Schneider-Stock Regine, Gerresheim F, Kolin-Gerresheim I, Meyer F, Jäger V, Epplen J T, Roessner A, Boltze C
Department of Pathology, Otto-von-Guericke University, Magdeburg, Germany.
Pathol Res Pract. 2003;199(6):445-50. doi: 10.1078/0344-0338-00444.
Like malignant fibrous histiocytoma (MFH), dedifferentiated liposarcoma represents a distinct subtype of liposarcoma and is characterized by an abrupt transition from well-differentiated liposarcoma (WDL) to highgrade dedifferentiated liposarcoma (DDL) . In addition, specific cytogenetic aberrations support the close biological relationship between WDL and DDL. Recent observations indicated the significance of cell cycle aberrations in tumor progression from the low-malignant, well differentiated to its dedifferentiated form, the prognosis of which is poor. Thus, alterations of mdm2 and p53 genes belong to the most frequently reported alterations in these two subtypes of liposarcoma. In previous investigations, we reported that loss of heterozygosity at the Rb gene locus, telomerase activity, hTERT, and c-Myc expression were associated with tumor progression in liposarcomas. In this study, we report on a case of a WD/DDL, in which both tumor components were separated using laser microdissection (P.A.L.M.) for the investigation of hTERT mRNA expression on a LightCycler. Macroscopically selected and histologically proven cryosections of low malignant and highly malignant tumor areas were cytogenetically investigated to confirm the diagnosis and to find additional chromosomal alterations with tumor progression.
去分化脂肪肉瘤与恶性纤维组织细胞瘤(MFH)一样,是脂肪肉瘤的一种独特亚型,其特征是从高分化脂肪肉瘤(WDL)突然转变为高级别去分化脂肪肉瘤(DDL)。此外,特定的细胞遗传学异常支持了WDL和DDL之间密切的生物学关系。最近的观察表明,细胞周期异常在肿瘤从低恶性、高分化向去分化形式进展中具有重要意义,而去分化脂肪肉瘤的预后较差。因此,mdm2和p53基因的改变是这两种脂肪肉瘤亚型中最常报道的改变。在之前的研究中,我们报道了Rb基因位点杂合性缺失、端粒酶活性、hTERT和c-Myc表达与脂肪肉瘤的肿瘤进展相关。在本研究中,我们报告了一例WD/DDL病例,其中使用激光显微切割(P.A.L.M.)将肿瘤的两个成分分离,以在LightCycler上研究hTERT mRNA表达。对宏观选择并经组织学证实的低恶性和高恶性肿瘤区域的冷冻切片进行细胞遗传学研究,以确诊并发现肿瘤进展过程中其他的染色体改变。