title: Malignant transformation of a chiasmatic pilocytic astrocytoma in a patient with diencephalic syndrome.
abstract: Chiasmatic gliomas with metastatic spread are rare in children and are usually associated with diencephalic syndrome. They are mostly pilocytic astrocytomas and their transformation to high-grade astrocytomas has never previously been reported in the pediatric population. We report leptomeningeal spread of a chiasmatic pilocytic astrocytoma in a child presenting with diencephalic syndrome. He was treated with chemotherapy and radiation. The tumor recurred with transformation into a high-grade astrocytoma. Radiation therapy may have played a role in transformation of the tumor, but more research is needed to further clarify the biological behavior of this tumor.
authors.affiliation: Department of Radiology, Indiana University, Division of Neuroradiology, James Whitcomb Riley Children's Hospital, 702 Barnhill Drive, MRI Suite, Indianapolis 46202-5200, USA. medward@IUPUI.edu
authors.forename: Ester J
authors.initials: EJ
authors.lastname: van der Wal
authors.forename: Biagio
authors.initials: B
authors.lastname: Azzarelli
authors.forename: Mary
authors.initials: M
authors.lastname: Edwards-Brown
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D001254:Astrocytoma / Q000473:pathology / Q000556:secondary* / Q000628:therapy; D001707:Biopsy, Needle; D002471:Cell Transformation, Neoplastic / Q000473:pathology*; D002675:Child, Preschool; D003131:Combined Modality Therapy; D016371:Cranial Irradiation; D004027:Diencephalon / Q000503:physiopathology*; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D008577:Meningeal Neoplasms / Q000473:pathology / Q000556:secondary* / Q000628:therapy; D009361:Neoplasm Invasiveness / Q000473:pathology*; D009897:Optic Chiasm / Q000473:pathology*; D019574:Optic Nerve Neoplasms / Q000473:pathology* / Q000628:therapy; D018570:Risk Assessment; D013577:Syndrome