肢端黏液样炎性纤维母细胞肉瘤2例及文献复习

Acral myxoinflammatory fibroblastic sarcoma: a report of two cases and review of literature

目的 探讨肢端黏液样炎性纤维母细胞肉瘤的临床病理学特征及鉴别诊断。方法 对 2例发生在下肢末端的黏液样炎性纤维母细胞肉瘤进行光镜观察和免疫组化标记 ,并复习文献。结果 2例发生在下肢末端的病程较长的渐进性肿块 ,术后局部复发。镜检 :病变呈多结节状 ,边界不清 ;黏液样基质中见数量不等的各类炎细胞浸润 ,散在或灶性分布梭形、奇异形和多空泡状脂肪母细胞样 3种形态的瘤细胞。免疫表型 :肿瘤细胞Vim弥漫阳性 ,CD6 8和CD34灶性阳性 ,CK、SMA、HHF 35、S 10 0蛋白、CD4 5、CD4 5RO、CD15、CD30均阴性。结论 此病病程较长 ,术后易局部复发 ,是一种低度恶性的肿瘤。鉴于病变黏液样基质及各类炎细胞浸润的背景较为突出 ,而特征性的瘤细胞稀疏 。

Purpose To study the clinical pathological features and differential diagnosis of acral myxoinflammatory fibroblastic sarcoma (AMIFS). Methods Two cases of AMIFS were investigated by light microscopy, immunohistochemistry and literature review. Results Two cases of AMIFS presented clinically as slowly growing painless or painful masses occurred in the distal extremities. Microscopically, the lesions were multinodular, poorly delineated, and characterized by a prominent myxoid matrix containing numerous inflammatory cells. Amidst the inflammatory background were scattered with large, bizarre and multivacuolated lipoblast-like tumor cells (fibroblast). Immunohistochemically, the fibroblast was stained strong positivity for vimentin, focal positive for CD68 and CD34, no expression of S-100 protein, SMA, NF, HHF-35, CK, CD45, CD45RO, CD15 and CD30. Conclusions The clinical course of AMIFS is long, with a tendency of local recurrent after surgical resection. We believe that it is a low-grade sarcoma. Because the tumors are poorly delineated, and characterized by a prominent myxoid matrix containing numerous inflammatory cells, bizarre and bubbly cells, differential diagnosis of AMIFS should include inflammatory lesions, benign and malignant tumors with similar morphology.