Treatment of children and adolescents with localized parameningeal sarcoma: Experience of the Intergroup Rhabdomyosarcoma Study Group protocols IRS-II through -IV, 1978–1997*†
For the Intergroup Rhabdomyosarcoma Study Group (IRSG) representing the Children's Cancer Group, the Pediatric Oncology Group, the Children's Oncology Group, and the Intergroup Rhabdomyosarcoma Statistical Office, including Richard J. Andrassy, MD, Carola A.S. Arndt, MD, K. Scott Baker, MD, Frederic G. Barr, MD, W. Archie Bleyer, MD, Philip P. Breitfeld, MD, Julia A. Bridge, MD, Kenneth Brown, MD, Holcombe E. Grier, MD, Douglas Hawkins, MD, Peter J. Houghton, PhD, Michael P. Link, MD, Thom E. Lobe, MD, William H. Meyer, MD, Sharon B. Murphy, MD, Charles N. Paidas, MD, Alberto S. Pappo, MD, David M. Parham, MD, Leslie Robison, PhD, Lynn Smith, MD, Poul H.B. Sorensen, MD, PhD, Lisa A. Teot, MD, Timothy Triche, MD, PhD, Teresa J. Vietti, MD, David O. Walterhouse, MD, Suzanne L. Wolden, MD, and Richard B. Womer, MD.
Presented in part at the Third International Congress on Soft Tissue Sarcoma in Children and Adolescents in Stuttgart, Germany, on May 3, 1997, by Dr. Fryer; and at the 32nd Meeting of the International Society of Pediatric Oncology (SIOP) in Amsterdam, The Netherlands, on October 5, 2000, by Dr. Raney.
Abstract
Background
We reviewed 611 patients with parameningeal sarcoma entered on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols-II through-IV (1978–1997), to delineate treatment results and evaluate prognostic factors.
Procedure
Primary sites were the middle ear/mastoid (N = 138), nasopharynx/nasal cavity (N = 235), paranasal sinuses (N = 132), parapharyngeal region (N = 29), and the pterygopalatine/infratemporal fossa (N = 77). Treatment was initial biopsy or surgery followed by multiagent chemotherapy and radiation therapy (XRT). Beginning in 1977, patients with cranial nerve palsy, cranial base bony erosion, and/or intracranial extension at diagnosis were considered as having meningeal involvement. They received triple intrathecal medications, whole brain XRT, and then spinal XRT. These treatments were successively eliminated from 1980 to 1991.
Results
The 611 patients' overall survival rate at 5 years was 73% (95% confidence interval, 70–77%). Favorable prognostic factors were: age 1–9 years at diagnosis; primary tumor in the nasopharynx/nasal cavity, middle ear/mastoid, or parapharyngeal areas; no meningeal involvement; and non-invasive tumors (T1). Thirty-five of 526 patients (6.7%) with information about presence/absence of meningeal involvement at diagnosis developed central nervous system (CNS) extension at 5–164 weeks (median, 46 weeks) after starting therapy. The estimated 5-year cumulative incidence rate of CNS extension during the study period was 5–7% (P = 0.88).
Conclusions
Biopsy, XRT to the target volume, and systemic chemotherapy are successful treatments for the large majority of patients with localized parameningeal sarcoma. Carefully defining and irradiating the initial volume should reduce the risk of CNS failure. Aggressive initial surgical management of these patients is unnecessary. Med Pediatr Oncol 2002;38:22–32. © 2002 Wiley-Liss, Inc.
