丛状神经纤维瘤10例临床及组织病理分析

Plexiform Neurofibromas: Clinicopathologic Analysis of 10 Cases

目的进一步了解丛状神经纤维瘤患者的临床表现及组织病理学特征。方法对于1972年1月-2014年6月在本院诊治的10例丛状神经纤维瘤患者的，临床资料进行回顾性分析，并进行文献复习。结果10例丛状神经纤维瘤患者中，男6例，女4例；年龄4—52岁，发病年龄0～51岁，5例出生时即有皮损存在。皮损分布：头颈部6例，下肢3例，躯干1例。均表现为多发皮下结节，7例伴疼痛，7例伴多发牛奶咖啡斑，4例B超检查提示“血管瘤”。病理检查显示：真皮及皮下脂肪可见梭形瘤细胞呈同心圆样或不规则样排列，形成丛状或簇状结构，部分丛状结构内见神经轴索。可见间质黏液增生。结论本病好发于儿童，多数以发现肿块而就诊，常伴有疼痛，与神经纤维瘤病1型关系密切。肿瘤主要由Sehwann细胞、纤维母细胞和神经束膜细胞混合组成。

Objective To further understand the clinical and pathological features of the plexiform neurofibroma （PNF）. Methods Clinical data of 10 patients being diagnosed pathologically as plexiform neurofibroma in the department of dermatology of Xijing Hospital from January 1972 to June 2014 were collected and ana- lyzed. Related literatures were reviewed. Results Of the 10 patients,6 were male,4 were female. The age ranged from 4 to 52 years old. The age of onset ranged from 0 to 51. The lesions of 5 patients were discovered after delivery. In 6 cases, the tumors were located in heads and necks,3 in lower limbs and 1 in trunk. The lesions were multiple subcutaneous nodules ,7 of which were accompanied with pain. Seven cases had multi- ple cafe-au-lait macules. Four patients were diagnosed as ＂ hemangioma＂ by ultrasound examination. The histopathology of neurofibromas was characterized by slender spindle cells arranged circularly or irregularly in dermis and subcutaneous adipose tissue, shaping into plexiform or areatus configurations, in some of which neuraxis could be found. Stromal hyperplasia of mucus could often be seen. Conclusion PNF was more likely to occur in children, the most common complaint leading to seeking medical advice was the discovery of masses which were usually accompanied with pain. PNF was closely related with neurofibromatosis type 1. The tumor mainly consisted of Schwann cells, fibroblasts and perineurium cells.