中华临床医师杂志（电子版）
中華臨床醫師雜誌（電子版）
중화림상의사잡지（전자판）
CHINESE JOURNAL OF CLINICIANS(ELECTRONIC VERSION)
2014年
24期
4392-4395
,共4页
病理学,临床%诊断%肢端黏液炎症性成纤维细胞肉瘤
病理學,臨床%診斷%肢耑黏液炎癥性成纖維細胞肉瘤
병이학,림상%진단%지단점액염증성성섬유세포육류
Pathology,clinical%Diagnosis%Acral myxoinflammatory fibroblastic sarcoma
目的：探讨罕见的肢端黏液炎症性成纤维细胞肉瘤的临床病理学特征。方法回顾分析4例肢端黏液炎症性成纤维细胞肉瘤的临床资料，观察4例肢端黏液炎症性成纤维细胞肉瘤的形态及免疫表型。结果患者男3例，女1例，年龄45～61岁，平均52岁；肢端黏液炎性成纤维细胞肉瘤具有特征性的异型细胞，包括假脂肪母细胞、印戒样细胞及病毒样细胞、神经节细胞和R-S细胞。组织结构由明显黏液样区、纤维透明变性和炎症性病变混杂组成。肿瘤细胞表达vim阳性， CD34、SMA和CD68部分阳性，S-100和CD30均为阴性，Ki-67指数3例10%～15%，1例约为25%。结论肢端黏液炎症性成纤维细胞肉瘤是一种低度恶性的罕见肿瘤，极易与良性肿瘤混淆，因组织学多样而易于误诊，掌握其共同的特征性方能正确诊断。
目的：探討罕見的肢耑黏液炎癥性成纖維細胞肉瘤的臨床病理學特徵。方法迴顧分析4例肢耑黏液炎癥性成纖維細胞肉瘤的臨床資料，觀察4例肢耑黏液炎癥性成纖維細胞肉瘤的形態及免疫錶型。結果患者男3例，女1例，年齡45～61歲，平均52歲；肢耑黏液炎性成纖維細胞肉瘤具有特徵性的異型細胞，包括假脂肪母細胞、印戒樣細胞及病毒樣細胞、神經節細胞和R-S細胞。組織結構由明顯黏液樣區、纖維透明變性和炎癥性病變混雜組成。腫瘤細胞錶達vim暘性， CD34、SMA和CD68部分暘性，S-100和CD30均為陰性，Ki-67指數3例10%～15%，1例約為25%。結論肢耑黏液炎癥性成纖維細胞肉瘤是一種低度噁性的罕見腫瘤，極易與良性腫瘤混淆，因組織學多樣而易于誤診，掌握其共同的特徵性方能正確診斷。
목적：탐토한견적지단점액염증성성섬유세포육류적림상병이학특정。방법회고분석4례지단점액염증성성섬유세포육류적림상자료，관찰4례지단점액염증성성섬유세포육류적형태급면역표형。결과환자남3례，녀1례，년령45～61세，평균52세；지단점액염성성섬유세포육류구유특정성적이형세포，포괄가지방모세포、인계양세포급병독양세포、신경절세포화R-S세포。조직결구유명현점액양구、섬유투명변성화염증성병변혼잡조성。종류세포표체vim양성， CD34、SMA화CD68부분양성，S-100화CD30균위음성，Ki-67지수3례10%～15%，1례약위25%。결론지단점액염증성성섬유세포육류시일충저도악성적한견종류，겁역여량성종류혼효，인조직학다양이역우오진，장악기공동적특정성방능정학진단。
Objective To introduce and discuss the clinicopathological features of rare acral myxoinflammatory fibroblastic sarcoma. Methods The clinical and pathologic findings of 4 cases of acral myxoinflammatory fibroblastic sarcoma were evaluated. Hematoxylin and eosinstain, immunohistochemistry and histochemistry were performed on the archival paraffin sections. Results Amongst the 4 patients studied, 3 were males and 1 was female. Their age at the time of operation ranged from 45 to 61 years (mean=52 years). Acral myxoinflammatory fibroblastic sarcoma showed distinctive atypical cells, including pseudoli poblasts, signetring like cells and resembling virocytes, ganglion cells or R-S like cells. The architecture of acral myxoinflammatory fibroblastic sarcoma was characterized by themixture of prominent myxoid areas, fibrous hyalinede generation zone and prominent inflammatory change. The tumor cells were positive for vimentin with locally positive for CD34, SMA and CD68. Ki-67 proliferation index was 10%-15% in 3 cases and 25%in 1 case. Conclusions Acral myxoinflammatory fibroblastic sarcoma is a rare sarcoma, which is often confused with benign lesions. They can be mistaken as other neoplasms due to the morphologic heterogeneity present. Thorough understanding of the laboratory investigation results is helpful in arriving at the correct diagnosis.
