peg-interferon alfa-2b in treating young patients with plexiform neurofibroma

Conditions: Neoplasm of Uncertain Malignant Potential; Unspecified Childhood Solid Tumor, Protocol Specific

Summary: rationale: peg-interferon alfa-2b may interfere with the growth of tumor cells. purpose: this phase i trial is studying the side effects and best dose of peg-interferon alfa-2b in treating young patients with plexiform neurofibroma.

objectives: primary * determine the maximum tolerated dose of peg-interferon alfa-2b in patients with unresectable plexiform neurofibroma. (dose escalation portion of study closed to accrual as of 2/2005.) * determine the toxicity profile of this drug in these patients. secondary * obtain, preliminary, information about the efficacy of this drug in these patients. * evaluate the growth rate of plexiform neurofibroma using volumetric mri analysis in patients treated with this drug. * evaluate the impact of this drug, in terms of "worst symptom" score, in these patients. outline: this is a dose-escalation, multicenter study. (dose-escalation portion of the study closed to accrual as of 2/2005.) patients receive peg-interferon alfa-2b subcutaneously once weekly for 2 years in the absence of disease progression or unacceptable toxicity. cohorts of 3-6 patients receive escalating doses of peg-interferon alfa-2b until the maximum tolerated dose (mtd) is determined. the mtd is defined as the dose preceding that at which 1 of 6 patients experience dose-limiting toxicity. a total of 12 patients receive treatment at the mtd. after completion of study treatment, patients are followed every 6 months. projected accrual: a total of 36 patients will be accrued for this study.

Eligibility criteria:
disease characteristics: * diagnosis of plexiform neurofibroma which is progressive, symptomatic, or life threatening and for which there is no other standard medical management or surgical option * histologic confirmation of tumor is not required in the presence of consistent clinical and radiographic findings provided the following are true: * no clinical observation or scan suggestive of malignant transformation * meets ≥ 1 of the following diagnostic criteria for neurofibroma type 1 (nf1): * six or more cafe-au-lait spots (\> 0.5 cm in prepubertal patients or \> 1.5 cm in post pubertal patients) * freckling in axilla or groin * optic glioma * two or more lisch nodules * a distinctive bony lesion (e.g., dysplasia of the sphenoid bone, dysplasia, or thinning of long bone cortex) * a first degree relative with nf1 * no history of malignant peripheral nerve sheath tumor * no active visual pathway glioma * no active brain tumor or brain metastases patient characteristics: performance status * ecog 0-2 life expectancy * at least 12 months hematopoietic * absolute neutrophil count \> 1,500/mm\^3 * hemoglobin \> 10 g/dl * platelet count \> 100,000/mm\^3 hepatic * bilirubin \< 1.5 mg/dl * sgpt ≤ 2 times upper limit of normal * no significant hepatic dysfunction renal * creatinine based on age as follows: * ≤ 0.8 mg/dl (for patients age 5 years and under) * ≤ 1.0 mg/dl (for patients age 6 to 10 years) * ≤ 1.2 mg/dl (for patients age 11 to 15 years) * ≤ 1.5 mg/dl (for patients age 16 to 21 years) or * creatinine clearance ≥ 70 ml/min cardiovascular * no significant cardiac dysfunction * no severe cardiovascular disease * no cardiac arrhythmia requiring chronic treatment * no congestive heart failure * no symptomatic ischemic heart disease pulmonary * no significant pulmonary dysfunction other * not pregnant or nursing * negative pregnancy test * fertile patients must use effective contraception * no serious infection * no other significant unrelated systemic illness * no significant organ dysfunction * no other malignancy except surgically cured nonmelanoma skin cancer or carcinoma in situ of the cervix * no history of severe psychiatric condition or psychiatric disorder requiring hospitalization * no history of suicidal ideation or attempt * no thyroid dysfunction unresponsive to therapy * no uncontrolled diabetes mellitus * no history of hiv positivity * no alcohol or drug abuse prior concurrent therapy: biologic therapy * no concurrent immunotherapy * no concurrent colony-stimulating factors (e.g., erythropoietin or filgrastim \[g-csf\]) chemotherapy * no concurrent chemotherapy for this disease endocrine therapy * no concurrent chronic systemic corticosteroids * no concurrent hormonal therapy for this disease radiotherapy * no concurrent radiotherapy for this disease surgery * prior surgery allowed provided it has been at least 21 days since surgery and there is presence of residual tumor other * recovered from prior therapy * more than 30 days since prior investigational agents

Keywords: neoplasm of uncertain malignant potential unspecified childhood solid tumor, protocol specific