An unusual finding of vulvar Ewing sarcoma - a case report and review of literature

Superficial Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) are rare and have a relatively favourable prognosis compared to the osseous or deep soft tissue extraskeletal ES/PNET. ES/PNET arising in the female genital tract are exceptionally rare with only a few cases reported. The authors report an unusual case of a 52-year-old woman with vulvar Ewing sarcoma and compare the clinical course and pathological features with cutaneous ES/PNET. The patient received nine cycles of adjuvant chemotherapy after surgical excision of the tumor almost two years prior. In the most recent imaging, there is no evidence of local recurrence of disease or any distant metastasis. Because of the rarity of the case, the long term outcome and prognosis is unclear at this point. However, given the literature on cutaneous ES/PNET and that the patient’s vulvar Ewing sarcoma is of the cutaneous subgroup of extraskeletal ES/PNET, it is likely that her disease prognosis will be more favourable than non-cutaneous extraskeletal ES/PNET. This case report and review of literature will help clinicians to inform patients of long term outcome of this rare tumor on initial presentation, and to help patients to make decisions regarding offered treatment and follow up.

Ewing sarcoma (ES); Extraskeletal peripheral neuroectodermal tumor (PNET); Cutaneous Ewing sarcoma; Vulvar Ewing sarcoma

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