肢端黏液炎症性成纤维细胞肉瘤临床病理学特征分析

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45⁶1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%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。

Clinicopathological features of acral myxoinflammatory fibroblastic sarcoma

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.