Primary Ewing's sarcoma of the sinonasal tract in adults: A challenging disease

Head Neck. 2017 Mar;39(3):E45-E50. doi: 10.1002/hed.24649. Epub 2016 Nov 29.

Abstract

Background: Sinonasal localization of Ewing's sarcoma in adults is an exceedingly rare event.

Methods: The clinical records of 5 patients with primary sinonasal Ewing's sarcoma treated from 1992 to 2012 were retrospectively analyzed. All pathologic slides were reviewed by 2 experienced pathologists. All patients underwent multimodality treatments.

Results: Median age was 36 years (range, 25-52 years). At referral, 2 patients had the original diagnosis changed by review of the histologic slides. Tumors were classified as T4aN0M0 (4 patients) and T2N0M0 (1 patient). Median follow-up was 110 months (range, 70-139 months). Only 1 patient, who started treatment elsewhere based on an incorrect histologic diagnosis, experienced multiple recurrences and eventually died of widespread metastasis.

Conclusion: Correct pathologic diagnosis can have a crucial impact on treatment planning and outcome. Multimodality therapy is the key for long-term successful results. Because of the rarity of the tumor, referral to highly experienced care centers is strongly recommended. © 2016 Wiley Periodicals, Inc. Head Neck 39: E45-E50, 2017.

Keywords: Ewing's sarcoma; chemotherapy; multimodality treatment; sinonasal cancer; transnasal endoscopic surgery.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage*
  • Chemoradiotherapy, Adjuvant / methods
  • Female
  • Follow-Up Studies
  • Humans
  • Magnetic Resonance Imaging / methods
  • Male
  • Middle Aged
  • Neoplasm Invasiveness / pathology
  • Neoplasm Staging
  • Paranasal Sinus Neoplasms / diagnostic imaging*
  • Paranasal Sinus Neoplasms / mortality
  • Paranasal Sinus Neoplasms / therapy*
  • Radiotherapy Dosage
  • Retrospective Studies
  • Sampling Studies
  • Sarcoma, Ewing / diagnostic imaging*
  • Sarcoma, Ewing / mortality
  • Sarcoma, Ewing / therapy*
  • Survival Rate
  • Time Factors
  • Tomography, X-Ray Computed / methods