Treatment of children and adolescents with localized parameningeal sarcoma: Experience of the intergroup rhabdomyosarcoma study group protocols IRS-II through-IV, 1978-1997

R. B. Raney, Jane L Meza, J. R. Anderson, C. J. Fryer, S. S. Donaldson, J. C. Breneman, T. J. Fitzgerald, E. A. Gehan, J. M. Michalski, J. A. Ortega, S. J. Qualman, E. Sandler, M. D. Wharam, E. S. Wiener, H. M. Maurer, W. M. Crist

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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 noninvasive 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.

Original languageEnglish (US)
Pages (from-to)22-32
Number of pages11
JournalMedical and Pediatric Oncology
Volume38
Issue number1
DOIs
StatePublished - Jan 1 2002

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Rhabdomyosarcoma
Sarcoma
Mastoid
Nasopharynx
Central Nervous System
Nasal Cavity
Middle Ear
Therapeutics
Pterygopalatine Fossa
Biopsy
Cranial Nerve Diseases
Drug Therapy
Paranasal Sinuses
Skull Base
Neoplasms
Radiotherapy
Survival Rate
Confidence Intervals
Incidence
Brain

Keywords

  • Childhood cancer
  • Cranial parameningeal sarcoma
  • Outcome
  • Therapy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Cancer Research

Cite this

Treatment of children and adolescents with localized parameningeal sarcoma : Experience of the intergroup rhabdomyosarcoma study group protocols IRS-II through-IV, 1978-1997. / Raney, R. B.; Meza, Jane L; Anderson, J. R.; Fryer, C. J.; Donaldson, S. S.; Breneman, J. C.; Fitzgerald, T. J.; Gehan, E. A.; Michalski, J. M.; Ortega, J. A.; Qualman, S. J.; Sandler, E.; Wharam, M. D.; Wiener, E. S.; Maurer, H. M.; Crist, W. M.

In: Medical and Pediatric Oncology, Vol. 38, No. 1, 01.01.2002, p. 22-32.

Research output: Contribution to journalArticle

Raney, RB, Meza, JL, Anderson, JR, Fryer, CJ, Donaldson, SS, Breneman, JC, Fitzgerald, TJ, Gehan, EA, Michalski, JM, Ortega, JA, Qualman, SJ, Sandler, E, Wharam, MD, Wiener, ES, Maurer, HM & Crist, WM 2002, 'Treatment of children and adolescents with localized parameningeal sarcoma: Experience of the intergroup rhabdomyosarcoma study group protocols IRS-II through-IV, 1978-1997', Medical and Pediatric Oncology, vol. 38, no. 1, pp. 22-32. https://doi.org/10.1002/mpo.1259
Raney, R. B. ; Meza, Jane L ; Anderson, J. R. ; Fryer, C. J. ; Donaldson, S. S. ; Breneman, J. C. ; Fitzgerald, T. J. ; Gehan, E. A. ; Michalski, J. M. ; Ortega, J. A. ; Qualman, S. J. ; Sandler, E. ; Wharam, M. D. ; Wiener, E. S. ; Maurer, H. M. ; Crist, W. M. / Treatment of children and adolescents with localized parameningeal sarcoma : Experience of the intergroup rhabdomyosarcoma study group protocols IRS-II through-IV, 1978-1997. In: Medical and Pediatric Oncology. 2002 ; Vol. 38, No. 1. pp. 22-32.
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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 noninvasive 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.",
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T1 - Treatment of children and adolescents with localized parameningeal sarcoma

T2 - Experience of the intergroup rhabdomyosarcoma study group protocols IRS-II through-IV, 1978-1997

AU - Raney, R. B.

AU - Meza, Jane L

AU - Anderson, J. R.

AU - Fryer, C. J.

AU - Donaldson, S. S.

AU - Breneman, J. C.

AU - Fitzgerald, T. J.

AU - Gehan, E. A.

AU - Michalski, J. M.

AU - Ortega, J. A.

AU - Qualman, S. J.

AU - Sandler, E.

AU - Wharam, M. D.

AU - Wiener, E. S.

AU - Maurer, H. M.

AU - Crist, W. M.

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N2 - 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 noninvasive 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.

AB - 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 noninvasive 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.

KW - Childhood cancer

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KW - Outcome

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