This case highlights a rare presentation of uterine angiosarcoma diagnosed via outpatient biopsy and an initial, transient regression of lung metastases with oral progesterone therapy.
Uterine mesenchymal malignancies typically include leiomyosarcoma, carcinosarcoma and endometrial stromal sarcoma, while uterine angiosarcoma is exceptionally rare, with only a few dozen cases reported worldwide. We describe a case of high-grade epithelioid uterine angiosarcoma in a woman in her early 50s, diagnosed via outpatient endometrial pipelle biopsy. MRI and CT demonstrated endometrial and bilateral ovarian masses with multiple cavitating lung nodules, consistent with metastatic disease, stage four B according to the International Federation of Gynaecology and Obstetrics classification (FIGO stage IVB). During oral progesterone therapy for abnormal uterine bleeding, lung metastases initially regressed before subsequently progressing. The patient underwent total abdominal hysterectomy, bilateral removal of fallopian tubes and ovaries and omental biopsy. Histopathology confirmed high-grade epithelioid angiosarcoma with bilateral serous borderline ovarian tumours. Despite surgical management, she experienced rapid disease progression and died 5 months after surgery.
Shamleh et al. (Wed,) studied this question.