Adherence to Treatment, Response and Patterns of Failure in Pediatric Parameningeal Rhabdomyosarcoma: Experience From a Tertiary Cancer Care Center From India

J Pediatr Hematol Oncol. 2017 Mar;39(2):e62-e68. doi: 10.1097/MPH.0000000000000745.

Abstract

The study was aimed at evaluating adherence to treatment protocol and outcome in pediatric parameningeal rhabdomyosarcoma (PM-RMS). We analyzed the characteristics, treatment administered, outcomes and patterns of failure of pediatric PM-RMS, who were treated with multimodality therapy between January 2005 and December 2013.Univariate and multivariate analysis (MVA) was completed to evaluate the impact of various prognostic factors. Thirty-seven patients were treated at our institution. Majority of them had the primary disease in paranasal sinuses (n=13). Majority of the patients belonged to group III (n=30) and stage III (n=24). The overall response rate to treatment was 52.5% (n=21). At a mean follow-up of 19.1 months, 23 patients developed disease progression. The actuarial rates of failure-free survival and overall survival (OS) at 2 years were 40% and 67.5%, respectively. Patients who received >20 weeks of intended chemotherapy schedule (P=0.02) and had complete response to first-line treatment (P=0.0004) were found to have superior failure-free survival on MVA. Complete response was the lone determinant of superior OS on MVA (P=0.006). Majority of patients with PM-RMS present with advanced stage disease. Response to first-line treatment is a significant predictor of superior progression-free survival and OS in these patients.

MeSH terms

  • Adolescent
  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Cancer Care Facilities / statistics & numerical data
  • Chemoradiotherapy
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Cranial Irradiation
  • Cyclophosphamide / administration & dosage
  • Dactinomycin / administration & dosage
  • Disease-Free Survival
  • Female
  • Head and Neck Neoplasms / epidemiology*
  • Head and Neck Neoplasms / pathology
  • Head and Neck Neoplasms / surgery
  • Head and Neck Neoplasms / therapy
  • Humans
  • India / epidemiology
  • Infant
  • Kaplan-Meier Estimate
  • Male
  • Palliative Care
  • Paranasal Sinus Neoplasms / epidemiology
  • Paranasal Sinus Neoplasms / pathology
  • Paranasal Sinus Neoplasms / surgery
  • Paranasal Sinus Neoplasms / therapy
  • Patient Compliance*
  • Prognosis
  • Proportional Hazards Models
  • Remission Induction
  • Retrospective Studies
  • Rhabdomyosarcoma / epidemiology*
  • Rhabdomyosarcoma / pathology
  • Rhabdomyosarcoma / surgery
  • Rhabdomyosarcoma / therapy
  • Salvage Therapy
  • Tertiary Care Centers / statistics & numerical data
  • Treatment Outcome
  • Vincristine / administration & dosage
  • Young Adult

Substances

  • Dactinomycin
  • Vincristine
  • Cyclophosphamide

Supplementary concepts

  • VAC protocol