Malignant Peripheral Nerve Sheath Tumor
刘正智 夏成青 赵明
更新时间:2020-11-10 11:05:00
同义词(或曾用名):神经源性肉瘤、神经纤维肉瘤、和恶性神经鞘瘤
概述:
发病部位:多起源于颈部,前臂,下肢和臀部的大神经。
诊断要点:
8.可见异源性成分,如横纹肌母细胞、软骨、骨、腺体等;
9. 低级别MPNST显示中等程度的细胞密度,散在的核非典型性, 3个/10HPF<核分裂象<9个10/HPF,无肿瘤性坏死;
10.高级别MPNST显示瘤细胞密度明显增加,弥漫的核非典型性,核分裂象≥10个/10HPF或存在肿瘤性坏死。
免疫组织化学染色:
鉴别诊断:
1、单相型滑膜肉瘤:较难鉴别,因为约30%的单相型滑膜肉瘤可表达S100;结合IHC:EMA\BCL2\CD99\CK7\CK19(+)有助于滑膜肉瘤的诊断,必要时可检测t(X;18)的SYT-SSX1/2融合基因来确诊。
2、富细胞性神经鞘瘤:缺乏恶性细胞学特征;常见血管壁玻璃样变性,血管周围淋巴细胞浸润,泡沫细胞反应;异源性成分不常见;S100弥漫强阳性。
3、平滑肌肉瘤:缺乏稀疏区与致密区相间的趋势,无核扭曲逗点状、波浪状等,S100阴性,SMA阳性。
预后:
治疗:
参考文献:
1.Bates JE et al: Malignant peripheral nerve sheath tumors (MPNST): a SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population. Pediatr Blood Cancer. 61(11):1955-60, 2014
2.Pekmezci M et al: Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas. Mod Pathol. ePub, 2014
3.Thway K et al: Malignant peripheral nerve sheath tumor: pathology and genetics. Ann Diagn Pathol. 18(2):109-16, 2014
4.Endo M et al: Conventional spindle cell-type malignant peripheral nerve sheath tumor arising in a sporadic schwannoma. Hum Pathol. 44(12):2845-8, 2013
5.Kolberg M et al: Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. Neuro Oncol. 15(2):135-47, 2013
6.Park SJ et al: Serum biomarkers for neurofibromatosis type 1 and early detection of malignant peripheral nerve-sheath tumors. BMC Med. 11:109, 2013
7.Stricker TP et al: Expression profiling of 519 kinase genes in matched malignant peripheral nerve sheath tumor/plexiform neurofibroma samples is discriminatory and identifies mitotic regulators BUB1B, PBK and NEK2 as overexpressed with transformation. Mod Pathol. 26(7):930-43, 2013