软组织非典型性骨化性纤维黏液样瘤临床病理观察  

Atypical ossifying fibromyxoid tumor of soft part:a clinicopathological observation

作　　者：林梅绥[1] 

机构地区：[1]上海交通大学医学院附属第九人民医院病理科,上海200011

出　　处：《诊断病理学杂志》2007年第5期350-352,共3页Chinese Journal of Diagnostic Pathology

摘　　要：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,诊断和鉴别诊断及其组织学分化方向。方法报道1例右上颌窦复发的低度恶性的非典型性OFMT,从其病理形态和免疫表型,并结合最初的误诊进行讨论,以加深对其组织学分化的认识。结果患者男性,60岁。11年前曾行上腭肿瘤切除术,近发现上颌窦肿瘤。术后组织镜检见肿瘤形态前后相同,为黏液纤维性肿瘤伴散在骨样组织,细胞密度不均,有异型,核分裂约2-3个/10HPF。免疫表型主要为vimentin和S-100蛋白（＋）,上皮标记（-）。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。Objective To expound the clinical and pathological characteristics of atypical/malignant ossifying fibromyxoid tumor （OFMT） of soft part, as well as its diagnosis, differential diagnoses and histological differentiation. Methods A recurrent case of low grade atypical OFMT was reported with histological and studies. Drawing a lesson from the initial misdiagnosis, the differentiation spectnlm of OFMT was further clarified. Results A 60-year-old man was found a tumor in the fight maxillary sinus, and he had a history of resection of a tumor in the palate 11 years ago. The two tuners showed the same morphology, a fibromyxoid tumor with osteoid tissues. The tumor composed of some hypereellular areas with atypia and mitotic figures （2 - 3/10HPF）. Immunophenotypes showed that the tumor cells were positive for vimentin and S-100 protein and negative for epithelial markers. Conclusion OFMT is a rare soft tissue tumor of tmcertain 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.

关 键 词：骨化性纤维黏液样瘤 非典型性 S-100蛋白 

分 类 号：R738.104[医药卫生—肿瘤]