Extraskeletal myxoid chondrosarcoma of the vulva: A case report

Abstract

Extraskeletal myxoid chondrosarcoma (ESMC) of the vulva is an extremely rare tumor and currently, there is little available information on its biological behavior and treatment strategy. The present study reports a case of recurrent ESMC of the vulva in a 32-year-old female. The patient presented with an increasingly painful mass of the right vulva, at the site of an exision which had been performed 7-months previously. The tumor mass was histopathologically diagnosed as primary ESMC of the vulva and subsequently, vulvectomy was performed. Cytological examination showed negative surgical margins. Intraoperative radiation therapy at a single dose of 10 Gy was administered to the bed of the removed tumor. The patient refused chemotherapy and five months after surgery, a new lesion was identified in the inguinal region. Bilateral inguinal-femoral lymph node dissection was performed and external beam radiation therapy at a dose of 40 Gy was administered to the inguinal region. Follow-up examination seven months after surgery revealed no evidence of disease progression and at present, the patient remains alive. This study highlights the importance of analyzing each clinical case of ESMC as this may lead to the development of guidelines for the optimal treatment of this rare tumor.

Myoepithelioma-like tumor of the vulvar region: a case report in China and review of the literature

“…The differential diagnosis of MELTVR also includes extraskeletal myxoid chondrosarcoma (EMC) due to its uniform, loosely cohesive tumor cells in a myxoid matrix. EMC is an extremely rare subtype of vulvar sarcoma that is consistently positive for vimentin, variable positivity for S100 protein, neuron-specific enolase and synaptophysin, completely negative for CK [10][11][12][13][14][15][16], and harhor EWSR1-NR4A3 fusion in about 65% of cases [17]. However, the present case showed S100 negativity, EMA, ER positivity, and EWSR1 was intact showed by FISH assay.…”

A 5‑year follow‑up of vulval swelling due to extraskeletal myxoid chondrosarcoma: A rare case report

“…Extraskeletal myxoid chondrosarcoma (EMC) is a rare subtype of sarcoma, which was first described in 1972 by Enzinger and Shiraki (4). Since that time, there have been few reported cases of this rare tumour in the literature, and fewer still reported to affect the female genital tract (Table I) (5)(6)(7)(8)(9)(10). EMCs tend to affect patients in the 6th decade of life and have a predilection for the deep soft tissue of the lower extremities.…”

“…Histologically, these tumours are described as having a multinodular architecture with mucoid material and foci of haemorrhage, so that they may be initially misdiagnosed as organised haematomas (10). Misdiagnosis is common (8) and preoperative diagnosis is very difficult due to the rarity of this tumour and its presentation as a slow-growing painless lump (6).…”

Extraskeletal Myxoid Chondrosarcoma of the Vulva Confirmed by EWSR1 FISH: A Case Report and Review of the Literature

“…Sarcomas account for only 3% of cancers of the female genital tract, and only 1% to 3% of these cases affect the vulva (1). The most common sarcomas of the vulva include rhabdomyosarcoma, leiomyosarcoma, epithelioid sarcoma, alveolar soft part sarcoma, and liposarcoma (2). Extraskeletal myxoid chondrosarcoma (EMC) is very rare in the vulva, with <10 cases reported in the literature.…”

“…EMC was first described by Enzinger and Shiraki 3 in1972 as a separate pathologic entity from chondrosarcoma of bone. In 1994, it was included into the group of soft tissue neoplasms of uncertain differentiation because despite its name, a derivation from cartilage has not been proven 2,4. EMC is a rare subtype that comprises <3% of all sarcomas 5.…”