Matsukuma Susumu, Koga Ayano, Suematsu Ryohei, Takeo Hiroaki, Sato Kimiya
Department of Pathology, Japan Self-Defense Forces Central Hospital, Tokyo 154-8532, Japan; Health Care Center, Japan Self-Defense Forces Central Hospital, Tokyo 154-8532, Japan.
Department of Pathology, Japan Self-Defense Forces Central Hospital, Tokyo 154-8532, Japan.
Mol Clin Oncol. 2017 Jan;6(1):83-87. doi: 10.3892/mco.2016.1078. Epub 2016 Nov 10.
Lipomatous angiomyofibroblastoma is extremely rare, with only 16 cases reported to date. We herein describe an additional case arising in the left vulvar region of a 49-year-old woman. The resected 5.3-cm yellowish tumor predominantly consisted of fat cells (85% of the tumor), together with medium- and small-sized vessels, multifocal fibrotic areas and pseudoangiomatous spaces. Spindle, rounded, and/or epithelioid tumor cells proliferated in a nest and/or cord-like pattern, or singly within perivascular fibrous tissues and between fat cells. The tumor cells were positive for vimentin, estrogen receptor, progesterone receptor, B-cell lymphoma 2, and CD10, but were negative for desmin, cytokeratin, epithelial membrane antigen, S-100 protein, human melanoma black 45, C-kit and p40. Ultrastructural examination revealed that these tumor cells exhibited fibroblastic characteristics. Lipomatous angiomyofibroblastoma should be discriminated from other lipomatous tumors, including spindle cell lipoma, angiomyolipoma and cellular angiofibroma containing numerous fat cells.
脂肪血管肌纤维母细胞瘤极为罕见，迄今为止仅有16例报道。我们在此描述1例发生于一名49岁女性左侧外阴区域的病例。切除的5.3厘米黄色肿瘤主要由脂肪细胞组成（占肿瘤的85%），伴有中小血管、多灶性纤维化区域及假血管瘤样间隙。梭形、圆形和/或上皮样肿瘤细胞呈巢状和/或条索状增殖，或单个存在于血管周围纤维组织及脂肪细胞之间。肿瘤细胞波形蛋白、雌激素受体、孕激素受体、B细胞淋巴瘤2及CD10呈阳性，但结蛋白、细胞角蛋白、上皮膜抗原、S-100蛋白、人黑色素瘤黑色45、C-kit及p40呈阴性。超微结构检查显示这些肿瘤细胞具有成纤维细胞特征。脂肪血管肌纤维母细胞瘤应与其他含大量脂肪细胞的脂肪瘤性肿瘤相鉴别，包括梭形细胞脂肪瘤、血管平滑肌脂肪瘤及细胞性血管纤维瘤。
