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梭形细胞横纹肌肉瘤:简要诊断性综述及鉴别诊断。

Spindle cell rhabdomyosarcoma: a brief diagnostic review and differential diagnosis.

机构信息

Department of Pathology, University of Tennessee Medical Center Knoxville, Knoxville, Tennessee 37920, USA.

出版信息

Arch Pathol Lab Med. 2013 Aug;137(8):1155-8. doi: 10.5858/arpa.2012-0465-RS.

Abstract

Spindle cell rhabdomyosarcoma is a rare variant of embryonal rhabdomyosarcoma that has a predilection for young males and most commonly involves the paratesticular region followed by head and neck. Histopathology demonstrates elongated spindle cells with fusiform to cigar-shaped nuclei and indistinct eosinophilic cytoplasm arranged in fascicles or whorls. Although the tumor demonstrates increased cellularity and moderate atypia, the microscopic and architectural patterns can allow this tumor to be confused with multiple entities, such as leiomyosarcoma, spindle cell carcinoma, desmoplastic melanoma, or fibrosarcoma, with important therapeutic implications. Immunohistochemical workup demonstrates sarcomeric differentiation with reactivity for desmin, myogenin, and MyoD1 markers. Compared with other subtypes, the spindle cell variant in children is associated with a favorable outcome; however, in the adult population there does not appear to be any prognostic advantage.

摘要

梭形细胞横纹肌肉瘤是胚胎性横纹肌肉瘤的一种罕见亚型,好发于年轻男性,最常累及精索部位,其次为头颈部。组织病理学表现为梭形细胞拉长,纺锤形至雪茄形细胞核,嗜酸性细胞质不明显,呈束状或漩涡状排列。虽然肿瘤细胞密度增加,异型性中等,但显微镜下形态和组织结构模式可能会使该肿瘤与多种实体肿瘤混淆,如平滑肌肉瘤、梭形细胞癌、促结缔组织增生性黑色素瘤或纤维肉瘤,这具有重要的治疗意义。免疫组织化学检查显示肌源性分化,表达结蛋白、肌球蛋白和 MyoD1 标志物。与其他亚型相比,儿童的梭形细胞变体与良好的预后相关;然而,在成年人群中,似乎没有任何预后优势。

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