BACKGROUND: Malignant peripheral nerve sheath tumors (MPNST) are very rare in the general population and challenging to treat. A paucity of data exists regarding the incidence of MPNST across all age groups and treatment outcomes in the pediatric population. We aimed to characterize both using the Survival, Epidemiology, and End Results (SEER) database. PROCEDURE: The SEER-18 database with information on the United States population from 1973 to 2009 was queried for cases of MPNST. For incidence data, 1,182 cases were found among the general population. Of those, 165 cases were in individuals aged 0-19. After exclusions, 139 cases from the SEER-18 database met study criteria for outcomes analysis. For each patient, variables including gender, age, race, stage (localized, regional, or distant), surgical treatment, and radiotherapy were obtained. RESULTS: The overall incidence of MPNST was 1.46 per million person-years, with increased incidence among the elderly. In the pediatric population, the incidence was 0.56 per million person-years, and was higher among post-pubertal children aged 10-19. Median overall survival in the pediatric population was 30 months, with only localized disease and treatment with surgery being positive prognostic factors on multivariate analysis. CONCLUSIONS: MPNST is a rare disease and, among children, is most frequent seen in adolescents. Surgery is crucial as first-line treatment for MPNST, especially if the tumor is localized at diagnosis. In patients with non-localized MPNST, the disease remains extremely difficult to manage, and both surgery and radiotherapy are interventions that should be considered.
BACKGROUND:Malignant peripheral nerve sheath tumors (MPNST) are very rare in the general population and challenging to treat. A paucity of data exists regarding the incidence of MPNST across all age groups and treatment outcomes in the pediatric population. We aimed to characterize both using the Survival, Epidemiology, and End Results (SEER) database. PROCEDURE: The SEER-18 database with information on the United States population from 1973 to 2009 was queried for cases of MPNST. For incidence data, 1,182 cases were found among the general population. Of those, 165 cases were in individuals aged 0-19. After exclusions, 139 cases from the SEER-18 database met study criteria for outcomes analysis. For each patient, variables including gender, age, race, stage (localized, regional, or distant), surgical treatment, and radiotherapy were obtained. RESULTS: The overall incidence of MPNST was 1.46 per million person-years, with increased incidence among the elderly. In the pediatric population, the incidence was 0.56 per million person-years, and was higher among post-pubertal children aged 10-19. Median overall survival in the pediatric population was 30 months, with only localized disease and treatment with surgery being positive prognostic factors on multivariate analysis. CONCLUSIONS: MPNST is a rare disease and, among children, is most frequent seen in adolescents. Surgery is crucial as first-line treatment for MPNST, especially if the tumor is localized at diagnosis. In patients with non-localized MPNST, the disease remains extremely difficult to manage, and both surgery and radiotherapy are interventions that should be considered.
Authors: Fernando Guedes; Rosana Siqueira Brown; Francisco José Lourenço Torrão-Junior; Daniel A N Barbosa; Guilherme de Andrade Gagheggi Ravanini; Rogério Martin Pires Amorim Journal: Childs Nerv Syst Date: 2019-07-25 Impact factor: 1.475
Authors: Erin C Peckham-Gregory; Roberto E Montenegro; David A Stevenson; David H Viskochil; Michael E Scheurer; Philip J Lupo; Joshua D Schiffman Journal: J Neurooncol Date: 2018-04-16 Impact factor: 4.130
Authors: Gregory K Friedman; Elizabeth A Beierle; George Yancey Gillespie; James M Markert; Alicia M Waters; Chun-Yu Chen; Nicholas L Denton; Kellie B Haworth; Brian Hutzen; Jennifer L Leddon; Keri A Streby; Pin-Yi Wang; Timothy P Cripe Journal: Mol Ther Oncolytics Date: 2015-09-16 Impact factor: 7.200