[von Recklinghausen's disease associated with pheochromocytoma--an autopsy case report and review of 24 cases in the Japanese literature].
A 56-year-old woman was admitted because of vomiting and right hypochondralgia. Since she was 25 years old, she had been sick with neurofibromatosis. Systemic neurofibromatosis with café-au-lait spots, blood hypertension and tenderness on the right hypochondrium were found. Ultrasonic examination revealed a right adrenal mass. Levels of serum catecholamines were elevated. She developed ileus and died of shock from the release of adrenal catecholamines. Postmortem examination showed right adrenal pheochromocytoma. The tumor measured 11.5 X 8 cm and weighed 25 gm. Von Recklinghausen's disease associated with pheochromocytoma is very rare, and only 24 cases have been reported in Japan. We present our case here with a review of the literature.
D000310:Adrenal Gland Neoplasms / Q000473:pathology*; D000313:Adrenal Medulla / Q000473:pathology; D005260:Female; D006801:Humans; D006973:Hypertension / Q000150:complications; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary; D009456:Neurofibromatosis 1 / Q000473:pathology*; D010673:Pheochromocytoma / Q000473:pathology*; D012878:Skin Neoplasms / Q000473:pathology*