Suzuki Kayo, Yasuda Taketoshi, Watanabe Kenta, Hori Takeshi, Kanamori Masahiko, Kimura Tomoatsu
Department of Orthopaedic Surgery, University of Toyama, Toyama, Toyama 930-0194, Japan.
Department of Orthopaedic Surgery, Iiyama Red Cross Hospital, Iiyama, Nagano 389-2295, Japan.
Oncol Lett. 2017 Dec;14(6):6789-6794. doi: 10.3892/ol.2017.7056. Epub 2017 Sep 25.
Myxoid liposarcoma (MLPS) is the second most common histologic subtype of liposarcoma. However, cartilaginous differentiation within MLPS is an extremely rare phenomenon, with only 7 cases of MLPS with cartilaginous differentiation reported to date. The majority of MLPS cases show the t(12;16)(q13;p11) translocation, resulting in the () fusion gene. This fusion gene as a hallmark of MLPS is very useful for differential diagnosis from other soft tissue sarcomas, and the associated protein, FUS-DDIT3, performs an important role in the phenotypic selection of targeted multipotent mesenchymal cells during oncogenesis. In this report, a case of MLPS with cartilaginous differentiation that occurred in the thigh of a 44-year-old woman is described. Histopathologically, the tumor was composed of a typical myxoid liposarcoma area and a mature hyaline cartilaginous area. Using fluorescence hybridization analysis, rearrangement of the gene was detected in not only the liposarcomatous area but also in the chondrocytes of the cartilaginous area. Based on these findings, the cartilaginous differentiation area appears to be partially associated with oncogenesis through the specific fusion gene .
黏液样脂肪肉瘤(MLPS)是脂肪肉瘤中第二常见的组织学亚型。然而,MLPS内的软骨分化是一种极为罕见的现象,迄今为止仅有7例伴有软骨分化的MLPS病例报道。大多数MLPS病例显示t(12;16)(q13;p11)易位,产生()融合基因。这种融合基因作为MLPS的标志,对与其他软组织肉瘤的鉴别诊断非常有用,并且相关蛋白FUS-DDIT3在肿瘤发生过程中靶向多能间充质细胞的表型选择中发挥重要作用。在本报告中,描述了1例发生在一名44岁女性大腿的伴有软骨分化的MLPS病例。组织病理学上,肿瘤由典型的黏液样脂肪肉瘤区域和成熟的透明软骨区域组成。使用荧光杂交分析,不仅在脂肪肉瘤区域而且在软骨区域的软骨细胞中均检测到该基因的重排。基于这些发现,软骨分化区域似乎通过特定的融合基因与肿瘤发生部分相关。