Paranasal sinus rhabdomyosarcoma: a rare tumor of poor prognosis

Eur Ann Otorhinolaryngol Head Neck Dis. 2013 Feb;130(1):26-9. doi: 10.1016/j.anorl.2012.07.002. Epub 2012 Sep 27.

Abstract

Introduction: Rhabdomyosarcoma (RMS) is a rare sarcoma, in which paranasal sinus locations are exceptional in adults.

Case report: We report a case of ethmoid metastatic RMS in a 48-year-old patient, discovered in connection with recurrent epistaxis associated with exophthalmia and ophthalmoplegia. The tumor was inoperable and chemotherapy based on adriamycin was initiated. The course was, however, marked by rapid worsening of symptoms and the patient's death.

Discussion: Paranasal sinus RMS shows no specific clinical signs, and diagnosis is mainly based on immunohistochemical analysis. The association of surgery and chemo-radiotherapy is the optimal attitude, but surgical resection is often impossible due to local extension. Prognosis in adults is poor.

Publication types

  • Case Reports

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols
  • Chemoradiotherapy
  • Disease Progression
  • Doxorubicin / administration & dosage
  • Epistaxis / etiology
  • Ethmoid Sinus*
  • Exophthalmos / etiology
  • Fatal Outcome
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Neoplasm Invasiveness
  • Neoplasm Staging
  • Ophthalmoplegia / etiology
  • Paranasal Sinus Neoplasms / diagnosis*
  • Paranasal Sinus Neoplasms / pathology
  • Paranasal Sinus Neoplasms / therapy
  • Positron-Emission Tomography
  • Prognosis
  • Rhabdomyosarcoma / diagnosis*
  • Rhabdomyosarcoma / pathology
  • Rhabdomyosarcoma / therapy
  • Tomography, X-Ray Computed
  • Whole Body Imaging

Substances

  • Doxorubicin