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软组织多形性透明变性血管扩张性肿瘤:1例报告并文献复习

Pleomorphic hyalinizing angiectatic tumor of soft parts: a case report and literature review.

作者信息

Matsumoto Koshi, Yamamoto Taiich

机构信息

Division of Pathology, Second Hospital of Nippon Medical School, Kawasaki, Japan.

出版信息

Pathol Int. 2002 Oct;52(10):664-8. doi: 10.1046/j.1440-1827.2002.01406.x.

Abstract

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare, recently recognized neoplasm occurring predominantly in the subcutaneous tissue of the lower limbs of adults. We report a case of PHAT in an 83-year-old woman who presented with a 5.0 x 5.0 x 2.0 cm mass in the soft part of her left thigh. Histologically, the tumor was well circumscribed by a thin fibrous capsule and predominantly composed of fusiform cells with eosinophilic cytoplasm and round-to-oval or pleomorphic nuclei. The tumor cells resembled those of malignant fibrous histiocytoma, but differed from them by less prominent mitotic figures. Immunohistochemically, the tumor cells were diffusely and strongly positive for CD34; partially positive for vimentin and CD99 (MIC-2); and negative for epithelial and non-epithelial markers. Ultrastructurally, the tumor cells had pleomorphic cytoplasm and nucleus. Intermediate-sized cytoplasmic filaments were observed in a few tumor cells, but neurosecretory-type granule-like intracytoplasmic organelles were not seen. These findings suggest that this tumor is derived from stromal fibroblast, such as solitary fibrous tumors or giant cell angiofibroma.

摘要

多形性透明变性血管扩张性肿瘤(PHAT)是一种罕见的、最近才被认识的肿瘤,主要发生于成年人下肢的皮下组织。我们报告一例83岁女性的PHAT,患者左大腿软组织有一个5.0×5.0×2.0 cm的肿块。组织学上,肿瘤被一层薄纤维包膜完整包绕,主要由梭形细胞组成,这些细胞胞质嗜酸性,核呈圆形至椭圆形或多形性。肿瘤细胞类似于恶性纤维组织细胞瘤的细胞,但有丝分裂象不如后者明显。免疫组化方面,肿瘤细胞CD34弥漫性强阳性;波形蛋白和CD99(MIC-2)部分阳性;上皮和非上皮标记物阴性。超微结构上,肿瘤细胞的胞质和细胞核多形性。少数肿瘤细胞可见中等大小的胞质细丝,但未见神经分泌型颗粒样胞质内细胞器。这些发现提示该肿瘤起源于间质成纤维细胞,如孤立性纤维瘤或巨细胞血管纤维瘤。

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