副脊索瘤一例报告并文献综述_李远.docVIP

· 76· 中国骨肿瘤骨病2007年4月第6卷第2期 Chin J Bone Tumor Bone Disease, April 2007, Vol 6, No. 2 · 论著· 副脊索瘤一例报告并文献综述 李远 邓志平 刘文生 牛晓辉 作者单位：100035北京，北京积水潭医院骨肿瘤科 【摘要】 目的 介绍一种罕见软组织肿瘤－副脊索瘤的临床、病理特点及治疗原则。方法 我院收治一 位60 岁女性患者，右足跖骨间软组织肿瘤。临床经X 线、CT、MRI 等检查，确定肿瘤位于右足第 2、3 列 跖骨之间累及跖骨。穿刺活检报告提示软组织低度恶性肿瘤，组织学诊断考虑副脊索瘤可能。遂行肿瘤广泛 切除，同时切除右足第 2、3 列跖骨、趾骨。结果 术后大标本病理检查，HE染色肿瘤分叶状富含黏液，细 胞呈梭形及星形，细胞核轻度异型性。免疫组化结果：Vim（++），SMA（++），Actin（+），S-100（+）， Ki-67（+），Collagen-II（+），Syn（－），CK（－），34 β E12（－）。最终病理论断副脊索瘤。结论 副 脊索瘤是一种罕见的软组织肿瘤，其临床表现及影像学无明显特异性，需要病理常规染色及免疫组化染色与 脊索瘤和黏液样软骨肉瘤鉴别。其治疗方法主要以手术治疗为主，手术需要外科边界达到广泛切除，术后仍 可能局部复发，少数出现转移。 【关键词】 副脊索瘤；诊断；外科治疗 Parachordoma of foot-A case report and literature reviLeIwYuan, DENG Zhiping, LIU Wensheng, et al. Department of Orthopeadic, Oncology, Beijing Jishuitan Hospital, Beijing 100035, China 【Abstract】 Objective To introduce the clinical presentation, pathological characteristic and treatment principle of a rare kind of soft tissue tumor-parachordoMmeat.hods A 60-year-old female had a soft tumor occurring between the second and third metatarsal bones and involving a part of metatarsal bones. The biopsy indicated the tumor had the possibility of being parachordoma. The tumor was en-bloc resected with the sexond and third metatarsal bones to achieve wide marginsR.esults Histologically, the tumor consisted of spindly and stellate cells in abundant myxoid matrix. The nuclei presented mild atypia. Immunohistochemically,the tumor were positive for S-100 protein, vimentin (Vim), smooth muscle actin(SMA), Actin, Ki-67 and Collagen-II. The negative immunoreactions of the tumor with cytokeratin(CK), synaptophysin(Syn) and 3§4E12.Conclusions Parachordoma is a very rare soft tissue tumor and only few cases were reported in literature. The differential diagnosis includes chordoma and myxoid chondrosarcoma. The clinical and radiological presentations of Parachordoma are not obviously. The major differential diagnosis depends on histological and immunohistochemical. Parcahordoma is mostly treated