Extensive lipoma-like changes of myxoid liposarcoma: morphologic, immunohistochemical, and molecular cytogenetic analyses.
Extensive lipoma-like changes of myxoid liposarcoma: morphologic, immunohistochemical, and molecular cytogenetic analyses.
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DOI：
10.1007/s00428-015-1721-z
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发表时间：
2015-04
期刊：
影响因子：
3.5
通讯作者：
Nabeshima, Kazuki
中科院分区：
文献类型：
--
作者：
Iwasaki, Hiroshi;Ishiguro, Masako;Nishio, Jun;Aoki, Mikiko;Yokoyama, Ryohei;Yokoyama, Koichiro;Taguchi, Kenichi;Nabeshima, Kazuki
粘液样脂肪肉瘤（MLSs）伴广泛性脂肪瘤样改变（MLSLC）是罕见的，通常很难将其与分化良好的脂肪肉瘤（LS）/去分化LS（WDLS/DDLS）伴粘液样改变区分开来。对于这些肿瘤的特征，我们研究了8个MLSLCs，11个普通MLSs，4个WDLSs和6个DDLS。MLSLC和普通MLS的细胞遗传学特征为t（12;16）（q13;p11）和FUS-DDIT 3融合基因，而WDLS/DDLS缺乏融合基因，但具有巨大的标记/环状染色体。这两个脂肪瘤样和粘液样成分的同一MLSLC表现出相同的FUS-DDIT 3，证实了荧光原位杂交（FISH）和逆转录聚合酶链反应（RT-PCR）。免疫组化MDM 2和CDK 4在WDLS/DDLS中均为阳性，而在MLSLC和普通MLS中均为阴性。在各型LS中均检测到PPARγ、C/EBPα、adipophilin和perilipin。Adipophilin主要表达于未成熟成脂细胞的微小脂肪滴中，而perilipin在印戒和多棘成脂细胞的大脂肪泡中显示强阳性染色。MLSLC的脂肪瘤样成分中Ki-67标记指数低于相同肿瘤的粘液样成分以及普通MLS（p < 0.001）。与普通MLS相比，MLSLC在广泛手术切除后的临床行为（罕见复发或转移）可能不那么积极。总之，MLSLC和WDLS/DDLS之间的区别是很重要的，因为这些不同的肉瘤之间的分子细胞遗传学特征以及临床行为的差异呈现相似的形态特征。此外，联合检测adipophilin和perilipin可能为软组织肉瘤中成脂细胞的识别提供一个有用的辅助工具。
Myxoid liposarcomas (MLSs) with extensive lipoma-like changes (MLSLC) are rare, and it is often difficult to distinguish them from well-differentiated liposarcoma (LS)/dedifferentiated LS (WDLS/DDLS) with myxoid changes. For the characterization of these neoplasms, we studied 8 MLSLCs, 11 ordinary MLSs, 4 WDLSs, and 6 DDLSs. Cytogenetically, MLSLC and ordinary MLS were characterized by t(12;16)(q13;p11) and FUS-DDIT3 fusion gene, whereas WDLS/DDLS lacked the fusion gene but possessed giant marker/ring chromosomes. Both lipoma-like and myxoid components of the same MLSLC exhibited the identical FUS-DDIT3, as confirmed by fluorescence in situ hybridization (FISH) and reverse transcription polymerase chain reaction (RT-PCR). Immunohistochemically, MDM2 and CDK4 were positive in WDLS/DDLS but negative in MLSLC and ordinary MLS. PPARγ, C/EBPα, adipophilin, and perilipin were found in each type of LS. Adipophilin was expressed chiefly in tiny fat droplets of immature lipoblasts, whereas perilipin showed a strong positive staining in large fat vacuoles of signet ring and multivacuolated lipoblasts. The Ki-67 labeling index was lower in the lipoma-like component of MLSLC when compared with the myxoid component of the same tumors as well as ordinary MLS (p < 0.001). When compared with ordinary MLS, MLSLC may be less aggressive in clinical behavior (rare recurrences or metastases) after a wide surgical excision. In conclusion, the distinction between MLSLC and WDLS/DDLS is important, because of the differences of molecular cytogenetic features as well as clinical behaviors between these distinct sarcomas presenting similar morphologic features. In addition, the combined immunohistochemical detection of adipophilin and perilipin may provide a useful ancillary tool for identification of lipoblastic cells in soft tissue sarcomas.
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影响因子：
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作者：
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通讯作者：
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Ushijima, M
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