A Population-Based Analysis of Survival for Sinonasal Rhabdomyosarcoma

Aykut A Unsal, Sei Yeon Chung, Aylin B. Unsal, Soly Baredes, Jean Anderson Eloy

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Objectives: Literature detailing factors affecting survival in sinonasal rhabdomyosarcoma (SNRMS) is limited due to its rarity. We analyze the demographics, potential prognostic factors, overall survival, and treatment efficacy of SNRMS. Study Design and Setting: Administrative database study. Methods: SNRMS was queried in the SEER database (1973-2013; Surveillance, Epidemiology, and End Results). Data were analyzed for demographic and clinicopathologic trends. Kaplan-Meier model was utilized for assessing survival. Results: A total of 286 cases of SNRMS were identified. The median age at diagnosis was 24.5 years, and the mean tumor size was 5.1 cm. Overall 5-, 10-, and 20-year disease-specific survival was 35.1%, 25.4%, and 12.0%, respectively. Regional lymph node involvement was present at diagnosis in more than half of cases (54.3%) and distant metastasis in 32.2% of cases. Alveolar rhabdomyosarcoma (54.9%) and embryonal rhabdomyosarcoma (23.8%) were the primary histologies. Alveolar-type SNRMS was more likely than the embryonal type to metastasize to distant sites (41.7% vs 24.1%), and it possessed poorer 5-year survival (29.2% vs 45.6%). Distant metastasis decreased 5-year survival from 35.1% to 3.8%, whereas lymph node involvement did not significantly affect survival. Patients <10 years old had the highest survival rates. Primary tumor origin by subsite did not significantly affect survival. Smaller tumor sizes and earlier Intergroup Rhabdomyosarcoma Study Group stages were associated with improved outcomes. Surgical therapy had the highest survival rates. Conclusion: SNRMS has overall poorer prognosis than all other parameningeal rhabdomyosarcomas studied. Age <10 years, smaller tumor sizes, lack of distant metastasis, localized tumors, earlier Intergroup Rhabdomyosarcoma Study Group stages, and embryonal histology were all associated with improved outcomes.

Original languageEnglish (US)
Pages (from-to)142-149
Number of pages8
JournalOtolaryngology - Head and Neck Surgery (United States)
Volume157
Issue number1
DOIs
StatePublished - Jul 1 2017
Externally publishedYes

Fingerprint

Rhabdomyosarcoma
Survival Analysis
Survival
Population
Alveolar Rhabdomyosarcoma
Neoplasms
Neoplasm Metastasis
Histology
Survival Rate
Lymph Nodes
Demography
Embryonal Rhabdomyosarcoma
Databases
Epidemiology

Keywords

  • SEER
  • Surveillance Epidemiology and End Results
  • disease-specific survival
  • population based
  • rhabdomyosarcoma
  • sinonasal cancer
  • sinonasal cancer
  • sinonasal rhabdomyosarcoma
  • survival

ASJC Scopus subject areas

  • Surgery
  • Otorhinolaryngology

Cite this

A Population-Based Analysis of Survival for Sinonasal Rhabdomyosarcoma. / Unsal, Aykut A; Chung, Sei Yeon; Unsal, Aylin B.; Baredes, Soly; Eloy, Jean Anderson.

In: Otolaryngology - Head and Neck Surgery (United States), Vol. 157, No. 1, 01.07.2017, p. 142-149.

Research output: Contribution to journalArticle

Unsal, Aykut A ; Chung, Sei Yeon ; Unsal, Aylin B. ; Baredes, Soly ; Eloy, Jean Anderson. / A Population-Based Analysis of Survival for Sinonasal Rhabdomyosarcoma. In: Otolaryngology - Head and Neck Surgery (United States). 2017 ; Vol. 157, No. 1. pp. 142-149.
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abstract = "Objectives: Literature detailing factors affecting survival in sinonasal rhabdomyosarcoma (SNRMS) is limited due to its rarity. We analyze the demographics, potential prognostic factors, overall survival, and treatment efficacy of SNRMS. Study Design and Setting: Administrative database study. Methods: SNRMS was queried in the SEER database (1973-2013; Surveillance, Epidemiology, and End Results). Data were analyzed for demographic and clinicopathologic trends. Kaplan-Meier model was utilized for assessing survival. Results: A total of 286 cases of SNRMS were identified. The median age at diagnosis was 24.5 years, and the mean tumor size was 5.1 cm. Overall 5-, 10-, and 20-year disease-specific survival was 35.1{\%}, 25.4{\%}, and 12.0{\%}, respectively. Regional lymph node involvement was present at diagnosis in more than half of cases (54.3{\%}) and distant metastasis in 32.2{\%} of cases. Alveolar rhabdomyosarcoma (54.9{\%}) and embryonal rhabdomyosarcoma (23.8{\%}) were the primary histologies. Alveolar-type SNRMS was more likely than the embryonal type to metastasize to distant sites (41.7{\%} vs 24.1{\%}), and it possessed poorer 5-year survival (29.2{\%} vs 45.6{\%}). Distant metastasis decreased 5-year survival from 35.1{\%} to 3.8{\%}, whereas lymph node involvement did not significantly affect survival. Patients <10 years old had the highest survival rates. Primary tumor origin by subsite did not significantly affect survival. Smaller tumor sizes and earlier Intergroup Rhabdomyosarcoma Study Group stages were associated with improved outcomes. Surgical therapy had the highest survival rates. Conclusion: SNRMS has overall poorer prognosis than all other parameningeal rhabdomyosarcomas studied. Age <10 years, smaller tumor sizes, lack of distant metastasis, localized tumors, earlier Intergroup Rhabdomyosarcoma Study Group stages, and embryonal histology were all associated with improved outcomes.",
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AU - Eloy, Jean Anderson

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N2 - Objectives: Literature detailing factors affecting survival in sinonasal rhabdomyosarcoma (SNRMS) is limited due to its rarity. We analyze the demographics, potential prognostic factors, overall survival, and treatment efficacy of SNRMS. Study Design and Setting: Administrative database study. Methods: SNRMS was queried in the SEER database (1973-2013; Surveillance, Epidemiology, and End Results). Data were analyzed for demographic and clinicopathologic trends. Kaplan-Meier model was utilized for assessing survival. Results: A total of 286 cases of SNRMS were identified. The median age at diagnosis was 24.5 years, and the mean tumor size was 5.1 cm. Overall 5-, 10-, and 20-year disease-specific survival was 35.1%, 25.4%, and 12.0%, respectively. Regional lymph node involvement was present at diagnosis in more than half of cases (54.3%) and distant metastasis in 32.2% of cases. Alveolar rhabdomyosarcoma (54.9%) and embryonal rhabdomyosarcoma (23.8%) were the primary histologies. Alveolar-type SNRMS was more likely than the embryonal type to metastasize to distant sites (41.7% vs 24.1%), and it possessed poorer 5-year survival (29.2% vs 45.6%). Distant metastasis decreased 5-year survival from 35.1% to 3.8%, whereas lymph node involvement did not significantly affect survival. Patients <10 years old had the highest survival rates. Primary tumor origin by subsite did not significantly affect survival. Smaller tumor sizes and earlier Intergroup Rhabdomyosarcoma Study Group stages were associated with improved outcomes. Surgical therapy had the highest survival rates. Conclusion: SNRMS has overall poorer prognosis than all other parameningeal rhabdomyosarcomas studied. Age <10 years, smaller tumor sizes, lack of distant metastasis, localized tumors, earlier Intergroup Rhabdomyosarcoma Study Group stages, and embryonal histology were all associated with improved outcomes.

AB - Objectives: Literature detailing factors affecting survival in sinonasal rhabdomyosarcoma (SNRMS) is limited due to its rarity. We analyze the demographics, potential prognostic factors, overall survival, and treatment efficacy of SNRMS. Study Design and Setting: Administrative database study. Methods: SNRMS was queried in the SEER database (1973-2013; Surveillance, Epidemiology, and End Results). Data were analyzed for demographic and clinicopathologic trends. Kaplan-Meier model was utilized for assessing survival. Results: A total of 286 cases of SNRMS were identified. The median age at diagnosis was 24.5 years, and the mean tumor size was 5.1 cm. Overall 5-, 10-, and 20-year disease-specific survival was 35.1%, 25.4%, and 12.0%, respectively. Regional lymph node involvement was present at diagnosis in more than half of cases (54.3%) and distant metastasis in 32.2% of cases. Alveolar rhabdomyosarcoma (54.9%) and embryonal rhabdomyosarcoma (23.8%) were the primary histologies. Alveolar-type SNRMS was more likely than the embryonal type to metastasize to distant sites (41.7% vs 24.1%), and it possessed poorer 5-year survival (29.2% vs 45.6%). Distant metastasis decreased 5-year survival from 35.1% to 3.8%, whereas lymph node involvement did not significantly affect survival. Patients <10 years old had the highest survival rates. Primary tumor origin by subsite did not significantly affect survival. Smaller tumor sizes and earlier Intergroup Rhabdomyosarcoma Study Group stages were associated with improved outcomes. Surgical therapy had the highest survival rates. Conclusion: SNRMS has overall poorer prognosis than all other parameningeal rhabdomyosarcomas studied. Age <10 years, smaller tumor sizes, lack of distant metastasis, localized tumors, earlier Intergroup Rhabdomyosarcoma Study Group stages, and embryonal histology were all associated with improved outcomes.

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