软组织非典型性骨化性纤维黏液样瘤一例附文献回顾

目的:探讨非典型性或恶性骨化性纤维黏液样瘤(OFMT)的临床、病理特征,诊断及其组织学分化方向。方法:报道1例左颊部低度恶性的非典型性OFMT,从其病理形态和免疫表型,并回顾以往的文献报道,以加深对其组织学分化的认识。结果:患者女性,20岁。近发现左颊部肿胀伴疼痛。病理活检免疫组化见:CK(-),SMA(+),Vim(+),S100(-),Ki67阳性细胞数50%,P63(-),Des(-),HMB45(-),Calpdsome(+),MelanA(-)。考虑(左颊部)非典型骨化性纤维粘液样肿瘤。结论:OFMT是分化不确定的罕见肿瘤,属中间型肿瘤(偶尔转移型),其中富于细胞和/或核分裂数增多、有不规则骨样基质形成并位于肿瘤中心部,考虑为"非典型性";如有转移则为恶性。OFMT的分化方向显示出软骨样、神经及肌上皮的部分特点,基因分析提示其可能是有基因错位的肿瘤,形成凑合型的实体,有特殊的表型。

One case report of atypical ossifying fibromyxoid tumor in soft tissue

Objective:To explore the clinical and pathological characteristics of atypical/malignant ossifying fibromyxoid tulnor(OFMT) of soft tissue,as well as its diagnosis,differential diagnoses and histological differentiation.Methods :A case of low grade atypical OFMT was reported.Results:A tumor was found in the left cheek of a 20-year-old girl.Immunohistochemical study showed that the tumor cells were positive for vimentin and SMA protein and negative for CK,S100 and P63 protein.Conclusions:OFMT is a rare soft tissue tumor with uncertain differentiation which belongs to intermediate malignancy category(rarely metastasizing).OFMT,showing hypercellular and/or having increased numbers of mitotic figures and deposition of osteoid randomly,often within the center of the lesion,is regarded as atypical variant,and malignant one if that metastasizes.It has been suggested that OFMT has chondroid schwannian and myoepithelial differentiation,however,genetic analysis proposes that it might belong to translocation-associated sarcoma forming a "Scrambled entity" with a unique phenotype.