Embryonal and alveolar rhabdomyosarcoma of parameningeal sites in adults: a report of 13 cases

Int J Surg Pathol. 2009 Feb;17(1):22-30. doi: 10.1177/1066896908325876. Epub 2008 Oct 22.

Abstract

This study reports 13 adult parameningeal rhabdomyosarcomas. Ages ranged from 18 to 86 years. Nine lesions were alveolar, 3 were embryonal, and 1 could not be further classified. All cases were positive for desmin and/or myogenin and 1 tumor was strongly positive for cytokeratin. PAX3-FKHR or PAX7-FKHR fusion transcripts or FKHR breaks were identified in 5 cases confirming a diagnosis of alveolar rhabdomyosarcoma. Three cases were negative supporting a diagnosis of embryonal rhabdomyosarcoma. A total of 10 patients were treated with chemotherapy and radiation. Three patients are alive with no disease, 3 are alive with disease, 3 died of disease, and 4 patients are lost to follow-up. Adult sinonasal rhabdomyosarcoma is uncommon and should be considered in the differential of sinonasal neoplasms. Disease can occur in the elderly. Desmin and myogenin can aid in the diagnosis but cytokeratin reactivity can be seen and care must be taken not to diagnose carcinoma.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Combined Modality Therapy
  • Drug Therapy
  • Female
  • Forkhead Box Protein O1
  • Forkhead Transcription Factors / genetics
  • Forkhead Transcription Factors / metabolism
  • Gene Fusion
  • Humans
  • Male
  • Middle Aged
  • Nasal Cavity*
  • Nose Neoplasms / diagnosis*
  • Nose Neoplasms / metabolism
  • Nose Neoplasms / therapy
  • PAX3 Transcription Factor
  • PAX7 Transcription Factor / genetics
  • PAX7 Transcription Factor / metabolism
  • Paired Box Transcription Factors / genetics
  • Paired Box Transcription Factors / metabolism
  • Paranasal Sinus Neoplasms / diagnosis*
  • Paranasal Sinus Neoplasms / metabolism
  • Paranasal Sinus Neoplasms / therapy
  • Radiotherapy
  • Rhabdomyosarcoma, Alveolar / diagnosis*
  • Rhabdomyosarcoma, Alveolar / metabolism
  • Rhabdomyosarcoma, Alveolar / therapy
  • Rhabdomyosarcoma, Embryonal / diagnosis*
  • Rhabdomyosarcoma, Embryonal / metabolism
  • Rhabdomyosarcoma, Embryonal / therapy
  • Treatment Outcome
  • Young Adult

Substances

  • FOXO1 protein, human
  • Forkhead Box Protein O1
  • Forkhead Transcription Factors
  • PAX3 Transcription Factor
  • PAX3 protein, human
  • PAX7 Transcription Factor
  • PAX7 protein, human
  • Paired Box Transcription Factors