Pediatric ovarian angiosarcoma treated with systemic chemotherapy and cytoreductive surgery with heated intraperitoneal chemotherapy: Case report and review of therapy

Abstract

Ovarian angiosarcoma is a rare and aggressive vascular tumor, which has a 5‐year overall survival of less than 30% for patients with nonmetastatic disease and almost certain death within 1 year for those with metastasis. Here, we briefly review historical approaches to therapy and present a long‐term survivor in the case of an 11‐year‐old female with metastatic ovarian angiosarcoma. This is the second reported case to utilize heated intraperitoneal chemotherapy in the treatment of this disease. Our patient is currently alive and well 3 years after initial diagnosis, significantly longer than any reported case of advanced‐stage ovarian angiosarcoma.

Angiosarcoma of the ovary treated with polyadenosine ribose polymerase Inhibition, a case report and review of the literature

“…Angiosarcoma of the ovary is very rare, accounting for less than 1% of adult or pediatric sarcomas. It typically occurs in premenopausal women with an average age of presentation of 31 ( Ye et al, 2021 ); but there are multiple case reports of pediatric diagnosis, with the youngest reported patient only 11 years old ( Thankamony et al, 2018 Dec 1 , Pariury et al, 2019 ). Most patients are initially asymptomatic until their tumor causes mass-related secondary effects, as in this case ( Quesenberry et al, 2005 ).…”

“…Recently Pariury et al reported a patient with a 9 month ongoing disease-free survival using a polyadenosine-ribose polymerase (PARP) inhibitor combined with a PD-L1 inhibitor after pelvic radiation to treat a patient with stage I ovarian angiosarcoma ( Pariury et al, 2019 ). PARP inhibitors have some advantages over chemotherapy which include oral administration, low rates of grade 3 or 4 toxicity and particular efficacy in treating BRCA-related or “BRCA-like” tumors.…”

A Review of the Use of Hyperthermic Intraperitoneal Chemotherapy for Peritoneal Malignancy in Pediatric Patients

“…Angiosarcoma is a vascular malignancy that is rare in children and also rarely metastasizes to the peritoneal cavity but has been successfully treated in a few cases with HIPEC. An 11-year-old diagnosed with metastatic ovarian angiosarcoma with peritoneal sarcomatosis and malignant ascites was found to be in relapse after exploratory laparotomy and drainage of the ascites [15]. The patient was initially treated with ifosfamide and doxorubicin then switched to gemcitabine and docetaxel with partial response, further consolidated with CRS (20 mm or less) with oophorectomy and paclitaxel HIPEC, followed by adjuvant chemotherapy.…”