Sequence confirmation of the EWS-WT1 fusion gene transcript in the peritoneal effusion of a patient with desmoplastic small round cell tumor

Diagn Cytopathol. 2003 Dec;29(6):341-3. doi: 10.1002/dc.10397.

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare undifferentiated neoplasm. The prognosis is poor, even if therapy is instituted promptly, and thus it is important to differentiate it from other histologically and cytologically similar-looking malignancies of the young adult. We present a case of DSRCT in a 17-yr-old male with disseminated peritoneal disease and peritoneal effusion. The cytology sample showed a malignant small round cell tumor, the classical cytological features of DSRCT, and immunohistochemistry performed in the prepared cell block exhibited an antibody expression profile in keeping with DSRCT. Further material from the effusion was prepared for RNA extraction, following which a reverse-transcriptase polymerase chain reaction (RT-PCR) and sequencing of the t(11;22)(p13;q11 or q12) were carried out. The result showed the presence of the reciprocal translocation and thus confirmed the diagnosis of DSRCT. This case shows how molecular techniques (including sequencing) can be applied to cytology in clarifying and confirming certain difficult diagnosis of undifferentiated neoplasms, DSRCT in this particular case.

MeSH terms

  • Adolescent
  • Ascites / diagnosis
  • Ascites / genetics
  • Ascites / metabolism
  • Ascitic Fluid / diagnosis
  • Ascitic Fluid / genetics*
  • Base Sequence
  • Biomarkers, Tumor / analysis
  • Carcinoma, Small Cell / chemistry
  • Carcinoma, Small Cell / genetics*
  • Carcinoma, Small Cell / pathology
  • Humans
  • Male
  • Molecular Sequence Data
  • Oncogene Proteins, Fusion / genetics*
  • Proto-Oncogene Protein c-fli-1
  • RNA, Messenger / genetics
  • RNA, Messenger / metabolism
  • RNA, Neoplasm / analysis
  • RNA-Binding Protein EWS
  • Reverse Transcriptase Polymerase Chain Reaction
  • Sequence Analysis, RNA*
  • Soft Tissue Neoplasms / chemistry
  • Soft Tissue Neoplasms / genetics*
  • Soft Tissue Neoplasms / pathology
  • Transcription Factors / genetics*
  • Translocation, Genetic

Substances

  • Biomarkers, Tumor
  • EWS-FLI fusion protein
  • Oncogene Proteins, Fusion
  • Proto-Oncogene Protein c-fli-1
  • RNA, Messenger
  • RNA, Neoplasm
  • RNA-Binding Protein EWS
  • Transcription Factors