Vulvar “proximal-type” epithelioid sarcoma: report of a case and review of the literature

Abstract

BackgroundThe “proximal-type” epithelioid sarcoma is a very rare kind of mesenchimal tumor characterized by the difficulty in histological diagnosis and the very aggressive biological behavior.CaseWe report of a case of a 63 years old woman with a vulvar “proximal-type” epithelioid sarcoma that underwent a radical surgical staging followed by an adjuvant radiotherapy. She is on follow-up care for 14 months and there is no clinical evidence of disease.ConclusionEven if quite rare the proximal type epithelioid sarcoma should be regarded as a separate entity of particularly aggressive biologic behaviour. Its diagnosis attracts controversies and criticism related to the surgical approach and the choice of an adjuvant therapy.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1508554852942125

Proximal-type epithelioid sarcoma—unusual presentation: unilateral vulvar mass

“…Epithelioid sarcoma is a very uncommon soft tissue tumour of mesenchymal origin 1 4 5 7. These tumours can be divided into distal or proximal types, occurring, respectively, at upper and lower extremities or in the trunk, abdomen or pelvis 1 4.…”

“…Until 2012, only about 29 cases had been reported in the English literature 5. It usually occurs, similarly to other sarcomas, between the ages of 21 and 57 years, with 35 years being the mean age 1 3…”

“…Locoregional lymph nodes can be involved 6. Regional metastasis can occur via lymphatic spread or through direct extension, while distant metastases are due to haematogenous dissemination to lung or liver 5 6. These tumours characteristically arise out of satellite lesions between the primary tumour and the regional lymph nodes 1 3.…”

“…Until now, it has not been proven to be beneficial on local or distant recurrence related to lymphadenectomy 1–5 7. Adjuvant radiotherapy is advisable in the presence of a high-grade tumour or positive margins 2 5. The beneficial effect of adjuvant chemotherapy is not well established, but is recommended in cases of disseminated disease 5–7.…”

“…Epithelioid sarcoma is a very aggressive soft tissue tumour of mesenchymal origin and was first recognised as a distinct entity in 1970 by Enzinger 3–7. Its typical presentation is characterised by a painless nodule or mass 1 5. When located in the Bartholin’s glands area it can be mistaken for a wide variety of benign lesions, leading to diagnosis at later stages 3–6.…”