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Li-Fraumeni syndrome is a relatively rare disease entity characterized by sarcomas of the soft tissues, bone and miscellaneous tumors ofjuvenile onset and frequent occurrence of metachronous tumors (1). Germline mutation of the p53 tumor suppressor gene has been reported in approximately 70% ofthe Li-Fraumeni kindreds (2). Here we report a case of Li-Fraumeni syndrome presenting a gastric cancer in the proband and a hepatosarcoma in his son. The proband was a 38-year-old Japanese male referred to a clinic with a diagnosis of gastric carcinoma with multiple liver metastases. There was no previous history of malignant disorders, but his 1] -year-old son suffered from a liver tumor diagnosed as a hepatosarcoma and underwent surgical resection followed by systemic chemotherapy (Fig. l ). The proband received systemic chemotherapy comprising seven courses of 5FU and MTX.

In 1971, the Japanese Society of Pediatric Surgeons' Committee on Malignancies proposed new criteria for neuroblastoma staging. It was fundamentally, based on the system of Evans et al. described in 1971. The main difference was the separation of stage IV disease into stages IV-A, with metastases to bone, orbita, distant lymph nodes and viscera other than liver, IV-B, the primary tumor extending over the midline and with metastases to bone marrow, liver and skin, and IV-S, which was the same as that of Evans et al. The new criteria did not include the resectability of the primary tumor, assessment of regional lymph node involvement or any other disease assessment resulting from therapeutic intervention. For the purpose of international usage, the Japanese system has been newly formulated and proposed as the Japanese Tumor Node Metastasis (TNM) Postsurgical Histopathological Classification for Neuroblastoma. In the present report, 495 neuroblastomas, registered between 1970 and 1985, were analyzed retrospectively according to the International Union Against Cancer (UICC) TNM classification and the proposed Japanese TNM system. The analyses suggested that the Japanese system reflected both the extent of tumor invasion and its biological neuroblastoma characteristics better than the UICC TNM classification based on statistical analysis.