Improved outcome for patients with middle ear rhabdomyosarcoma: A children's oncology group study

D. S. Hawkins, J. R. Anderson, C. N. Paidas, M. D. Wharam, S. J. Qualman, A. S. Pappo, K. Scott Baker, W. M. Crist

Research output: Contribution to journalArticlepeer-review

Abstract

The goal of this study was to define the clinical features and optimal therapy for children and adolescents with middle ear (ME) rhabdomyosarcoma (RMS). Patients and Methods: We reviewed demographic data, clinical features, therapy (including chemotherapy, surgery, and radiation), and outcome for the 179 eligible patients with ME RMS who were enrolled onto Intergroup Rhabdomyosarcoma Studies (IRS) I through IV or pilot studies between November 1972 and December 1997. Results: Most patients were younger than 10 years old (90%), and 63% were male. Because of the parameningeal location, most tumors were not resected before chemotherapy (group I, < 1%; group II, 4%; group III, 84%; group IV, 12%). Although most tumors were locally invasive (T2, 89%), the majority were small (≤ 5 cm, 66%), lacked nodal metastases (NO, 86%), and had embryonal histology (85%). The 5-year failure-free survival (FFS) and overall survival (OS) estimates were 67% and 72%, respectively. Both FFS and OS improved significantly over the course of IRS I through IV (3-year FFS and OS: IRS-I, 42% and 42%; IRS-II, 70% and 74%; IRS-III, 65% and 72%; IRS-IV pilot, 81% and 96%; IRS-IV, 88% and 88%, P < .001). Lower clinical group or stage and smaller tumor size were associated with better outcome. Age, sex, tumor invasiveness, and nodal metastases were not predictive of outcome. Conclusion: Patients with ME RMS generally present with small, unresectable, invasive tumors at a site traditionally considered prognostically unfavorable. Nevertheless, such patients have benefited markedly from improvements in multimodal, risk-based therapy during the course of IRS I through IV, and with contemporary therapy, most are cured.

Original languageEnglish (US)
Pages (from-to)3073-3079
Number of pages7
JournalJournal of Clinical Oncology
Volume19
Issue number12
DOIs
StatePublished - Jun 15 2001

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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