzed at the Hormone Laboratory of the Institute of Cardiology. They are 75 pheochromocytoma patients and 18 children with neuroblastoma. Fluorimetric methods were used to measure urinary and plasma catecholamines on neuroblastoma and pheochromocytoma patients. Dopamine high excretion (mean value 2889 micrograms/24 hs), was constantly observed in the neuroblastoma children as were adrenaline and noradrenaline in the benign and malignant pheochromocytoma patients. The mean values for the malignant tumours were 53 for adrenaline and 1436 micrograms/24 hs for noradrenaline. Structural and biochemical differences of the catecholamine producing tumours are reflected on the clinical manifestations which are observed in the patients bearing such neoplasms.