外阴周围神经外胚层肿瘤：一例报告。
Peripheral neuroectodermal tumor of the vulva: a case report.
机构信息
Department of Obstetrics & Gynaecology, All India Institute of Medical Sciences Ansari Nagar, New Delhi, India.
出版信息
J Low Genit Tract Dis. 2010 Jan;14(1):59-62. doi: 10.1097/LGT.0b013e3181b0f8f3.
BACKGROUND
Peripheral neuroectodermal tumors arising from vulva are extremely rare; only 12 cases have been reported so far. These tumors belong to the Ewing sarcoma family of tumors. They occur in bones and variety of soft tissues of chest wall, pelvis, paravertebral region, and lower extremities. Being aggressive tumors, they have poor prognosis.
CASE
We report a case of a 20-year-old woman who presented with a 20 x 15 x 10-cm pedunculated mass arising from the right labium majus. Judicious use of a panel of markers led to diagnosis of peripheral neuroectodermal tumor. The patient died of metastatic disease.
CONCLUSIONS
Judicious use of a panel of markers and a molecular test, if necessary, helps in confirming the diagnosis. Surgery followed by chemotherapy with or without radiation is the treatment of choice.
背景
外阴起源的外周神经外胚层肿瘤极为罕见；迄今为止，仅报告了 12 例病例。这些肿瘤属于尤文肉瘤家族肿瘤。它们发生在骨骼和胸壁、骨盆、脊柱旁区域和下肢的各种软组织中。作为侵袭性肿瘤，它们预后不良。
病例
我们报告了一例 20 岁女性，她出现了一个 20 x 15 x 10 厘米的带蒂肿块，位于右侧大阴唇。明智地使用一组标志物导致了外周神经外胚层肿瘤的诊断。该患者死于转移性疾病。
结论
明智地使用一组标志物和分子检测（如有必要）有助于确认诊断。手术加化疗，或有或无放疗，是治疗的选择。
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