小児悪性褐色細胞腫 : 本邦集計例の検討

概要

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A case of adrenal malignant pheochromocytoma with liver, bone, and lymph node metastases, in a 7-year-old girl, is reported. The primary tumor and metastatic tumors of liver and lymph node were surgically excised. After operation, she was treated with combination chemotherapy for advanced neuroblastoma. By the end of 8 cycles of chemotherapy, she had a decrease in both plasma and urine catecholamine concentration and there was improvement on the recent bone scan. At present, plasma noradrenalin level is slightly elevates but she is nearly asymptomatic. Although follow-up is still short in duration, combination chemotherapy is effective in our case. Malignant pheochromocytoma is a rare neoplasm and 114 cases have been reported in japan. Among 114 patients there were 66 males and 48 females (including 7 children, one boy and 6 girls) and our patient was the youngest. In 75 patients (65.8 per cent) the primary tumors were located in the adrenal glands. The remaining 39 patients (34.2 per cent) had the extra-adrenal tumors. In two cases of malignant pheochromocytoma, multiple metastases were observed 10 to 12 years after the initial operation under the diagnosis of benign pheochromocytoma in childhood. The diagnosis of malignancy in pheochromocytoma is difficult since benign and malignant pheochromocytomas have similar histologic features. The patients with pheochromocytoma shoud be followed closely and longer.
日本小児外科学会の論文
1992-02-20