Cytoreductive surgery and HIPEC for intraabdominal synovial sarcoma with peritoneal carcinomatosis = Cytoreductive surgery and HIPEC for intraabdominal synovial sarcoma with peritoneal carcinomatosis

다국어 초록 (Multilingual Abstract)

Synovial sarcoma is a malignant mesenchymal neoplasm with variable epithelial differentiation that mainly occurs adjacent to joint capsules’ bursal and tendon sheath. In spite of the name, Synovial sarcoma can occur everywhere in the body but among these, intra-abdominal synovial sarcoma is rare and accounts 1.8% of its distribution while the mortality is relatively high. There is currently no effective treatment except surgery for this type of sarcoma. We present a case of primary intra-abdominal synovial sarcoma with peritoneal carcinomatosis successfully treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC). A 27 years old nulliparous woman was referred to our hospital for abdominal mass. She had a two-month history of progressive abdominal distension and dizziness. The abdominopelvic CT scan showed greater than 15cm-sized cystic-solid papillary mass lesion with large amount of hemorrhagic ascites. Her serum CA-125 was 222.6 U/mL. Under the impression of ovarian cancer, diagnostic laparoscopy was performed. Around 15cm-sized huge hemorrhagic mass was found. On the frozen section of the tumor, high-grade malignancy was suspected and the final result was synovial sarcoma. At surgical exploration, about 15cm-sized omental mass was seen. Multiple metastatic nodules involving the peritoneum and omentum were found. Primary cytoreductive surgery was performed with following procedures; total parietal peritonectomy, total hysterectomy, bilateral salpingo-oophorectomy, greater omentectomy, appendectomy and HIPEC using cisplatin. There was no gross residual disease and the postoperative course was uncomplicated. She received 6 cycles of adjuvant systemic chemotherapy using AD (doxorubicin + dacarbazine) regimen and has been followed without disease recurrence until now. This is the first demonstration of intraperitoneal synovial sarcoma with peritoneal metastasis successfully treated with cytoreductive surgery and HIPEC.