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Regina I. Jakacki, Bruce H. Cohen, Cheryl Jamison, Vincent P. Mathews, Edward Arenson, Darryl C. Longee, Joanne Hilden, Al Cornelius, Michael Needle, Doug Heilman, Joel C. Boaz and Thomas G. Luerssen

Object. Craniopharyngiomas originate from the same cells as squamous cell skin carcinoma, which can be treated successfully with interferon-α (IFNα)-2a. The authors evaluated the activity and toxicity of systemic IFN in young patients with craniopharyngiomas.

Methods. Fifteen patients between the ages of 4.2 and 19.8 years who had progressive or recurrent craniopharyngiomas were enrolled in this study. Nine of these patients had never received external-beam radiation therapy. Therapy consisted of 8,000,000 U/m2 IFNα-2a administered daily for 16 weeks (induction phase) followed by the same dose three times per week for an additional 32 weeks (maintenance phase). Of the 12 patients who could be evaluated, radiological studies demonstrated a response to treatment in three with predominantly cystic tumors (one minor response, one partial response, and one complete response); one of these patients also showed improvement in visual fields. The size of the cystic component of the tumors often increased temporarily during the first several months of therapy. Three patients met the criteria for progressive disease during therapy. The median time to progression was 25 months. The need for radiation therapy in patients treated with IFN was delayed for 18 to 35 months (median 25 months) in six patients. All patients developed transient flulike symptoms shortly after receiving the first dose of IFN. Other toxicities (predominantly hepatic, neurological, and cutaneous) were seen in nine (60%) of the 15 patients during the first 8 weeks of treatment but resolved after temporary discontinuation and/or dose reduction.

Conclusions. Interferon-α-2a is active against some childhood craniopharyngiomas; its toxicity precludes administration of high daily doses, and the optimum dose level and schedule remain to be defined.

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Regina I. Jakacki

P Primitive neuroectodermal tumors (PNETs) are malignant tumors with a high propensity to disseminate throughout the cerebrospinal fluid. Current treatment guidelines are largely determined by clinically based prognostic factors, the most important of which are tumor location and the extent of tumor spread. Although the cure rate for high-risk PNETs has improved, the irreversible sequelae of craniospinal axis radiation treatment in patients who survive have motivated researchers to investigate more fully which patients can safely receive less treatment. The author reviews the literature, describes currently available treatment options for patients with high-risk PNETs, and discusses strategies aimed at improving outcome and refining prognosis that are currently being explored.

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Ali I. Raja, Gabrielle A. Yeaney, Regina I. Jakacki, Ronald L. Hamilton and Ian F. Pollack

Neurocytomas are rare tumors of the central nervous system that are typically located in the ventricular system. The authors report a case of a child with neurofibromatosis Type 1 (NF1) who had a tumor of the optic nerves and chiasm with signal abnormality extending through the diencephalon, as well as an occipital lobe mass, which was presumed to be part of the visual pathway neoplasm. Because the occipital lobe lesion slowly increased in size over time, while the other areas remained stable, a biopsy was performed. Pathological evaluation revealed an extraventricular neurocytoma of extraventricular neurocytoma. To the authors' knowledge, neurocytomas have not been previously reported in patients with NF1. Because visual pathway gliomas are extremely common in children with NF1, they are often treated empirically as low-grade gliomas without histological confirmation. The importance of obtaining a biopsy in lesions that have atypical imaging features is highlighted.

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Jacky T. Yeung, Ian F. Pollack, Ashok Panigrahy and Regina I. Jakacki


Previous studies of systemic and intralesional administration of nonpegylated interferon have shown efficacy against craniopharyngioma. Pegylaion of interferon-α-2b (PI) prolongs the half-life, allowing sustained exposure of the drug over time, and enhances efficacy. The authors report the results of the use of PI in 5 children with recurrent craniopharyngiomas.


Five children, ranging in age from 9 to 15 years, with recurrent craniopharyngiomas were treated for up to 2 years with subcutaneous injections of PI at a dose of 1–3 μg/kg/week. Tumor response was assessed using MRI.


All patients had stable disease or better in response to PI. One patient experienced a recurrence after gross-total resection (GTR). She initially showed an increase in the predominantly cystic tumor after 3 months of treatment, followed by a complete response. She required no further intervention and remains without evidence of disease 10 years after starting treatment. Another patient experienced recurrence 3.3 years after subtotal resection (STR) and radiation therapy. He had complete disappearance of the predominantly cystic component after 4 months of treatment, and a small residual calcified mass remains 5 years later. The third patient experienced recurrence after 3 GTRs. He had a complete response after 7 months of treatment and remains without evidence of disease 19 months after starting treatment. The fourth patient experienced recurrence after 2 STRs. He had a 30% decrease in tumor size after 4 months of treatment, which was maintained for 12 months at which point the cyst began to increase in size. The final patient experienced recurrence after GTR and has stable disease 6 months after starting treatment with PI.


The use of PI in children with recurrent craniopharyngiomas can result in significant and durable responses and potentially delay or avoid the need for radiation therapy.