Malignant Pheochromocytoma of the Adrenal Medulla (Paraganglioma)

Abstract

This malignant pheochromocytoma occurred in a 43 yr. old white [female], whose chief complaints were weakness and pigmentation of the skin of 8-10 yrs. duration. Electrolytic and other disturbances suggested a diagnosis of adreno-cortical insufficiency secondary to carcinoma. However, at autopsy, the cortex of the adrenal was intact, while the right medulla was replaced by a chromaffinoma 15 cm. in diam. Metastases to parenchymatous and skeletal structures were widespread. A critical analysis of the findings in this and the 7 previously reported cases of malignant paraganglionoma of the adrenal reveals that the [male] and [female] are equally affected; the avg. age at onset is 45 yrs.; a palpable mass is present in 4-8 cases; metastases to regional lymph nodes are always observed; invasion of distant organs occurs in at least 75%; the signs and symptoms are those of malignant cachexia with or without a palpable mass in the flank. A striking feature in all cases is the absence of bouts of paroxysmal hypertension considered so characteristic of the benign forms of paraganglioma.