Hyalinizing spindle cell tumor with giant rosettes arising in the lung: report of a case with FUS-CREB3L2 fusion transcripts

Pathol Int. 2007 Mar;57(3):153-7. doi: 10.1111/j.1440-1827.2006.02073.x.

Abstract

Hyalinizing spindle cell tumor with giant rosettes (HSCT) is a very uncommon mesenchymal tumor that has similar morphological and biological features to the low-grade fibromyxoid sarcoma (LGFMS). Lung involvement of HSCT is extremely rare, and only one case has been reported in the English-language literature. Reported herein is a case of primary pulmonary HSCT that had FUS-CREB3L2 fusion transcripts, a product of characteristic chromosomal abnormality t(7;16)(q33;p11) of HSCT and LGFMS. The patient was a 50-year-old woman who had a large solitary mass in the lung. Histologically, it was composed of bland spindle cells with variable cellularity deposited in a densely hyalinized stroma alternating with myxoid areas. Characteristic collagen rosettes were scattered in the cellular areas. Reverse transcription-polymerase chain reaction (RT-PCR) assay using formalin-fixed, paraffin-embedded tissue detected FUS-CREB3L2 fusion transcripts. Despite its bland morphology, it is known as low-grade sarcoma and its recognition in the lung would be helpful for accurate diagnosis and proper management of this rare tumor. RT-PCR for detection of FUS-CREB3L2 fusion transcripts is a useful method for differential diagnosis of primary pulmonary HSCT.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Biomarkers, Tumor / metabolism
  • Cyclic AMP Response Element-Binding Protein / genetics
  • Cyclic AMP Response Element-Binding Protein / metabolism*
  • Female
  • Fibrosarcoma / metabolism
  • Fibrosarcoma / pathology*
  • Fibrosarcoma / surgery
  • Humans
  • Hyalin / metabolism
  • Lung Neoplasms / metabolism
  • Lung Neoplasms / pathology*
  • Lung Neoplasms / surgery
  • Middle Aged
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / metabolism*
  • Oncogene Proteins, Fusion / genetics
  • Oncogene Proteins, Fusion / metabolism*
  • Reverse Transcriptase Polymerase Chain Reaction
  • Transcription Factors
  • Treatment Outcome

Substances

  • Biomarkers, Tumor
  • CREB3L1 protein, human
  • Cyclic AMP Response Element-Binding Protein
  • Nerve Tissue Proteins
  • Oncogene Proteins, Fusion
  • Transcription Factors