[A case of malignant pheochromocytoma with high levels of serum neuron-specific enolase (NSE) and calcitonin].

A 71-year-old woman was admitted with the chief complaint of headache, lumbago and slight fever. Computerized tomographic (CT) scan demonstrated a large soft tissue mass with multiple cystic necrosis in the right adrenal region. The plasma norepinephrine concentration was excessive and serum levels of neuron-specific enolase (NSE), calcitonin and parathormone were elevated. MIBG scintigraphy showed a high uptake in the same region. Under the diagnosis of pheochromocytoma without distant metastasis, right adrenalectomy was performed. The tumor was removed en bloc with right kidney and a part of the liver because of inflammatory adhesion. The histological examination revealed benign pheochromocytoma. After the operation, norepinephrine and calcitonin decreased to normal but the levels of NSE and PTH remained high. One year after operation, chest X-ray revealed multiple lung metastases and after 1.5 years she died of respiratory failure. Autopsy revealed multiple lung and bone metastases and a liver metastasis, parathyroid glands showed hyperplasia but the thyroid gland showed no abnormal change. This clinical course suggests that serum NSE might be a useful tumor marker for differentiating malignant pheochromocytoma from benign one, and this tumor producing calcitonin caused secondary hyperparathyroidism.