Lingual alveolar soft part sarcoma with absent TFE3 rearrangement

Cancer Rep (Hoboken). 2021 Feb;4(1):e1298. doi: 10.1002/cnr2.1298. Epub 2020 Oct 7.

Abstract

Background: Lingual ASPS is extremely rare and aggressive tumor. rearrangement is typically detected in ASPS patients using FISH analysis.

Aim: To present the clinical, histopathological, and radiological features of lingual Alveolar Soft Part Sarcoma.

Method: A 30-year-old male presented with a painless, slowly growing mass of the tongue. Initial impression was of benign vascular lesion. Later, the patient became symptomatic as the mass progressed in size, which necessitated further investigations.

Result: A lip-split, mandibulotomy was performed for the excision of the tumor and revealed an alveolar soft part sarcoma with PAS-positive, diastase resistant intracytoplasmic granules. However, molecular analysis using FISH was negative for TFE3 rearrangement. Patient underwent partial glossectomy with postoperative radiotherapy.

Conclusion: Clinical and pathological correlation of ASPS is very useful to reach a proper diagnosis.

Keywords: glossectomy; lingual cancer; oral; sarcoma; tumor.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / genetics*
  • Biomarkers, Tumor / genetics*
  • Gene Rearrangement
  • Glossectomy
  • Humans
  • Male
  • Radiotherapy, Adjuvant
  • Sarcoma, Alveolar Soft Part / diagnosis*
  • Sarcoma, Alveolar Soft Part / genetics
  • Sarcoma, Alveolar Soft Part / pathology
  • Sarcoma, Alveolar Soft Part / therapy
  • Tongue / diagnostic imaging
  • Tongue / pathology
  • Tongue Neoplasms / diagnosis*
  • Tongue Neoplasms / genetics
  • Tongue Neoplasms / pathology
  • Tongue Neoplasms / therapy
  • Treatment Outcome

Substances

  • Basic Helix-Loop-Helix Leucine Zipper Transcription Factors
  • Biomarkers, Tumor
  • TFE3 protein, human