Gordhandas Jeenal, Lin Grace, Tipps Ann M P, Zare Somaye Y
Department of Pathology, University of California San Diego, La Jolla, San Diego, CA, USA.
Case Rep Pathol. 2019 Jul 15;2019:8025103. doi: 10.1155/2019/8025103. eCollection 2019.
Dedifferentiated liposarcomas most commonly arise in the retroperitoneum, accounting for 10% of liposarcomas. Heterologous differentiation occurs in 5-10% of dedifferentiated liposarcomas; however, divergent osteosarcomatous differentiation is rare. We report a rare case of initial presentation of dedifferentiated liposarcoma with osteosarcomatous component as a colonic mass in a 72-year-old man. The tumor is mainly composed of bony trabeculae with intervening highly atypical cells and adjacent high-grade mesenchymal nonlipogenic tumor, as well as areas of well-differentiated liposarcoma. Immunohistochemical studies showed diffuse positivity for SATB2 in the atypical cells and fluorescence in situ hybridization revealed high-level amplification of MDM2 gene, supporting the diagnosis of well-differentiated and dedifferentiated liposarcoma with heterologous osteosarcomatous differentiation.
去分化脂肪肉瘤最常发生于腹膜后,占脂肪肉瘤的10%。5% - 10%的去分化脂肪肉瘤会发生异源性分化;然而,分化为骨肉瘤的情况罕见。我们报告了一例罕见病例,一名72岁男性,最初表现为结肠肿块,病理为伴有骨肉瘤成分的去分化脂肪肉瘤。肿瘤主要由骨小梁、其间的高度非典型细胞和相邻的高级别间叶性非脂肪生成性肿瘤以及高分化脂肪肉瘤区域组成。免疫组织化学研究显示非典型细胞中SATB2弥漫阳性,荧光原位杂交显示MDM2基因高水平扩增,支持诊断为伴有异源性骨肉瘤分化的高分化和去分化脂肪肉瘤。