後腹膜悪性線維性組織球腫の1剖検例

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A 35-year-old woman with a 5-month history of lower abdominal dull pain and lumbago was referred to us for a possible left renal tumor in October 1979. Physical examination revealed a hard fixed painless and poorly demarkated mass, measuring 8×13cm in the left abdomen. Chest X-ray disclosed multiple round shadows in all lobes and an IVP showed left hydronephrosis. At laparotomy, a very large mass was seen originating in the retroperitoneum and tightly adherent to the left kidney, ureter, ovary, uterus, aorta, and sigmoid colon. Colostomy and biopsy was done. Histologic diagnosis of malignant fibrous histiocytoma was obtained and the patient was given immunotherapy (PSK and OK-432), radiotherapy to the abdominal mass (a total of 4110 rads on the linear accelerator) and ifosfamide, 1480 mg/sqm daily for 5 days for 2 courses. Then adriamycin, 37 mg/sqm was given for 2 courses. Abdominal mass was moderately reduced in size, but pulmonary shadows continued　to enlarge. Therefore CYVADIC regimen was began but the patient died in March 1980,4 month and a half after the initial diagnosis. At autopsy metastases were found in the lung, midbrain, ileum and omentum. The primary tumor was fibrotic, hyalinized and necrotic. Twelve Japanese cases of retroperitoneal malignant fibrous histiocytoma was reviewed.
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