Primary ovarian angiosarcoma: two case reports

Background: Primary angiosarcoma of the ovary is extremely rare, and its pathologic diagnosis is challenging due to its non-specific clinical symptoms, and morphologically. Cases: In this report, we presented the clinicopathological characteristics of 2 cases of primary ovary angiosarcoma. In case 1, a 31-year-old patient with abdominal pain for 1 month and a cystic-solid mass with a diameter of about 15 cm in the right uterine adnexa was described. After surgery, a right ovarian angiosarcoma of IA clinical stage was confirmed. The patient received 6 rounds of chemotherapy after surgery and was now alive 9 years with no evidence of recurrence. Case 2 was a 32-year-old patient presenting with abdominal pain and a cystic-solid mass in the left ovary with a diameter of 14 cm was detected. Pathological diagnosis confirmed left ovarian angiosarcoma of Federation International of Gynecology and Obstetrics (FIGO) stage IIIB. After 2 rounds of chemotherapy, the tumour relapsed and the patient died 7 months after the initial diagnosis. The morphology of the tumour was heterogenous in different areas, from well-differentiated areas of angioma-like benign morphology, to poorly differentiated spindle cell areas. Careful observation could find the formation of microcystic or fissure-like structures formed by blood vessels. Immunohistochemically, the tumour cells were diffusely positive for CD31, Fli-1, ERG, and factor VIII (FVIII). CD34 Showed diffusely positivity in case 1, and focal positivity in case 2. Some tumour cells expressed vascular endothelial growth factor (VEGF) and D2-40. Expression of epithelial markers along with SALL4, α-inhibin, calretinin, S-100 and desmin were all negative. The Ki-67 indexes of the 2 patients were 80% and 60%, respectively. Conclusions: The accurate diagnosis of primary ovarian angiosarcoma requires careful histomorphologic examination and reasonable immunohistochemistry staining.

Ovary; Angiosarcoma; Immunohistochemistry; Pathology

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