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细胞血管纤维瘤中的肉瘤样转化:病例报告。

Sarcomatous transformation in a cellular angiofibroma: a case report.

机构信息

Department of Pathology, University of Vermont, Burlington, VT 05401, USA.

出版信息

J Clin Pathol. 2009 Oct;62(10):945-7. doi: 10.1136/jcp.2009.067124.

Abstract

Cellular angiofibroma is a rare benign mesenchymal tumour of middle-aged adults. This tumour is usually located in the vulvovaginal or inguinoscrotal region. This report describes the case of a patient with a 3.5 cm subcutaneous mass, 2 cm below the left anterior superior iliac spine. Grossly, the mass had tan-white cut surface with a 1.5 cm tan-yellow, whorled, well-delineated nodule. Histologically, the tumour was composed primarily of cytologically bland spindle cells set in a collagenous stroma, with multiple dilated vessels. Other areas showed an abrupt transition to hypercellular sarcomatous elements, including pleomorphic cells with high mitotic activity. The tumour cells were diffusely positive for vimentin and factor XIIIa, and weakly positive for CD34. The patient did not develop any recurrences or metastases, and expired 3 years later of metastatic poorly differentiated carcinoma of unknown origin. This is believed to be the first reported case of sarcomatous transformation in a cellular angiofibroma.

摘要

细胞性血管纤维瘤是一种罕见的良性间叶组织肿瘤,主要发生于中青年人。此肿瘤通常位于外阴阴道或腹股沟阴囊区。本报告描述了 1 例患者,其左侧髂前上棘下 2cm 处有 3.5cm 皮下肿块。大体上,肿块切面呈灰白色,有 1.5cm 大小的灰黄色、旋涡状、边界清楚的结节。组织学上,肿瘤主要由细胞温和的梭形细胞组成,位于胶原基质中,伴多发扩张的血管。其他区域表现为突然向多形性细胞、高有丝分裂活性的肉瘤样成分过渡。肿瘤细胞弥漫性表达波形蛋白和因子 XIIIa,局灶性弱阳性表达 CD34。患者未出现复发或转移,3 年后死于来源不明的转移性低分化癌。这被认为是首例细胞性血管纤维瘤发生肉瘤样转化的报道。

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