Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma

Not Recruiting

Trial ID: NCT00346164


This phase III trial is studying observation to see how well a risk based treatment strategy works in patients with soft tissue sarcoma. In the study, patients are assigned to receive surgery +/- radiotherapy +/- chemotherapy depending on their risk of recurrence. Sometimes, after surgery, the tumor may not need additional treatment until it progresses. In this case, observation may be sufficient. Radiation therapy uses high-energy x-rays to kill tumor cells. Drugs used in chemotherapy, such as ifosfamide and doxorubicin, work in different ways to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. Giving chemotherapy and radiation therapy before surgery may make the tumor smaller and reduce the amount of normal tissue that needs to be removed. Giving these treatments after surgery may kill any tumor cells that remain after surgery.

Official Title

Risk-Based Treatment for Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS) in Patients Under 30 Years of Age

Stanford Investigator(s)


Inclusion Criteria:

   - Newly diagnosed non-rhabdomyosarcoma soft tissue sarcoma (STS), confirmed by central
   pathology review via concurrent enrollment on protocol COG-D9902

      - Metastatic or non metastatic disease

   - Meets 1 of the following criteria:

      - Intermediate (i.e., rarely metastasizing) or malignant STS, including any of the

         - Adipocytic tumor, including liposarcoma of any of the following histology

            - Dedifferentiated

            - Myxoid

            - Round cell

            - Pleomorphic type

            - Mixed-type

            - Not otherwise specified (NOS)

         - Fibroblastic/myofibroblastic tumors, including any of the following:

            - Solitary fibrous tumor

            - Hemangiopericytoma

            - Low-grade myofibroblastic sarcoma

            - Myxoinflammatory fibroblastic sarcoma

            - Adult fibrosarcoma*

            - Myxofibrosarcoma

            - Low-grade fibromyxoid sarcoma or hyalinizing spindle-cell tumor

            - Sclerosing epithelioid fibrosarcoma

         - So-called fibrohistiocytic tumors, including any of the following:

            - Plexiform fibrohistiocytic tumor

            - Giant cell tumor of soft tissues

            - Pleomorphic malignant fibrous histiocytoma (MFH)/undifferentiated
            pleomorphic sarcoma

            - Giant cell MFH/undifferentiated pleomorphic sarcoma with giant cells

            - Inflammatory MFH/undifferentiated pleomorphic sarcoma with prominent

         - Smooth muscle tumor (leiomyosarcoma)

         - Pericytic [perivascular] tumor (malignant glomus tumor or glomangiosarcoma)

         - Vascular tumor, including angiosarcoma

         - Chondro-osseous tumors of any of the following types:

            - Mesenchymal chondrosarcoma

            - Extraskeletal osteosarcoma

         - Tumors of uncertain differentiation, including any of the following:

            - Angiomatoid fibrous histiocytoma

            - Ossifying fibromyxoid tumor

            - Myoepithelioma/parachordoma

            - Synovial sarcoma

            - Epithelioid sarcoma

            - Alveolar soft-part sarcoma

            - Clear cell sarcoma of soft tissue

            - Extraskeletal myxoid chondrosarcoma ("chordoid type")

            - Malignant mesenchymoma

            - Neoplasms with perivascular epithelioid cell differentiation (PEComa)

            - Clear cell myomelanocytic tumor

            - Intimal sarcoma

      - Malignant peripheral nerve sheath tumor

      - Dermatofibrosarcoma protuberans meeting both of the following criteria:

         - Non metastatic disease

         - Tumor must be grossly resected prior to study enrollment

      - Embryonal sarcoma of the liver

      - Unclassified STS that is too undifferentiated to be placed in a specific
      pathologic category (undifferentiated STS or STS NOS)

   - Gross resection of the primary tumor ≤ 42 days prior to enrollment required except if
   any of the following circumstances apply:

      - Non metastatic high-grade tumor > 5 cm in maximal diameter and gross or
      microscopic residual tumor is anticipated after resection

      - Tumor of either high- or- low-grade that cannot be grossly excised without
      unacceptable morbidity

      - High-grade tumor with metastases

         - Patients with metastatic low-grade tumor whose disease is amenable to gross
         resection at all sites must undergo gross resection of all sites prior to
         study entry

   - Patients with a tumor recurrence after a gross total resection are not eligible

   - Tumors arising in bone are not eligible

   - Patients with epithelioid sarcoma, clear cell sarcoma, or clinical or radiologic
   evidence of regional lymph node enlargement must undergo sentinel lymph node biopsies
   or lymph node sampling to confirm the status of regional lymph nodes* NOTE: *Except in
   cases where the study radiologist reviews the imaging and indicates that a biopsy is
   not needed to confirm that the patient has lymph node involvement.

      - If lymph node biopsies are positive for tumor (or the lymph nodes are classified
      as positive by the study radiologist), formal lymph node dissection must be done
      at the time of definitive surgery(prior to study entry for patients assigned to
      study regimen C)

   - Patients with metastatic disease must undergo a biopsy to confirm the presence of
   metastatic tumor if all metastases are < 1 cm in maximal diameter (except in cases
   where the study radiologist reviews the imaging and indicated that a biopsy is not
   needed to confirm that the patient has metastatic disease)

   - Lansky performance status (PS) 50-100% (for patients ≤ 16 years of age) OR Karnofsky
   PS 50-100% (for patients > 16 years of age)

   - Life expectancy ≥ 3 months

   - Absolute neutrophil count ≥ 1,000/mm³*

   - Platelet count ≥ 100,000/mm³*

   - Creatinine clearance or radioisotope glomerular filtration rate ≥ 70 mL/min (≥ 40
   mL/min for infants < 1 year of age)* or serum creatinine based on age and/or gender as

      - 0.4 mg/dL (1 month to < 6 months of age)

      - 0.5 mg/dL (6 months to < 1 year of age)

      - 0.6 mg/dL (1 year to < 2 years of age)

      - 0.8 mg/dL (2 years to < 6 years of age)

      - 1.0 mg/dL (6 years to < 10 years of age)

      - 1.2 mg/dL (10 years to < 13 years of age)

      - 1.5 mg/dL (male) or 1.4 mg/dL (female) (13 years to < 16 years of age)

      - 1.7 mg/dL (male) or 1.4 mg/dL (female) (≥ 16 years of age)

   - Patients with urinary tract obstruction by tumor must meet the renal function criteria
   listed above AND must have unimpeded urinary flow established via decompression of the
   obstructed portion of the urinary tract

   - Bilirubin ≤ 1.5 times upper limit of normal (ULN)*

   - Shortening fraction ≥ 27% by echocardiogram* OR ejection fraction ≥ 50% by
   radionuclide angiogram*

   - Not pregnant or nursing (patients undergoing radiotherapy and/or chemotherapy)

      - No nursing for ≥ 1 month after completion of study treatment in study regimens C
      or D

   - Fertile patients must use effective contraception during and for ≥ 1 month after
   completion of study treatment

   - Negative pregnancy test

   - No evidence of dyspnea at rest*

   - No exercise intolerance*

   - Resting pulse oximetry reading > 94% on room air (for patients with respiratory

   - Prior treatment for cancer allowed provided the patient meet the prior therapy

   - No prior anthracycline (e.g., doxorubicin or daunorubicin) or ifosfamide chemotherapy
   for patients enrolled on arm C or arm D

   - No prior radiotherapy to tumor-involved sites


other: clinical observation

drug: doxorubicin hydrochloride

drug: ifosfamide

procedure: therapeutic conventional surgery

radiation: 3-dimensional conformal radiation therapy

Not Recruiting

Contact Information

Stanford University
School of Medicine
300 Pasteur Drive
Stanford, CA 94305
Peds Hem/Onc CRAs

New Trial Alerts

Receive email alerts when trials open to patients.