title: Primary extracranial neuroblastoma with central nervous system metastases characterization by clinicopathologic findings and neuroimaging.
abstract: The authors report the clinicopathologic and neuroimaging findings in ten children with primary abdominal or thoracic neuroblastoma who relapsed in the central nervous system (CNS) without evidence of concurrent intracranial extension from adjacent bone, dura, or dural sinus metastases. At diagnosis, the patients ranged in age from 0.3 to 4.5 years (median, 2 years). Their times to CNS relapse ranged from 2 to 34 months from diagnosis. In seven patients the relapse occurred from 1 to 14 months after elective discontinuation of therapy. In four patients, the CNS relapse was the primary (isolated) adverse event. Four patients could not be treated at the time of relapse, and they died within 7 days of progressive CNS disease. In the remaining group, craniospinal irradiation with or without administration of a platinum compound and an epipodophyllotoxin caused complete CNS remissions lasting 4, 5, 16, and 62+ months. Neuroimaging and autopsy findings indicated that cerebrospinal fluid is the major pathway for neuraxis dissemination by neuroblastoma cells. There was no evidence of dural penetration in any patient. The possibility of relapse in the neuraxis should be considered for any patient with neuroblastoma who had neurologic deterioration. A combination of craniospinal radiation and administration of a platinum compound and an epipodophyllotoxin will induce complete responses in some patients with neuraxis involvement by neuroblastoma, but the risk of subsequent failure outside the CNS remains high.
authors.affiliation: Department of Hematology/Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105.
authors.forename: S J
authors.initials: SJ
authors.lastname: Kellie
authors.forename: F A
authors.initials: FA
authors.lastname: Hayes
authors.forename: L
authors.initials: L
authors.lastname: Bowman
authors.forename: E H
authors.initials: EH
authors.lastname: Kovnar
authors.forename: J
authors.initials: J
authors.lastname: Langston
authors.forename: J J
authors.initials: JJ
authors.lastname: Jenkins
authors.forename: W J
authors.initials: WJ
authors.lastname: Pao
authors.forename: R
authors.initials: R
authors.lastname: Ducos
authors.forename: A A
authors.initials: AA
authors.lastname: Green
mesh_terms: D000008:Abdominal Neoplasms / Q000473:pathology; D000971:Antineoplastic Combined Chemotherapy Protocols; D016543:Central Nervous System Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology / Q000556:secondary* / Q000628:therapy; D002675:Child, Preschool; D003131:Combined Modality Therapy; D016371:Cranial Irradiation; D003952:Diagnostic Imaging; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009364:Neoplasm Recurrence, Local; D009447:Neuroblastoma / Q000000981:diagnostic imaging / Q000473:pathology / Q000556:secondary* / Q000628:therapy; D012074:Remission Induction; D013899:Thoracic Neoplasms / Q000473:pathology; D014057:Tomography, X-Ray Computed