Medpedia

• Sarcoma

DISEASE CHARACTERISTICS:
-  Histologically confirmed embryonal (EMB) rhabdomyosarcoma (RMS) or botryoid or
spindle cell variants of EMB RMS or embryonal ectomesenchymoma meeting 1 of the
following criteria:
-  Stage 1, no clinical group IV: Tumor in favorable site (orbit, head and neck
[excluding parameningeal], genitourinary [not bladder/prostate], or biliary
tract) and no metastatic disease
-  Stage 2 or 3, clinical group I or II: Tumor in unfavorable site
(bladder/prostate, extremity, cranial parameningeal, trunk, retroperitoneum,
pelvis, perineal/perianal, intrathoracic, gastrointestinal, or liver), no gross
residual disease after initial surgery, and no metastatic disease
-  Must have ipsilateral lymph node dissection if age 10 or over with primary
paratesticular cancer OR under age 10 with clinically positive regional lymph nodes
-  Low risk of recurrence
-  Previously untreated disease
-  No alveolar RMS or undifferentiated sarcoma
-  No intermediate-risk disease
-  No metastatic disease at diagnosis
PATIENT CHARACTERISTICS:
Age:
-  Under 50
Performance status:
-  Not specified
Hematopoietic:
-  Not specified
Hepatic:
-  Bilirubin elevation secondary to biliary or hepatic primaries allowed
Renal:
-  Creatinine elevation secondary to tumor obstruction allowed
Other:
-  No uncontrolled infection
-  Not pregnant or nursing
-  Fertile patients must use effective contraception
PRIOR CONCURRENT THERAPY:
Biologic therapy
-  Not specified
Chemotherapy
-  Not specified
Endocrine therapy
-  Not specified
Radiotherapy
-  Not specified
Surgery
-  Not specified

OBJECTIVES:

-  Determine the failure-free survival (FFS) rate in patients with newly diagnosed
low-risk rhabdomyosarcoma of embryonal or botryoid subtype meeting criteria for group I
after treatment with dactinomycin and vincristine with or without radiotherapy.

-  Determine the FFS rate in these patients meeting criteria for group II after treatment
with dactinomycin, vincristine, and cyclophosphamide with or without radiotherapy.

-  Determine the FFS rate in patients with ectomesenchymomas containing
rhabdomyosarcomatous elements (embryonal histiotype) who receive one of the above
treatments.

-  Determine new molecular markers specific to embryonal and botryoid tumor histologies
which are of diagnostic and prognostic significance in patients treated with these
regimens.

OUTLINE: Patients are assigned to 1 of 2 groups, depending on histology and site of disease.

-  Group I (favorable tumor site, negative lymph nodes, stage 1, clinical group I, IIA, or
III (orbit only), node negative [N0] OR unfavorable tumor site, negative or unknown
lymph nodes, stage 2, clinical group I): Patients receive vincristine IV over 1 minute
weekly for 8 weeks and dactinomycin IV over 1 minute once every 3 weeks for 4 doses.
Treatment repeats every 12 weeks for 4 courses. Radiotherapy is administered to
patients with clinical group II or III disease on weeks 3-8.

-  Group II (favorable tumor site, positive lymph nodes, stage 1, clinical group III
(orbit only), node positive [N1] OR favorable tumor site except orbit, any lymph nodes,
stage 1, clinical group III OR unfavorable tumor site, stage 2, clinical group II OR
unfavorable tumor site, stage 3, clinical group I or II): Patients receive vincristine
and dactinomycin as in group I. Patients also receive cyclophosphamide IV over 30-60
minutes and filgrastim (G-CSF) or sargramostim (GM-CSF) subcutaneously once daily
beginning 24 hours after completion of chemotherapy and continuing for 10 days or until
blood counts recover. Radiotherapy is administered on weeks 3-8, 12-17, or 28-33, if
clinically indicated as in group I.

Patients are followed every 3-4 months for 3 years (4 years after diagnosis), every 6 months
for 1 year, and then annually thereafter.

PROJECTED ACCRUAL: A total of 254 patients for group I will be accrued for this study within
6 years. Approximately 12 patients per year will be accrued for group II.

• Biological: dactinomycin
• Biological: filgrastim
• Biological: sargramostim
• Drug: cyclophosphamide
• Drug: vincristine sulfate
• Radiation: radiation therapy

• R. Beverly Raney, MD
  Study Chair, M.D. Anderson Cancer Center

• Swiss Pediatric Oncology Group Lausanne
  Lausanne, CH 1011, Switzerland
  Completed
• Swiss Pediatric Oncology Group Geneva
  Geneva, CH 1211, Switzerland
  Completed
• Swiss Pediatric Oncology Group Bern
  Bern, CH 3010, Switzerland
  Completed
• San Jorge Childrens Hospital
  Santurce, 00912, Puerto Rico
  Completed
• Puerto Rico Cancer Center at University of Puerto Rico - Medical Sciences Campus
  San Juan, 00936-5067, Puerto Rico
  Completed
• Starship Children's Hospital
  Auckland, New Zealand
  Completed
• Academisch Ziekenhuis Groningen
  Groningen, 9700 RB, Netherlands
  Completed
• Saskatoon Cancer Centre
  Saskatoon, Saskatchewan, S7N 4H4, Canada
  Completed
• Allan Blair Cancer Centre at Pasqua Hospital
  Regina, Saskatchewan, S4T 7T1, Canada
  Completed
• Centre de Recherche du Centre Hospitalier de l'Universite Laval
  Sainte Foy, Quebec, GIV 4G2, Canada
  Completed
• Hopital Sainte Justine
  Montreal, Quebec, H3T 1C5, Canada
  Completed
• McGill University Health Center - Montreal Children's Hospital
  Montreal, Quebec, H3G 1A4, Canada
  Completed

• Children's Oncology Group
  Lead Sponsor
• United States: Federal Government

• Clinical trial summary from the National Cancer Institute's PDQ® database
  http://cancer.gov/clinicaltrials/COG-D9602