Jurcić Vesna, Zidar Andreja, Montiel Maria Delia Perez, Frković-Grazio Snjezana, Nayler Simon J, Cooper Kumarasen, Suster Saul, Lamovec Janez
Department of Pathology, University of Ljubljana, Slovenia.
Ann Diagn Pathol. 2002 Oct;6(5):272-80. doi: 10.1053/adpa.2002.35738.
We report on nine new cases of myxoinflammatory fibroblastic sarcoma; in six of them the location of the tumor was distal (acral), and proximal in three (forearm, arm, and thigh). Tumors varied in size from 1.5 to 18 cm, were well-circumscribed, yellow-tan, and focally myxomatous. Histologically, they were similar in appearance and showed vaguely lobular architecture and oval, spindle, and epithelioid neoplastic cells with scattered, focally aggregated inflammatory cells. In all cases, in different numbers, bizarre giant cells with large, lobulated, or multiple nuclei were also admixed, some of them morphologically imitating Reed-Sternberg cells, lipoblasts, or ganglion cells; they showed distinct nucleoli or intranuclear inclusions. Myxoid areas were always present, to different extent. Immunohistochemically, tumor cells were uniformly positive for vimentin; some cells were also positive for CD68 and CD34. Ultrastructurally, tumor cells were nondescript, consistent with fibroblastic origin. On flow cytometry, two of the examined cases showed diploid pattern with low S-phase fraction. In none of the cases, metastases were observed, in one case the tumor recurred 5 years following surgery. We conclude that myxoinflammatory fibroblastic sarcoma is a distinct soft tissue tumor of low-grade malignancy and, until now, described only in extremities, although not confined to acral sites.
我们报告了9例黏液炎性成纤维细胞肉瘤新病例；其中6例肿瘤位于远端（肢端），3例位于近端（前臂、上臂和大腿）。肿瘤大小从1.5厘米到18厘米不等，边界清晰，呈黄棕色，有局灶性黏液样变。组织学上，它们外观相似，呈模糊的小叶状结构，有椭圆形、梭形和上皮样肿瘤细胞，伴有散在的、局灶性聚集的炎性细胞。在所有病例中，均可见数量不等的具有大的、分叶状或多个核的怪异巨细胞，其中一些在形态上类似里-施（Reed-Sternberg）细胞、脂肪母细胞或神经节细胞；它们有明显的核仁或核内包涵体。黏液样区域总是不同程度地存在。免疫组化显示，肿瘤细胞波形蛋白均呈阳性；部分细胞CD68和CD34也呈阳性。超微结构上，肿瘤细胞无特征性表现，符合成纤维细胞起源。流式细胞术检测显示，2例病例为二倍体模式，S期分数低。所有病例均未观察到转移，1例病例术后5年复发。我们得出结论，黏液炎性成纤维细胞肉瘤是一种独特的低度恶性软组织肿瘤，目前仅在四肢有报道，尽管并不局限于肢端部位。
