外阴恶性周围神经鞘膜瘤：一种多模式治疗方法。
Malignant peripheral nerve sheath tumor of the vulva: a multimodal treatment approach.
作者信息
Lambrou Nicholas C, Mirhashemi Ramin, Wolfson Aaron, Thesiger Paul, Penalver Manuel
机构信息
Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, University of Miami/Jackson Memorial Hospital, Holtz Building, Suite 3003, 1611 NW 12th Avenue, Miami, FL 33136, USA.
出版信息
Gynecol Oncol. 2002 May;85(2):365-71. doi: 10.1006/gyno.2002.6600.
BACKGROUND
Malignant peripheral nerve sheath tumors (MPNSTs) are rare in the gynecological population and have a high risk for local and distant failures. Multimodal management of a patient with MPNST of the vulva and review of the literature are outlined.
CASE
A 34-year-old woman presented with a complaint of a rapidly increasing pelvic mass, pain, and difficulty ambulating. A disfiguring 20 x 20-cm vulvar mass was identified and a recurrent MPNST diagnosed. Therapy included external-beam radiation, anterior pelvic exenteration with pelvic reconstruction, and adjuvant chemotherapy without complication.
CONCLUSION
It is recommended that for malignant peripheral nerve sheath tumors of the vulva, complete surgical resection be performed with adjuvant radiation and chemotherapy in selected cases.
背景
恶性外周神经鞘瘤（MPNSTs）在妇科人群中罕见，局部和远处复发风险高。本文概述了一名外阴MPNST患者的多模式治疗及文献复习。
病例
一名34岁女性因盆腔肿物迅速增大、疼痛及行走困难就诊。发现一个20×20cm的外阴肿物，诊断为复发性MPNST。治疗包括外照射放疗、前盆腔脏器清除术加盆腔重建及辅助化疗，无并发症。
结论
对于外阴恶性外周神经鞘瘤，建议在选定病例中进行完整手术切除，并辅以放疗和化疗。
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