Extremely Rare Case of Vulvar Myxoid Epithelioid Sarcoma

Abstract

Epithelioid sarcoma is a distinct sarcoma type with specific morphology and immunophenotype. An epithelioid sarcoma of the vulva is an extremely rare and aggressive tumor and most commonly occurs on the labia majora in women of reproductive age. Only few cases have been reported, especially with the presence of focal myxoid changes. Early diagnosis is difficult because of its benign appearance as a painless subcutaneous nodule. Optimal treatment is not well established due to its rarity. We report a successfully approached case of vulvar epithelioid sarcoma that occurred in a 34-year-old female patient, treated with wide local excision, and review of the current medical literature.

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

“…Therefore, it can be diagnosed using IHC [ 6 ]. Our case showed high IHC positivity rates for CD34, CK-PAN, and VT, which is in agreement with the results of the previous studies in this series [ 11 , 12 ].…”

“…Adjuvant radiotherapy is recommended for high-grade tumors or cases with inadequate surgical margins [ 13 ]. We did not perform chemotherapy for our case, in accordance with previous studies that showed that the treatment effects of postoperative chemotherapy are still controversial [ 7 , 11 - 14 ]. Argenta et al [ 13 ] performed adjuvant chemotherapy for 7 of 31 patients with vulva ES or malignant rhabdoid tumor and reported that of the 7 patients, 3 were disease-free at 8, 11, and 21 months but the remaining 4 died of disseminated disease within 8 months of diagnosis.…”

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] .…”

“…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.…”