Primary ovarian angiosarcoma: a rare and recognizable ovarian tumor

Abstract

The diagnosis of primary angiosarcoma of ovary is still a challenge as it has no specific clinical symptoms and is easily confused with other malignant neoplasms in morphology. Here, we described a case of primary ovarian angiosarcoma and reviewed the literature. A 47-year-old female showed a left ovary mass. Grossly, the cut surface of the tumor was solid and gray-white with intermediate texture. Some areas were spongy and atropurpureus with a soft texture. Microscopically, the tumor cells were arranged into a variety of different structures with visible hemorrhage. Immunochemically, the tumor cells were positive for CD31, ERG, Fli1, D2–40 and vimentin in a strong and diffused manner. CD34 stain showed focal positivity. Epithelial markers (e.g. CK, CK7, CK8/18 and PAX8) were all negative. Negative immunostaining for SMA, S-100, P53 and calretinin also were detected. The proliferative index (Ki-67) was approximately 40%. After surgery, the patient was treated with radiotherapy, targeted therapy and immunotherapy. In the 9-month follow-up, the patient was survival without evidence of disease. The diagnosis of ovarian angiosarcoma required the careful observation of morphology and the reasonable application of immunohistochemistry. Targeted therapy and immunotherapy are the potential directions for the treatment of 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 ).…”

“…Pathological diagnosis can be difficult due to diverse and complex pathologic appearance among angiosarcomas, and a predilection for coexisting with mature teratomas ( Ye et al, 2021 ). Differential diagnoses include other sarcomas; and it is also critical to rule out benign hemangiomas.…”

“…The most common antibody staining in angiosarcomas are CD31 and CD34. The combination of CD31, CD34 and vascular endothelial antibodies of ERG and Fli1 can confer an angiosarcoma diagnosis ( Ye et al, 2021 ). Angiosarcoma can be differentiated from other sarcomas such as leiomyosarcoma by the relative abundance of vascular structures ( Ye et al, 2021 ).…”

“…Angiosarcomas arising from visceral organs are very uncommon without clear predilection for one site over another. Ovarian angiosarcoma is very rare, accounting for roughly 1 in 1,000,000 ovarian malignancies ( Ye et al, 2021 ). To date only 35 cases have been reported, with no clear standard of care established for metastatic or localized cancer.…”

“…To date only 35 cases have been reported, with no clear standard of care established for metastatic or localized cancer. Reported outcomes of patients with unifocal disease this ranged from 3 months to 9 years; while patients with multifocal disease have a poorer reported outcomes between 18 days and 30 months ( Ye et al, 2021 .…”