Combination Chemotherapy in Treating Patients With Stage III or Stage IV Malignant Peripheral Nerve Sheath Tumors
Condition: Neurofibromatosis Type 1; Sarcoma
MeSH terms: Sarcoma; Neurofibromatoses; Neurofibromatosis 1; Neurofibroma; Nerve Sheath Neoplasms; Neurofibrosarcoma
Intervention: filgrastim; doxorubicin hydrochloride; etoposide; ifosfamide; conventional surgery; radiation therapy
Intervention description: Given subcutaneously; Given IV; Given IV; Given IV; Patients undergo surgery; Patients undergo radiotherapy
Intervention MeSH terms: Doxorubicin; Liposomal doxorubicin; Etoposide; Ifosfamide
Phase: Phase 2
Study type: Interventional
Primary purpose: Treatment
Allocation: Non-Randomized
Intervention model: Parallel Assignment
Masking: None (Open Label)
Enrollment: 48.0
Sex: All
Healthy volunteers: No
Keywords: adult neurofibrosarcoma; childhood neurofibrosarcoma; metastatic childhood soft tissue sarcoma; nonmetastatic childhood soft tissue sarcoma; stage IV adult soft tissue sarcoma; neurofibromatosis type 1

Summary:

      RATIONALE: Drugs used in chemotherapy, such as doxorubicin, ifosfamide, and etoposide, work
      in different ways to stop the growth of tumor cells, either by killing the cells or by
      stopping them from dividing. Radiation therapy uses high-energy x-rays to kill tumor cells.
      Giving combination chemotherapy with or without radiation therapy before surgery may make the
      tumor smaller and reduce the amount of normal tissue that needs to be removed. Giving
      combination chemotherapy after surgery may kill any tumor cells that remain after surgery.

      PURPOSE: This phase II trial is studying how well combination chemotherapy works in treating
      patients with stage III or stage IV malignant peripheral nerve sheath tumors.
    

Eligibility criteria:

        DISEASE CHARACTERISTICS:

          -  Newly diagnosed sporadic or neurofibromatosis type 1 (NF1)-associated high-grade
             malignant peripheral nerve sheath tumors (MPNSTs)

               -  Stage III or stage IV (metastatic) disease

          -  Measurable disease, defined as at least 1 tumor that is measurable in 2 dimensions on
             CT scan or MRI

        PATIENT CHARACTERISTICS:

          -  Ejection fraction normal by echocardiogram or MUGA

          -  Serum creatinine normal for age OR creatinine clearance > 60 mL/min

          -  SGPT < 5 times upper limit of normal (ULN)

          -  Bilirubin < 2.5 times ULN

          -  Absolute neutrophil count ≥ 1,500/mm^3*

          -  Hemoglobin ≥ 9.0 g/dL*

          -  Platelet count ≥ 100,000/mm^3*

          -  ECOG performance status 0-2

          -  Not pregnant or nursing

          -  Negative pregnancy test

          -  Fertile patients must use effective contraception during and for 6 months after
             completion of study treatment NOTE: * Unsupported

        PRIOR CONCURRENT THERAPY:

          -  No prior chemotherapy for MPNST

          -  Prior surgical resection of MPNST allowed provided residual or recurrent measurable
             disease is present

          -  Recovered from toxic effects of all prior therapy

          -  At least 3 weeks since prior chemotherapy or biologic therapy for treatment of a
             plexiform neurofibroma, optical pathway tumor, or other NF1-associated tumor (in
             patients with NF1)

          -  At least 6 weeks since prior radiotherapy for treatment of a plexiform neurofibroma,
             optical pathway tumor, or other NF1-associated tumor (in patients with NF1)

          -  At least 4 weeks since prior radiotherapy to the area involved by MPNST

          -  No other concurrent growth factors (e.g., sargramostim [GM-CSF] or interleukin-11)

               -  Concurrent epoetin alfa allowed