【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(-),34βE12(-)。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。更多还原

【Abstract】 Objective To introduce the clinical presentation, pathological characteristic and treatment principle of a rare kind of soft tissue tumor-parachordoma. Methods 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 margins. Results 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 34§E12. 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 by surgery, which needs wide margin. The local recurrence may occur and few also can develop metastases.更多还原