Primary ovarian angiosarcoma in a 12- year -old girl: a case report of an exceptional localization in a context of limited resources country

Abstract

BackgroundOvarian sarcomas represent less than 1% of all ovary cancers and usually are frequent in adults. Primary angiosarcomas are exceptional in the ovaries within children.Case presentationWe reported a case of primary ovarian angiosarcoma in a 12-year-old girl in a resource-constrained context. Immunohistochemistry study showed the positivity of CD34, CD31, factor VIII, while S100 was negative. The diagnosis of primary non-metastatic angiosarcoma was retained. She was unable to undergo the CWS-2002P chemotherapy since her parents could not afford it.ConclusionThis case report described a rare type of a primary ovarian angiosarcoma within a child, diagnosed in a low-income country in a laboratory with limited resources.

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

“…To date, there have been less than 40 cases of ovarian angiosarcoma reported in the literature, and only six reported cases in children and young adults less than 21 years of age. [2][3][4][5][6] This is one of the first reported cases to utilize HIPEC in the treatment of ovarian angiosarcoma and she is currently alive and well at 43 months after initial diagnosis, significantly longer than any other reported case of advanced-stage ovarian angiosarcoma.…”

“…To date, there have been less than 40 cases of ovarian angiosarcoma reported in the literature, and only six reported cases in children and young adults less than 21 years of age …”

“…Angiosarcoma is a rare vascular tumor, representing 2–3% of soft tissue sarcomas (STS) in adults and 0.3% of STS in children . The majority occur in older patients and rarely arise from within the ovary .…”

“…Angiosarcoma is a rare vascular tumor, representing 2–3% of soft tissue sarcomas (STS) in adults and 0.3% of STS in children . The majority occur in older patients and rarely arise from within the ovary . The prognosis is poor with less than a 30% 5‐year overall survival (OS) for patients with nonmetastatic disease and almost certain death within <1 year for those with metastatic disease .…”

“…2 The majority occur in older patients and rarely arise from within the ovary. 2,8 The prognosis is poor with less than a 30% 5-year overall survival (OS) for patients with nonmetastatic disease and almost certain death within <1 year for those with metastatic disease. 7 Tumor marker elevation is uncommon, although there have been five reported cases of elevated CA-125 in addition to our case, four of which had advanced-stage disease.…”