A case of massive adrenal malignant pheochromocytoma: management of a large pheochromocytoma

Abstract

69歳男.直径約30cmの左側腹部腫瘤の診断及び治療の為, 当科へ紹介された.血中ノルエピネフリン値及びその尿中代謝産物の高値, CTスキャンにより巨大な嚢胞性後腹膜腫瘤, 大動静脈リンパ節の腫大が認められ, 悪性褐色細胞腫と診断.血行動態学的に循環血液量の低下と血管抵抗値の上昇が認められた為, 血管拡張剤の使用と共に循環血液量の補充が行われた.これらにより血行動態は正常化し, 腫瘍摘除術が施行された.病理学的に, リンパ節転移を伴う褐色細胞腫と診断され, 腫瘍重量は5, 930gであった

A 69-year-old man was referred to our hospital for a left abdominal tumor measuring about 30 cm in diameter. Laboratory examination revealed an elevation of norepinephrine in plasma and of its metabolites in urine. CT scan disclosed a huge cystic tumor in the retroperitoneal space and an enlarged aortocaval lymph node, suggesting a diagnosis of malignant pheochromocytoma. The hemodynamic studies showed low blood volume and high vascular resistance, and therefore, he was treated with vasodilators and volume expansion. His hemodynamic status normalized and a complete excision was performed. Pathological examination revealed that the patient had a pheochromocytoma with metastasis to a lymph node. The total weight of the tumor was 5, 930 g. Since pheochromocytomas can become a large with a risk of malignancy, they should be surgically excised as completely as possible with further treatment after making a definite diagnosis.