Adult rhabdomyosarcoma survival improved with treatment on multimodality protocols

Int J Radiat Oncol Biol Phys. 2013 May 1;86(1):58-63. doi: 10.1016/j.ijrobp.2012.12.016. Epub 2013 Feb 13.

Abstract

Purpose: Rhabdomyosarcoma (RMS) is a pediatric sarcoma rarely occurring in adults. For unknown reasons, adults with RMS have worse outcomes than do children.

Methods and materials: We analyzed data from all patients who presented to Memorial Sloan-Kettering Cancer Center between 1990 and 2011 with RMS diagnosed at age 16 or older. One hundred forty-eight patients met the study criteria. Ten were excluded for lack of adequate data.

Results: The median age was 28 years. The histologic diagnoses were as follows: embryonal 54%, alveolar 33%, pleomorphic 12%, and not otherwise specified 2%. The tumor site was unfavorable in 67% of patients. Thirty-three patients (24%) were at low risk, 61 (44%) at intermediate risk, and 44 (32%) at high risk. Forty-six percent were treated on or according to a prospective RMS protocol. The 5-year rate of overall survival (OS) was 45% for patients with nonmetastatic disease. The failure rates at 5 years for patients with nonmetastatic disease were 34% for local failure and 42% for distant failure. Among patients with nonmetastatic disease (n=94), significant factors associated with OS were histologic diagnosis, site, risk group, age, and protocol treatment. On multivariate analysis, risk group and protocol treatment were significant after adjustment for age. The 5-year OS was 54% for protocol patients versus 36% for nonprotocol patients.

Conclusions: Survival in adult patients with nonmetastatic disease was significantly improved for those treated on RMS protocols, most of which are now open to adults.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Analysis of Variance
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Combined Modality Therapy / methods
  • Female
  • Humans
  • Male
  • Middle Aged
  • Prognosis
  • Retrospective Studies
  • Rhabdomyosarcoma / epidemiology
  • Rhabdomyosarcoma / mortality*
  • Rhabdomyosarcoma / pathology
  • Rhabdomyosarcoma / therapy*
  • Rhabdomyosarcoma, Alveolar / epidemiology
  • Rhabdomyosarcoma, Alveolar / mortality
  • Rhabdomyosarcoma, Alveolar / pathology
  • Rhabdomyosarcoma, Embryonal / epidemiology
  • Rhabdomyosarcoma, Embryonal / mortality
  • Rhabdomyosarcoma, Embryonal / pathology
  • Survival Rate
  • Treatment Failure
  • Young Adult