ABSTRACT
Malignancy must be considered in the management of adrenal lesions, including those incidentally identified on imaging studies. Adrenocortical carcinomas (ACCs) are rare tumors with an estimated annual incidence of 0.7-2 cases per year and a worldwide prevalence of 4-12 cases per million/year. However, a much higher incidence of these tumors (>15 times) has been demonstrated in south and southeastern Brazil. Most ACCs cause hypersecretion of steroids including glucocorticoids and androgens. ACC patients have a very poor prognosis with a 5-year overall survival (OS) below 30% in most series. Pheochromocytoma or paraganglioma (PPGL) is a metabolically active tumor originating from the chromaffin cells of the adrenal medulla. The incidence of PPGL is 0.2 to 0.9 cases per 100,000 individuals per year. Pheochromocytomas are present in approximately 4-7% of patients with adrenal incidentalomas. Classically, PPGL manifests as paroxysmal attacks of the following 4 symptoms: headaches, diaphoresis, palpitations, and severe hypertensive episodes. The diagnosis of malignant PPGL relies on the presence of local invasion or metastasis. In this review, we present the clinical and biochemical characteristics and pathogenesis of malignant primary lesions that affect the cortex and medulla of human adrenal glands.
Subject(s)
Humans , Paraganglioma/therapy , Pheochromocytoma/therapy , Adrenal Cortex Neoplasms/therapy , Adrenal Gland Neoplasms/therapy , Adrenocortical Carcinoma/therapy , Paraganglioma/diagnosis , Paraganglioma/pathology , Pheochromocytoma/diagnosis , Pheochromocytoma/pathology , Adrenal Cortex Neoplasms/diagnosis , Adrenal Cortex Neoplasms/pathology , Adrenal Gland Neoplasms/diagnosis , Adrenal Gland Neoplasms/pathology , Adrenocortical Carcinoma/diagnosis , Adrenocortical Carcinoma/pathology , Antineoplastic Agents, Hormonal/therapeutic use , Mitotane/therapeutic useABSTRACT
Os autores estudaram 24 casos de carcinoma funcionante do cørtex adrenal com o objetivo de revisar o tratamento cir£rgico e quimioter pico. Procurou-se fazer correlaüoes com o tipo de cirurgia e a efi cia da quimioterapia com a sobrevida. Observou-se que tanto a adrenalectomia como a nefroadrenalectomia ofereceram resultados semelhantes em termos de sobrevida e significativamente a sobrevida. (Arq Bras Endocrinol Metab 1994; 38/1:16-22).