Results in patients with cranial parameningeal sarcoma and metastases (stage 4) treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols II-IV, 1978-1997

Report from the Children's Oncology Group

Beverly Raney, James Anderson, John Breneman, Sarah S. Donaldson, Winston Huh, Harold Maurice Maurer, Jeff Michalski, Stephen Qualman, Fred Ullrich, Moody Wharam, William Meyer

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

Purpose. Determine outcome of patients with cranial parameningeal sarcoma and concurrent metastases treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV. Patients. We identified 91 patients in the database, which includes newly diagnosed subjects <21 years old with rhabdomyosarcoma (RMS) and undifferentiated sarcoma, and reviewed their charts in detail. Results. The 54 males and 37 females were <1-19 years at diagnosis. Primary sites were nasopharynx-nasal cavity, middle ear/mastoid and parapharyngeal area ("better" sites, 55%), paranasal sinus and infratemporal- pterygopalatine area ("worse" sites, 42%), and other (3%). Sixty-eight percent of informative patients had direct intracranial extension. Major metastatic sites at diagnosis were lung (63%), bone marrow (33%), and bone (27%). Treatment included vincristine, actinomycin D, and cyclophosphamide (VAC) chemotherapy and radiotherapy to the primary tumor and up to five metastatic sites/tissues.Outcome. The estimated 10-year failure-free survival (FFS) rate was 32% (95% confidence interval [CI]: 22%, 42%). Sixty patients had progressive disease (N = 49) or death as a first event (N = 11); another developed myelodysplastic syndrome and died. Sites of first progression/relapse were distant (55%), local (12%), CNS extension (8%), mixed (6%), and uncertain (18%). Factors indicating likelihood of 10-year FFS included tumor arising in "better" versus "worse" sites (FFS 46% vs. 18%, P = 0.02) and embryonal versus other histology (FFS 37% vs. 19%, P = 0.06). Conclusions. Cure was possible for some patients with metastatic cranial parameningeal sarcoma. Patients with the best outlook had embryonal RMS located in the nasopharynx/nasal cavity, middle ear/mastoid, or parapharyngeal region. Distant metastases were the most frequent type of recurrence, indicating that more effective systemic agents are needed to eliminate residual disease.

Original languageEnglish (US)
Pages (from-to)17-22
Number of pages6
JournalPediatric Blood and Cancer
Volume51
Issue number1
DOIs
StatePublished - Jul 1 2008

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Rhabdomyosarcoma
Sarcoma
Neoplasm Metastasis
Mastoid
Nasopharynx
Nasal Cavity
Middle Ear
Survival
Embryonal Rhabdomyosarcoma
Recurrence
Paranasal Sinuses
Myelodysplastic Syndromes
Dactinomycin
Vincristine
Cyclophosphamide
Neoplasms
Histology
Radiotherapy
Survival Rate
Bone Marrow

Keywords

  • Childhood/adolescent metastatic parameningeal rhabdomyosarcoma
  • IRSG protocols

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

Results in patients with cranial parameningeal sarcoma and metastases (stage 4) treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols II-IV, 1978-1997 : Report from the Children's Oncology Group. / Raney, Beverly; Anderson, James; Breneman, John; Donaldson, Sarah S.; Huh, Winston; Maurer, Harold Maurice; Michalski, Jeff; Qualman, Stephen; Ullrich, Fred; Wharam, Moody; Meyer, William.

In: Pediatric Blood and Cancer, Vol. 51, No. 1, 01.07.2008, p. 17-22.

Research output: Contribution to journalArticle

Raney, Beverly ; Anderson, James ; Breneman, John ; Donaldson, Sarah S. ; Huh, Winston ; Maurer, Harold Maurice ; Michalski, Jeff ; Qualman, Stephen ; Ullrich, Fred ; Wharam, Moody ; Meyer, William. / Results in patients with cranial parameningeal sarcoma and metastases (stage 4) treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols II-IV, 1978-1997 : Report from the Children's Oncology Group. In: Pediatric Blood and Cancer. 2008 ; Vol. 51, No. 1. pp. 17-22.
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abstract = "Purpose. Determine outcome of patients with cranial parameningeal sarcoma and concurrent metastases treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV. Patients. We identified 91 patients in the database, which includes newly diagnosed subjects <21 years old with rhabdomyosarcoma (RMS) and undifferentiated sarcoma, and reviewed their charts in detail. Results. The 54 males and 37 females were <1-19 years at diagnosis. Primary sites were nasopharynx-nasal cavity, middle ear/mastoid and parapharyngeal area ({"}better{"} sites, 55{\%}), paranasal sinus and infratemporal- pterygopalatine area ({"}worse{"} sites, 42{\%}), and other (3{\%}). Sixty-eight percent of informative patients had direct intracranial extension. Major metastatic sites at diagnosis were lung (63{\%}), bone marrow (33{\%}), and bone (27{\%}). Treatment included vincristine, actinomycin D, and cyclophosphamide (VAC) chemotherapy and radiotherapy to the primary tumor and up to five metastatic sites/tissues.Outcome. The estimated 10-year failure-free survival (FFS) rate was 32{\%} (95{\%} confidence interval [CI]: 22{\%}, 42{\%}). Sixty patients had progressive disease (N = 49) or death as a first event (N = 11); another developed myelodysplastic syndrome and died. Sites of first progression/relapse were distant (55{\%}), local (12{\%}), CNS extension (8{\%}), mixed (6{\%}), and uncertain (18{\%}). Factors indicating likelihood of 10-year FFS included tumor arising in {"}better{"} versus {"}worse{"} sites (FFS 46{\%} vs. 18{\%}, P = 0.02) and embryonal versus other histology (FFS 37{\%} vs. 19{\%}, P = 0.06). Conclusions. Cure was possible for some patients with metastatic cranial parameningeal sarcoma. Patients with the best outlook had embryonal RMS located in the nasopharynx/nasal cavity, middle ear/mastoid, or parapharyngeal region. Distant metastases were the most frequent type of recurrence, indicating that more effective systemic agents are needed to eliminate residual disease.",
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T2 - Report from the Children's Oncology Group

AU - Raney, Beverly

AU - Anderson, James

AU - Breneman, John

AU - Donaldson, Sarah S.

AU - Huh, Winston

AU - Maurer, Harold Maurice

AU - Michalski, Jeff

AU - Qualman, Stephen

AU - Ullrich, Fred

AU - Wharam, Moody

AU - Meyer, William

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N2 - Purpose. Determine outcome of patients with cranial parameningeal sarcoma and concurrent metastases treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV. Patients. We identified 91 patients in the database, which includes newly diagnosed subjects <21 years old with rhabdomyosarcoma (RMS) and undifferentiated sarcoma, and reviewed their charts in detail. Results. The 54 males and 37 females were <1-19 years at diagnosis. Primary sites were nasopharynx-nasal cavity, middle ear/mastoid and parapharyngeal area ("better" sites, 55%), paranasal sinus and infratemporal- pterygopalatine area ("worse" sites, 42%), and other (3%). Sixty-eight percent of informative patients had direct intracranial extension. Major metastatic sites at diagnosis were lung (63%), bone marrow (33%), and bone (27%). Treatment included vincristine, actinomycin D, and cyclophosphamide (VAC) chemotherapy and radiotherapy to the primary tumor and up to five metastatic sites/tissues.Outcome. The estimated 10-year failure-free survival (FFS) rate was 32% (95% confidence interval [CI]: 22%, 42%). Sixty patients had progressive disease (N = 49) or death as a first event (N = 11); another developed myelodysplastic syndrome and died. Sites of first progression/relapse were distant (55%), local (12%), CNS extension (8%), mixed (6%), and uncertain (18%). Factors indicating likelihood of 10-year FFS included tumor arising in "better" versus "worse" sites (FFS 46% vs. 18%, P = 0.02) and embryonal versus other histology (FFS 37% vs. 19%, P = 0.06). Conclusions. Cure was possible for some patients with metastatic cranial parameningeal sarcoma. Patients with the best outlook had embryonal RMS located in the nasopharynx/nasal cavity, middle ear/mastoid, or parapharyngeal region. Distant metastases were the most frequent type of recurrence, indicating that more effective systemic agents are needed to eliminate residual disease.

AB - Purpose. Determine outcome of patients with cranial parameningeal sarcoma and concurrent metastases treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV. Patients. We identified 91 patients in the database, which includes newly diagnosed subjects <21 years old with rhabdomyosarcoma (RMS) and undifferentiated sarcoma, and reviewed their charts in detail. Results. The 54 males and 37 females were <1-19 years at diagnosis. Primary sites were nasopharynx-nasal cavity, middle ear/mastoid and parapharyngeal area ("better" sites, 55%), paranasal sinus and infratemporal- pterygopalatine area ("worse" sites, 42%), and other (3%). Sixty-eight percent of informative patients had direct intracranial extension. Major metastatic sites at diagnosis were lung (63%), bone marrow (33%), and bone (27%). Treatment included vincristine, actinomycin D, and cyclophosphamide (VAC) chemotherapy and radiotherapy to the primary tumor and up to five metastatic sites/tissues.Outcome. The estimated 10-year failure-free survival (FFS) rate was 32% (95% confidence interval [CI]: 22%, 42%). Sixty patients had progressive disease (N = 49) or death as a first event (N = 11); another developed myelodysplastic syndrome and died. Sites of first progression/relapse were distant (55%), local (12%), CNS extension (8%), mixed (6%), and uncertain (18%). Factors indicating likelihood of 10-year FFS included tumor arising in "better" versus "worse" sites (FFS 46% vs. 18%, P = 0.02) and embryonal versus other histology (FFS 37% vs. 19%, P = 0.06). Conclusions. Cure was possible for some patients with metastatic cranial parameningeal sarcoma. Patients with the best outlook had embryonal RMS located in the nasopharynx/nasal cavity, middle ear/mastoid, or parapharyngeal region. Distant metastases were the most frequent type of recurrence, indicating that more effective systemic agents are needed to eliminate residual disease.

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