Right hepatectomy for primary monophasic synovial sarcoma of the liver: case report and review of the literature

INTRODUCTION: Synovial sarcoma is a soft-tissue sarcoma whose histological origin has long been poorly understood. It occurs mainly near the extremities of larges joints but can occur anywhere in the body. Synovial sarcoma of hepatic origin is extremely rare, with only 13 cases reported in the literature. CASE PRESENTATION: We report the case of a 68-year-old female patient who underwent right hepatectomy for a single 12.5 cm mass arising in the S6-S7 segments and evolving for over three years, long considered to be hepatocellular carcinoma. An Anatomopathological examination of the operative specimen showed an undifferentiated tumoral process. The immunohistochemical complement demonstrated a monophasic synovial sarcoma. CLINICAL DISCUSSION: Clinical and radiological signs are not specific, and diagnosis is based on histology, of which there are three types. Complete surgical excision remains the best therapeutic option for localized forms. It may or may not be combined with adjuvant chemotherapy in other forms, although the prognosis of the disease remains guarded. CONCLUSION: Synovial sarcoma of the liver is a rare disease with a histological diagnosis. Treatment is not standardized, and surgical excision remains the best option for localized forms.