Angiosarcomas are rare malignant tumors of endothelial origin with morphological properties similar to the vascular and lymphatic endothelium. Associated risk factors include chronic lymph edema and previously irradiated areas. Our patient is the first case report of an angiosarcoma of the mons pubis after chemoradiation and the second reported angiosarcoma of the mons. She was a 74-year-old woman who initially presented with stage II keratinizing squamous cell carcinoma of the vulva that underwent neoadjuvant chemoradiation followed by a radical vulvectomy with bilateral inguinal-femoral lymphadenectomy. She presented 4 years later with a lesion on her mons, consistent with an angiosarcoma. Angiosarcomas are rare malignant tumors of endothelial origin with morphological properties similar to the vascular and lymphatic endothelium. Our patient is the first case report of an angiosarcoma of the mons pubis after chemoradiation for vulvar cancer and the second reported angiosarcoma of the mons. Time to presentation was approximately 4 years from the time of completion of chemoradiation. She recurred within 6 months of surgical resection and required a reexcision. She currently is undergoing systemic chemotherapy after being diagnosed with a metastatic pelvic lymph node. As the treatment of vulvar cancer evolves, and more radiation therapy is given, the incidence of angiosarcomas will rise, requiring better diagnostic and treatment protocols.

Primary multicentric cutaneous epithelioid angiosarcoma of the hand. Case report and review of the literature.Handchir Mikrochir Plast Chir. 2004 Oct;36(5):308-12.

CASE: The case of a primary multicentric cutaneous epithelioid angiosarcoma is described. A 39-year-old female patient presented with a tumor on the left distal forearm and the fourth finger of the left hand. Partial amputation of the left hand and local excision of the tumor on the left forearm were performed. For the next three years the patient was free of local or systemic recurrence. After this time period, a left shoulder exarticulation had to be performed because of tumor recurrence in the left axilla. The patient died 64 months after diagnosis. CONCLUSION: Cutaneous epithelioid angiosarcoma is a highly malignant tumor which has only been described once on the hand. The treatment primarily is a surgical one. If possible, the function of the extremity should be preserved as long as possible.

Cutaneous epithelioid angiosarcoma: a clinicopathological study of four cases.Histopathology. 1994 Nov;25(5):421-9.

Four cases of cutaneous epithelioid angiosarcoma are described together with the potential diagnostic trap of mistaking these tumours for poorly differentiated carcinoma or malignant melanoma. The immunophenotypic profile using four endothelial markers showed positive staining in all cases for factor VIII related antigen in a predominantly paranuclear dot-like fashion and for CD31 (JC70); in three cases for CD34 (QB-END/10) and in two cases with UEA-1. All four cases were cytokeratin (CAM 5.2 and AE1/AE3) negative in contrast to the positive staining reported at non-cutaneous sites. Aberrant S-100 protein expression was seen in one case. In two cases subsequent recurrences showed better differentiation than the original tumour. Electronmicroscopy confirmed the absence of non-endothelial lines of differentiation but failed to reveal Weibel-Palade bodies.