Management of pilocytic astrocytoma with diffuse leptomeningeal spread: two cases and review of the literature.

INTRODUCTION: Leptomeningeal dissemination of juvenile pilocytic astrocytoma (JPA) is a rare event. We report two children with disseminated JPAs treated with a chemotherapeutic agent, temozolomide, after progression of the disease despite surgery, traditional chemotherapy, and/or radiation therapy. CASE REPORTS: Patient 1 presented with hydrocephalus and progressive lower extremity weakness, and was found to have a suprasellar mass as well as extensive spinal disease. Ventriculoperitoneal shunting, decompressive laminectomy with spinal tumor debulking, and chemotherapy with carboplatin and vincristine were initially employed. However, disease progressed and craniospinal irradiation and temozolomide were used. Patient 1 remains in a fair condition today, 2 years later. Patient 2 presented at 8 months of age with failure to thrive. Imaging revealed a cystic lesion in the hypothalamic region with extensive subarachnoid metastatic disease to the spine. Biopsy was performed followed by chemotherapy with vincristine, cyclohexylchloroethylnitrosourea (CCNU), 6-TG, and procarbazine. Due to the continued progression of the disease, cytoreductive surgery was performed and her chemotherapeutic regimen was switched to temozolomide. Two years after initial presentation patient 2 is clinically much improved with stable residual disease. DISCUSSION: We review the literature and discuss treatment strategies for this challenging disease.