乳腺丛状神经纤维瘤1例报道及文献复习

The Plexiform Neurofibroma(PNF) of Breast:A Case Report and Review of Literature

目的：探讨丛状神经纤维瘤的临床病理学特征、诊断和鉴别诊断、生物学行为和治疗。方法：对1例女性乳腺丛状神经纤维瘤患者的病理组织学和免疫表型进行观察,结合临床资料进行分析并复习相关文献。结果：肉眼观：神经干丛状肿大,受累及的神经束扭曲、变形,大小和形状不一,呈“蠕虫袋状”。镜下：瘤细胞排列成丛状结构或多结节状,埋于神经组织的背景中。免疫组织化学：肿瘤中,神经鞘细胞S-100弥漫性阳性,丛状结构内的轴索NSE阳性,神经束衣上皮细胞EMA阳性,神经束衣的纤维母细胞NF阳性,CD68、p53、Ki-67均阴性。结论：丛状神经纤维瘤是一种少见而独特的神经纤维瘤亚型,常发生于神经纤维瘤病Ⅰ型,是诊断神经纤维瘤病Ⅰ型的一个关键指标。丛状神经纤维瘤的诊断主要依据其独特的大体特征和组织学特点,同时与具有丛状结构和/或富于粘液的肿瘤鉴别,免疫表型起关键作用。该肿瘤生物学行为潜在恶性,治疗首选早期手术切除。

Objective： To explore the clinieopathological features, diagnosis and differential diagnosis, biological behavior and the treatment of plexiform neurofiroma（PNF）. Methods： The clinical data and pathologic materials from a women patient with the PNF of breast were reviewed. The sample of the tumor was examined by HE and immunohistochemistry staining, and the related literatures were reviewed. Results： Macroscopically, the nerve trunk plexformly tumefacient, the affected nerve tracts twisted and distorted, and their sizes and shapes were discordant, looked like ＂bag of worms＂. Microscopically, the tumor cells emerged fascieulate or multi-no- dosity, and buried in the background of nervous tissue. Using Immtmohistochemical staining, the neurilemma cells were diffusely positive for S-100, the neuraxons in the plexiform fasciculates were positive for NSE, the perineurium endothelial ceils were positive for EMA, and perineurial fibroblasts were positive for NF and negative for CD68, p53 and Ki-67. Conclusion： PNF is a rare distinctive variant of neurofibroma, and usually derives from neurofibromatosis type I. It is one of key diagnostic indexes of neurofibromatosis type I. The diagnosis of PNF fundamentally depends on the distinctive macrosoopical features and histological features. Meanwhile, the plexiform tumors and/or the alive with mucus tumors should be looked as principal diseases in differential diagnosis, and the immunophenotypes play a key role. The feature of PNF＇ s biological bebaviour is potentially malignant, and the primary choice is pristine operation excise early.