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恶性混合性苗勒管肿瘤继发中枢神经系统转移:病例报告及治疗综述

CNS metastasis secondary to malignant-mixed Müllerian tumor: case report and review of therapeutics.

作者信息

Healy Vincent, O'Halloran Philip, O'Brien Sorca, Beausang Alan, Caird John

机构信息

Department of Neurosurgery, National Neurosurgery Centre, Beaumont Hospital, Dublin 11, Ireland.

Department of Gynaecological Oncology, UCD School of Medicine, MMUH, Dublin 7, Ireland.

出版信息

CNS Oncol. 2017 Oct;6(4):315-323. doi: 10.2217/cns-2017-0015. Epub 2017 Oct 9.

Abstract

This paper reviews CNS involvement secondary to malignant-mixed Müllerian tumor or uterine carcinosarcoma, a rare aggressive biphasic Müllerian tumor. We report a cerebellar metastasis with epithelial and mesenchymal components, demonstrating heterologous rhabdomyogenic and chondroblastic differentiation. The patient had undergone total abdominal hysterectomy and bilateral salpingo-oophorectomy for palliation of symptomatic chemotherapy-resistant node-positive disease. CNS involvement is rare, and prognostically poor, and suggestively poorer in predominantly sarcomatous metastases. Multimodal therapy is indicated; in solitary metastases, surgical resection or stereotactic radiosurgery is included, followed by whole brain radiotherapy. In unresectable brain metastases, stereotactic radiosurgery and whole brain radiotherapy warrant consideration in up to 2-3 metastases. In multiple metastases, palliative steroid therapy or cranial irradiation may be considered. Combination or platinum-based chemotherapy (i.e., ifosfamide-paclitaxel or carboplatin-paclitaxel) is indicated in all stages, with a role in both disease cure and control-directed management. Targeted therapeutics have thus far not demonstrated significant clinical efficacy.

摘要

本文综述了继发于恶性混合性苗勒管肿瘤或子宫癌肉瘤(一种罕见的侵袭性双相苗勒管肿瘤)的中枢神经系统受累情况。我们报告了一例具有上皮和间叶成分的小脑转移瘤,显示出异源性横纹肌母细胞和成软骨细胞分化。该患者因有症状的化疗耐药性淋巴结阳性疾病而接受了全腹子宫切除术和双侧输卵管卵巢切除术以缓解症状。中枢神经系统受累罕见,预后较差,在主要为肉瘤性转移的情况下提示预后更差。需要多模式治疗;对于孤立性转移瘤,包括手术切除或立体定向放射外科治疗,随后进行全脑放疗。对于不可切除的脑转移瘤,在多达2 - 3个转移瘤的情况下,立体定向放射外科和全脑放疗值得考虑。对于多发转移瘤,可考虑姑息性类固醇治疗或颅脑照射。在所有阶段均需采用联合或铂类化疗(即异环磷酰胺 - 紫杉醇或卡铂 - 紫杉醇),其在疾病治愈和控制导向治疗中均有作用。迄今为止,靶向治疗尚未显示出显著的临床疗效。

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