{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,12]],"date-time":"2026-03-12T14:47:57Z","timestamp":1773326877572,"version":"3.50.1"},"reference-count":70,"publisher":"American Academy of Pediatrics (AAP)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2000,11,1]]},"abstract":"<jats:sec>\n                  <jats:title>Objective.<\/jats:title>\n                  <jats:p>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.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Patients and Methods.<\/jats:title>\n                  <jats:p>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.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results.<\/jats:title>\n                  <jats:p>All 7 patients presented with focal cranial nerve deficits, and 2 were also hemiparetic. The median age at diagnosis was 2.7 (1\u20138 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\u201317) months. No mutation in the p53 gene was detected.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions.<\/jats:title>\n                  <jats:p>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.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1542\/peds.106.5.1045","type":"journal-article","created":{"date-parts":[[2004,8,13]],"date-time":"2004-08-13T23:50:41Z","timestamp":1092441041000},"page":"1045-1053","source":"Crossref","is-referenced-by-count":63,"title":["Primitive Neuroectodermal Tumors of the Brainstem:  Investigation of Seven Cases"],"prefix":"10.1542","volume":"106","author":[{"given":"David","family":"Zagzag","sequence":"first","affiliation":[{"name":"From the Department of Pathology,"},{"name":"Division of Neuropathology,"},{"name":"Department of Neurosurgery,"},{"name":"Kaplan Cancer Center, New York University Medical Center, New York, New York;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Douglas C.","family":"Miller","sequence":"additional","affiliation":[{"name":"From the Department of Pathology,"},{"name":"Division of Neuropathology,"},{"name":"Kaplan Cancer Center, New York University Medical Center, New York, New York;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Edmond","family":"Knopp","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery,"},{"name":"Department of Radiology, and"},{"name":"Kaplan Cancer Center, New York University Medical Center, New York, New York;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean-Pierre","family":"Farmer","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Montreal Children's Hospital, Montreal, Quebec;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Children's Medical Center, Medical College of Georgia, Augusta, Georgia; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shahriar","family":"Biria","sequence":"additional","affiliation":[{"name":"From the Department of Pathology,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angel","family":"Pellicer","sequence":"additional","affiliation":[{"name":"From the Department of Pathology,"},{"name":"Kaplan Cancer Center, New York University Medical Center, New York, New York;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fred J.","family":"Epstein","sequence":"additional","affiliation":[{"name":"Institute for Neurology and Neurosurgery, Beth Israel Medical Center, New York, New York."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffrey C.","family":"Allen","sequence":"additional","affiliation":[{"name":"Institute for Neurology and Neurosurgery, Beth Israel Medical Center, New York, New 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