15例骨和软组织内间叶性软骨肉瘤临床病理分析

Clinicopathology analasis of 15 cases of primary mesenchymal chondrosarcoma in bone and soft tissue

目的探讨原发间叶性软骨肉瘤的临床特点、诊断和鉴别诊断依据。方法复习本院15例骨及软组织原发间叶性软骨肉瘤的临床资料和病理学特征(包括对 HE 染色、免疫组化染色切片的观察分析),对14例行密切随访。结果 10年内15例间叶性软骨肉瘤占同期软骨肉瘤总数的9．4%,占小细胞恶性肿瘤的0．3%,占全部骨肿瘤的7．4%。患者年龄14～70岁,平均42岁。86%的患者在11～40岁之间,无明显性别差异。其中骨内发病13例,软组织内原发肿瘤2例。2 例有外伤史。诊断初期多中心性发病者5例,患者多主诉局部疼痛伴肿胀。影像学特征与普通型软骨肉瘤相似,表现为伴有点状钙化的大片溶骨性破坏。组织学表现通常具有双相性,表现为间变的未分化间叶性小细胞与分化较好的分叶状肿瘤性软骨及软骨样基质并存。两种组织之间界限较清晰,移行区可见肿瘤细胞成软骨现象。免疫组织化学显示小细胞区 CD99阳性,CollagenⅡ在小细胞区和软骨区均阳性,部分病例 CgA、Ki-67、P53有阳性表达,Syn 在少数病例有阳性表达。本组患者最长在确诊后7年内死亡,最短在9个月内死亡,其中2例70岁以上的患者均在2年内死于肿瘤转移。结论间叶性软骨肉瘤少见且预后差。虽然具有典型组织学特征,其诊断和鉴别诊断仍很困难。特别是在穿刺标本中,未穿到软骨性成分时与其他小细胞恶性肿瘤鉴别困难,未穿到小细胞成分时与其他类型软骨肉瘤鉴别困难。诊断中除仔细观察 HE 和免疫组化染色切片外,还需密切结合临床资料和影像学特征。

Objective To investigate the clinical and pathologic characteristics, diagnosis and differential diagnosis of primary bone and soft tissue mesenchymal chondrosarcoma. Methods Clinical data and pathologic slides （HE and immunohistochemical stains） of 15 cases of primary bone and soft tissue mesenchymal chondrosarcoma was reviewed. Results These 15 cases of mesenchymal chondrosarcoma rep- resent 9.4% of all chondrosarcomas patients admitted in authors hospital between 1995 and 2005. The ages of the patients ranged from 14 to 70 years and approximately 80% of them were in the second to forth decades of life. Tow cases arose in soft tissue and five cases involved multiple skeletal sites. Pain and swelling are the main complains. The bone lesions resembled classic chondrosarcomas in image. Histologically, atypical undifferentiated small cells and islands of matured chondroid matrix typically presented a bimorphic appearances and there is a distinct border between these two materials. Prognosis for patients with mesenchymal chondrosarcoma is usually poor and especially in the older patients. Conclusions Mesenchymal chondrosarcoma is a rare disease with poor prognosis. It is nessesary to distinguish it from Ewing＇s sarcoma and small cell osteosarcoma, and other small cell malignant tumors. His- tological characteristics and immunohistochemical results are helpful for diagnosis and differentiated diagnosis.