Molecular variability of TLS-CHOP structure shows no significant impact on the level of adipogenesis: a comparative ultrastructural and RT-PCR analysis of 14 cases of myxoid/round cell liposarcomas

Ultrastruct Pathol. 2003 Jul-Aug;27(4):217-26. doi: 10.1080/01913120309917.

Abstract

A specific TLS-CHOP fusion gene derived from the t(12;16) is present in at least 95% of myxoid/round cell liposarcomas (MLS). Rare cases of MLS show a variant t(12;22) translocation, resulting in EWS-CHOP fusion gene. The CHOP gene encodes a leucine-zipper transcription factor, which is implicated in both oncogenic transformation and inhibition of adipogenesis. To examine whether the molecular variability of TLS-CHOP or EWS-CHOP fusion transcript structure is associated with the degree of inhibition of adipogenesis, a comparative ultrastructural and RT-PCR-based analysis of 14 MLS was performed. The specimens consisted of 9 primary, 2 locally recurrent tumors, and one representative sample each from 3 patients with multifocal soft tissue metastases. Histologically, there were 8 high-grade and 6 low-grade MLS using 5% round cell (RC) component as the cutoff point. By RT-PCR assay there were 13 cases with TLS-CHOP fusion transcripts: 7 cases of type 5-2 (known as type II), 4 cases of type 7-2 (known as type I),1 case of type 8-2 (known as type III), and 1 unique case of type 6-2. The remaining 1 case showed an EWS-CHOP fusion transcript. Ultrastructural examination revealed that tumor cells were composed of a moderate-to-predominant proportion of well-formed lipoblasts in 4 cases, while in 6 cases such lipoblasts were very scant. The remaining 4 tumors were arrested in the stage of transitional cells. The heterogeneity of TLS-CHOP fusion transcript showed no apparent impact on adipogenesis, since both TLS-CHOP type I and II cases could randomly display various levels of lipoblastic differentiation. Furthermore, the 4 cases without definite lipoblasts showed no preference for any specific fusion variants and consisted of one each of TLS-CHOP subtypes. In addition, the fusion transcript variants did not correlate with other ultrastructural features, such as the presence and amount of glycogen, mitochondria, rough endoplasmic reticulum, vimentin-like intermediate filaments, and external lamina. However, there appeared to have a trend suggesting the predilections of glycogen particles and vimentin-like intermediate filaments in primitive mesenchymal cells and/or transitional cells. These findings cannot substantiate the hypothesis that the molecular variability of fusion transcripts has a biological impact on adipogenesis of MLS, and other factors might be implicated in their level of differentiation.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adipocytes* / metabolism
  • Adipocytes* / ultrastructure
  • Adolescent
  • Adult
  • CCAAT-Enhancer-Binding Proteins / genetics*
  • Female
  • Humans
  • Liposarcoma, Myxoid / genetics*
  • Liposarcoma, Myxoid / metabolism
  • Liposarcoma, Myxoid / secondary
  • Male
  • Microscopy, Electron
  • Middle Aged
  • Oncogene Proteins, Fusion / genetics*
  • RNA, Messenger / metabolism
  • RNA, Neoplasm / metabolism
  • RNA-Binding Protein EWS / genetics
  • RNA-Binding Protein FUS / genetics*
  • Reverse Transcriptase Polymerase Chain Reaction
  • Soft Tissue Neoplasms / genetics*
  • Soft Tissue Neoplasms / metabolism
  • Soft Tissue Neoplasms / pathology
  • Transcription Factor CHOP

Substances

  • CCAAT-Enhancer-Binding Proteins
  • Oncogene Proteins, Fusion
  • RNA, Messenger
  • RNA, Neoplasm
  • RNA-Binding Protein EWS
  • RNA-Binding Protein FUS
  • TLS-CHOP fusion protein, human
  • Transcription Factor CHOP