An extremely rare elderly case of proximal epithelioid sarcoma of the vulva: case report with a review of literatures

Abstract

We experienced an extremely rare case of proximal epithelioid sarcoma (PES) of the vulva in a 77-year-old woman. After history taking and physical examination, the patient was tentatively diagnosed as having Bartholin’s cyst in the right labium. Based on histopathological and immunohistochemical (IHC) findings, however, a final diagnosis of PES of the vulva was made. After receiving CyberKnife treatment, the patient survived but with recurrent episodes and poor prognosis. In conclusion, our case indicates that patients with PES of the vulva should be appropriately managed with radiotherapy after a differential diagnosis based on histopathological and IHC findings.

Proximal epithelioid sarcoma of the vulva: A case report and review of the literature

“…Vulvar cancer is the fourth most common cause of gynecological malignancy, constituting 5% of all cancers of the female genital tract [1,2] , Among the various histological types of vulvar cancer, squamous cell carcinoma is the most common type and represents 95% of all malignant neoplasms in this gynecological area, followed by melanoma, sarcoma, and basal cell carcinoma [1] . Primary vulvar sarcomas are rare, reaching an incidence of 1.5 to 5% of cancers in this topographic region [2,3] . Epithelioid sarcoma (ES) is a malignant soft tissue neoplasm of uncertain histogenesis, which was established as a distinctive disease by Ezinger in 1970 [3] .…”

“…Epithelioid sarcoma (ES) is a malignant soft tissue neoplasm of uncertain histogenesis, which was established as a distinctive disease by Ezinger in 1970 [3] . Epithelioid sarcomas (ES) are classified into distal, conventional, or classic type and proximal type based on its occurrence in the upper and lower or in the trunk and pubic region, respectively [2][3][4] . The proximal epithelioid sarcoma of the vulva are rare entities, with only 41 cases published in the English literature, and the first of them described by Piver et al in 1972 [2,4] .…”

“…Vulvar sarcoma constitutes 1.5-5% of all malignancies in this gynecologic region [1][2][3][4][5] . Types of sarcomas commonly present in the vulva include leiomyosarcomas, malignant fibrous histiocytoma, malignant rhabdoid tumor, angiosarcoma, rhabdomyosarcoma, and epithelioid sarcomas [1][2][3] .…”

“…Vulvar sarcoma constitutes 1.5-5% of all malignancies in this gynecologic region [1][2][3][4][5] . Types of sarcomas commonly present in the vulva include leiomyosarcomas, malignant fibrous histiocytoma, malignant rhabdoid tumor, angiosarcoma, rhabdomyosarcoma, and epithelioid sarcomas [1][2][3] . Epithelioid Sarcoma is subclassified into distal or proximal type about the involvement of upper and lower extremities or the pubic region and trunk, respectively.…”

“…Epithelioid Sarcoma is subclassified into distal or proximal type about the involvement of upper and lower extremities or the pubic region and trunk, respectively. Proximal type epithelioid sarcoma of the vulva is an exceedingly rare soft tissue tumor, with only 41 cases described in the English literature, Case Report and extremely aggressive with a high rate of local recurrence and distant metastasis [2,4] . The following publication provides the first case of vulvar epithelioid sarcoma reported in our country, Peru and Latin America; and reviews its clinical, histomorphological, treatment, and prognosis of this unusual pathology.…”