Lane K L, Shannon R J, Weiss S W
Department of Pathology, University of Michigan Hospitals, Ann Arbor 48109-0054, USA.
Am J Surg Pathol. 1997 Dec;21(12):1481-8. doi: 10.1097/00000478-199712000-00011.
We report the findings of 19 cases of a previously undescribed spindle cell tumor of soft tissues that resembles a low-grade fibromyxoid sarcoma but contains distinctive rosettelike structures. The tumors occurred principally as a painless, slowly growing, deeply situated mass of the proximal extremities in young to middle-aged adults (age range 14-65 years; mean 38). Although grossly circumscribed, the tumors had infiltrative borders microscopically and were composed of bland spindled cells situated in a hyalinized to myxoid stroma. The most characteristic feature of the tumor was scattered large rosettelike structures that often merged with serpinginous areas of dense hyalinization. The rosettes consisted of a central collagen core surrounded by a rim of rounded cells morphologically and immunophenotypically different from the cells of the spindled stroma. These cells expressed a number of antigens, including S-100 protein, neuron-specific enolase, and leu 22, in contrast to the stroma, which usually lacked these antigens. Of the 12 patients with available follow-up information, one patient treated with simple excision clinically developed local recurrence of the tumor 20 months after initial biopsy. No other recurrences were reported during the limited follow-up period, and no patient developed metastatic disease. However, the favorable prognosis of the patients in our series to date may relate to the limited follow-up period (approximately 3 years), as well as initial treatment by wide excision in nearly half of the patients. We regard the hyalinizing spindle cell tumor with giant rosettes as a distinctive type of low-grade fibroblastic tumor that with time may prove to behave similar to a low-grade fibromyxoid sarcoma and, hence, to represent an unusual variant thereof.
我们报告了19例一种先前未描述的软组织梭形细胞肿瘤的研究结果，该肿瘤类似于低度纤维黏液样肉瘤，但含有独特的玫瑰花结样结构。这些肿瘤主要表现为无痛、生长缓慢、位于近端肢体深部的肿块，发生于青年至中年成人（年龄范围14 - 65岁；平均38岁）。尽管大体上边界清晰，但肿瘤在显微镜下边界呈浸润性，由位于玻璃样变至黏液样基质中的温和梭形细胞组成。肿瘤最具特征性的表现是散在的大玫瑰花结样结构，这些结构常与致密玻璃样变的蜿蜒区域融合。玫瑰花结由中央胶原核心和一圈圆形细胞组成，这些圆形细胞在形态和免疫表型上与梭形基质细胞不同。与通常缺乏这些抗原的基质相反，这些细胞表达多种抗原，包括S - 100蛋白、神经元特异性烯醇化酶和leu 22。在12例有随访信息的患者中，1例接受单纯切除治疗的患者在初次活检后20个月临床出现肿瘤局部复发。在有限的随访期内未报告其他复发情况，也没有患者发生转移。然而，我们系列患者目前良好的预后可能与随访期有限（约3年）以及近一半患者最初采用广泛切除治疗有关。我们认为伴有巨大玫瑰花结的玻璃样变梭形细胞肿瘤是一种独特类型的低度成纤维细胞肿瘤，随着时间推移可能表现得与低度纤维黏液样肉瘤相似，因此代表其一种不寻常的变异型。
