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

Abstract

Vulval extraskeletal myxoid chondrosarcoma (EMC) is a rare cause of vulval swelling, reported <10 times in the literature to date. EMC in this location is frequently misdiagnosed due to its rarity, and patients may incur delays in diagnosis and treatment. We herein present the diagnosis and management of the case of vulval EMC in a 42-year-old Caucasian female patient who presented in 2011 with a swelling on the right labium majus. The tumour was initially misdiagnosed as a Bartholin's cyst and managed conservatively. The tumour was ultimately diagnosed as EMC and treated by radical surgical excision and adjuvant radiotherapy. The aim of the present study was to report the results after a long-term follow-up period and review the available relevant literature.

A rare case of vulvar extraskeletal myxoid chondrosarcoma: mimics and diagnostic clues

“…Malignant mesenchymal tumors of the vulva are exceedingly rare, with sarcomas accounting for only 1% to 2% of vulvar cancers. 1 The most common sarcomas of the vulva are leiomyosarcoma, rhabdomyosarcoma, and epithelioid sarcoma. In contrast, only fourteen cases of extraskeletal myxoid chondrosarcoma (EMC) of the vulva have been reported in the literature.…”

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

“…Squamous cell carcinoma is by far the most common malignancy of the vulva, accounting for > 90% of cases. 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).…”

“…This tumor arises more commonly in men, often from the deep soft tissue of the lower extremities and buttocks; however, other locations such as the orbit, shoulder, upper extremity, intra-abdominal wall and genitourinary tract have been reported (4,6). Considering the rarity of this tumor, misdiagnosis is common, and the initial diagnosis can sometimes be delayed because the tumor may present as a slowly growing, painless lump (1).…”

“…EMC is an aggressive malignancy that is prone to local recurrence and approximately half of cases metastasize, usually to the lungs (1). Prognostic factors such as older age, proximal site, tumor size ≥ 10 cm, high cellularity, anaplasia, and > 2 mitoses per 10 high-power fields are associated with decreased survival (9,10).…”

“…Prognostic factors such as older age, proximal site, tumor size ≥ 10 cm, high cellularity, anaplasia, and > 2 mitoses per 10 high-power fields are associated with decreased survival (9,10). First-line treatment is wide surgical resection, with or without adjuvant radiotherapy, as the tumor has a poor responsiveness to chemotherapy (1,6). Despite high metastatic potential, prolonged survival is common with 10-yr survival rates of 65% to 88% (8).…”