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PEDIATRICS Vol. 106 No. 5 November 2000, pp. 1045-1053

Primitive Neuroectodermal Tumors of the Brainstem: Investigation of Seven Cases

Received Jan 29, 1999; accepted Apr 17, 2000.

David Zagzag*, Dagger , §, , Douglas C. Miller*, Dagger , , Edmond Knopp§, parallel , , Jean-Pierre Farmer#, Mark Lee**, Shahriar Biria*, Angel Pellicer*, , Fred J. EpsteinDagger Dagger , and Jeffrey C. AllenDagger Dagger

From the * Department of Pathology, Dagger  Division of Neuropathology, § Department of Neurosurgery, parallel  Department of Radiology, and  Kaplan Cancer Center, New York University Medical Center, New York, New York; # Department of Neurosurgery, Montreal Children's Hospital, Montreal, Quebec; ** Department of Neurosurgery, Children's Medical Center, Medical College of Georgia, Augusta, Georgia; and Dagger Dagger  Institute for Neurology and Neurosurgery, Beth Israel Medical Center, New York, New York.

Objective.  We discuss the clinical aspects, pathology, and molecular genetics of 7 patients with primitive neuroectodermal tumors (PNETs) arising in the brainstem that were treated at our institution from 1986 through 1995. Most neuro-oncologists avoid performing biopsies in children with pontine tumors. This article raises the question as to whether biopsies should be performed, because treatment recommendations might differ if a PNET was diagnosed rather than a pontine glioma.

Patients and Methods.  We reviewed the clinical neuro-oncology database and the files of the Division of Neuropathology at New York University Medical Center from 1986 through 1995 and identified 7 histologically confirmed PNETs arising in the brainstem among 146 pediatric brainstem tumors. The clinical, neuroradiological, and neuropathological data were reviewed. Postmortem examinations were performed in 2 cases. Formalin-fixed, paraffin-embedded tumor tissues were also available in 6 of 7 patients that were tested for p53 gene mutations using single-strand conformation polymorphism analysis. We also tested 9 cerebellar PNETs, 9 brainstem gliomas, and 3 normal brains for p53 gene mutations as controls.

Results.  All 7 patients presented with focal cranial nerve deficits, and 2 were also hemiparetic. The median age at diagnosis was 2.7 (1-8 years). Magnetic resonance imaging (MRI) characteristics included a focal intrinsic exophytic nonenhancing brainstem lesion that had low T1-weighted and high T2-weighted signals. Hydrocephalus was present in 5 patients at diagnosis, 3 of whom had leptomeningeal dissemination. Meningeal dissemination occurred later in the course of the disease in 3 other patients. Five children required shunts at diagnosis and another 2 at recurrence. Despite therapy, all 7 PNET patients died within 17 months of diagnosis with a mean survival of 8 (4-17) months. No mutation in the p53 gene was detected.

Conclusions.  Brainstem PNETs tend to arise at a younger age than brainstem gliomas and medulloblastomas. The MRI pattern suggests a localized rather than a diffuse intrinsic nonenhancing brainstem tumor. Like other PNETs, brainstem PNETs have a high predilection to disseminate within the central nervous system. The absence of p53 mutations is similar to other PNETs. Despite their origin close to the cerebellum, brainstem PNETs exhibit a more aggressive behavior and result in worse clinical outcomes than do cerebellar PNETs.  Key words:  primitive neuroectodermal tumors, brainstem, children, p53 mutation.




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