Therapeutic outcome and prognostic factors in sinonasal rhabdomyosarcoma: a single-institution case series

J Cancer Res Clin Oncol. 2019 Nov;145(11):2793-2802. doi: 10.1007/s00432-019-03009-8. Epub 2019 Aug 23.

Abstract

Background: The purpose of this study was to explore the demographics, multimodality therapeutic outcomes*** and prognostic factors in sinonasal rhabdomyosarcoma (SNRMS).

Methods: We conducted a retrospective analysis of 40 patients who underwent treatment of SNRMS from March 2007 to March 2018. The Kaplan-Meier method and the log-rank test were used to assess survival rates. The Cox regression model was used for multivariate survival analysis.

Results: In total, 25 males and 15 females were included in the study; the median age was 33 years (range, 2-67 years). All patients underwent surgical resection, and surgery prior to or after adjuvant therapy (chemotherapy and radiotherapy) was performed in 91.4% of the patients. The overall 1-, 3- and 5-year survival rates were 77.0%, 46.5% and 46.5%, respectively, during a mean follow-up time of 27.9 (range, 2-128) months in all patients. The log-rank test showed Intergroup Rhabdomyosarcoma Study (IRS) group and infiltration of the skull base influenced overall survival (p = 0.001; p = 0.022). Advanced IRS stage, lymph node metastasis and tumor size ≥ 5 cm were also associated with an unfavorable outcome on overall survival (p = 0.01; p = 0.035; p = 0.02). The results of multivariate regression analysis showed patients with IRS group I were associated with better prognosis outcome on overall survival.

Conclusion: Patients with SNRMS have poor 5-year overall survival, and IRS group is the independent prognostic factor for overall survival.

Keywords: Prognostic factors; Rhabdomyosarcoma; Sinonasal; Skull base; Survival.

Publication types

  • Clinical Trial

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Paranasal Sinus Neoplasms / mortality*
  • Paranasal Sinus Neoplasms / therapy
  • Prognosis
  • Retrospective Studies
  • Rhabdomyosarcoma / mortality*
  • Rhabdomyosarcoma / therapy
  • Survival Rate
  • Young Adult