Upfront Cytoreductive Surgery as a Bridge to Systemic Therapy for Recurrent Peritoneal Dissemination of Ovarian...

Ovarian angiosarcoma is exceptionally rare, especially considering those few pediatric cases that have been reported, and displays a poor outcome. Recurrent peritoneal dissemination of ovarian angiosarcoma is generally managed with systemic therapy, but refractory malignant ascites may compromise performance status and hinder treatment delivery. Herein, we report a pediatric case in which upfront cytoreductive surgery (CRS) controlled refractory ascites and enabled subsequent systemic therapy for recurrent peritoneal dissemination.

A 14-year-old girl presented with acute abdominal pain and underwent left salpingo-oophorectomy for a large ovarian tumor. Histopathological examination revealed mucinous carcinoma occupying most of the mass, with focal areas containing angiosarcoma and mature teratoma components. Staging surgery 1 month later showed no residual malignancy, and no adjuvant chemotherapy was administered. Six months after the initial surgery, she developed diffuse peritoneal dissemination and massive hemorrhagic ascites. Paracentesis provided only transient relief; ascites rapidly reaccumulated, oral intake became impossible, and progressive anemia required daily red blood cell transfusions for 6 consecutive days. Cell-block cytology of ascitic fluid suggested angiosarcoma. As adequate ascites control was not achievable and chemotherapy was considered impracticable, upfront CRS was performed, consisting of a total hysterectomy with right salpingo-oophorectomy and debulking of peritoneal dissemination. Resected lesions were exclusively composed of angiosarcoma. Postoperatively, the ascites markedly decreased, enabling the initiation of chemotherapy on POD 7 and completion of adjuvant chemoradiotherapy without substantial interruption. The patient has remained free of radiologic disease and ascites for 1 year after the initial surgery.

This report demonstrated that CRS could provide effective control of malignant hemorrhagic ascites in recurrent peritoneal dissemination of ovarian angiosarcoma. Upfront CRS can be considered as a potential initial treatment option, facilitating the timely delivery and completion of subsequent systemic therapy.