{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T04:26:55Z","timestamp":1790483215234,"version":"4.1.0"},"reference-count":25,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["PED"],"published-print":{"date-parts":[[2012,8]]},"abstract":"\nObject<\/jats:title>\nThe diagnosis of diffuse pontine tumors has largely been made on the basis of MRI since the early 1990s. In cases of tumors considered \u201ctypical,\u201d as a rule, no biopsy specimen has been obtained, and the tumors have been considered diffuse intrinsic pontine gliomas (DIPGs). There have been sporadic reports that primitive neuroectodermal tumors (PNETs) of the pons may not be distinguishable from the DIPGs by radiological imaging. This study presents 2 cases of diffuse pontine PNETs with molecular evidence that these are indeed PNETs, distinct from DIPGs, thus supporting biopsy of diffuse pontine tumors as a standard of care.<\/jats:p><\/jats:sec>\n\nMethods<\/jats:title>\nBiopsy specimens were obtained from 7 diffuse pontine tumors and snap frozen. Two of these 7 tumors were identified on the basis of pathological examination as PNETs. All 7 of the diffuse pontine tumors were analyzed for gene expression using the Affymetrix HG-U133 Plus 2.0 GeneChip microarray. Gene expression was compared with that of supratentorial PNETs, medulloblastomas, and low- and high-grade gliomas outside the brainstem.<\/jats:p><\/jats:sec>\n\nResults<\/jats:title>\nUnsupervised hierarchical clustering analysis of gene expression demonstrated that pontine PNETs are most closely related to PNETs of the supratentorial region and not with gliomas. They do not cluster with the 5 DIPGs in the study. Thirty-eight genes, including GATA3<\/jats:italic>, are uniquely differentially expressed in pontine PNETs compared with other types of pediatric brain tumors, including DIPGs and other PNETs at a false discovery rate statistical significance of less than 0.05.<\/jats:p><\/jats:sec>\n\nConclusions<\/jats:title>\nThe cluster and individual gene expression analyses indicate that pontine PNETs are intrinsically different from DIPGs. The 2 pontine PNET cases cluster with supratentorial PNETs, rather than with DIPGs, suggesting that these tumors should be treated with a PNET regimen, not with DIPG therapy. Since diagnosis by imaging is not reliable and the biology of the tumors is disparate, a biopsy should be performed to enable accurate diagnosis and direct potentially more effective treatments.<\/jats:p><\/jats:sec>","DOI":"10.3171\/2012.3.peds11316","type":"journal-article","created":{"date-parts":[[2012,6,29]],"date-time":"2012-06-29T14:05:48Z","timestamp":1340978748000},"page":"81-88","source":"Crossref","is-referenced-by-count":26,"title":["Diffuse intrinsic pontine tumors: a study of primitive neuroectodermal tumors versus the more common diffuse intrinsic pontine gliomas"],"prefix":"10.3171","volume":"10","author":[{"given":"Alexandra","family":"Sufit","sequence":"first","affiliation":[{"name":"Division of Neuro-Oncology, Department of Pediatrics,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew M.","family":"Donson","sequence":"additional","affiliation":[{"name":"Division of Neuro-Oncology, Department of Pediatrics,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diane K.","family":"Birks","sequence":"additional","affiliation":[{"name":"Division of Pediatric Neurosurgery, Department of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey A.","family":"Knipstein","sequence":"additional","affiliation":[{"name":"Division of Neuro-Oncology, Department of Pediatrics,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura Z.","family":"Fenton","sequence":"additional","affiliation":[{"name":"Division of Radiology, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Jedlicka","sequence":"additional","affiliation":[{"name":"Department of Pathology, The Children's Hospital of Colorado, University of Colorado, Aurora, Colorado"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Todd C.","family":"Hankinson","sequence":"additional","affiliation":[{"name":"Division of Pediatric Neurosurgery, Department of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael H.","family":"Handler","sequence":"additional","affiliation":[{"name":"Division of Pediatric Neurosurgery, Department of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas K.","family":"Foreman","sequence":"additional","affiliation":[{"name":"Division of Neuro-Oncology, Department of Pediatrics,"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-peds11316","series-title":"Neurosurgery","first-page":"1026","article-title":"Magnetic resonance scans should replace biopsies for the diagnosis of diffuse brain stem gliomas: a report from the Children's Cancer Group","volume":"33","author":"Albright","year":"1993"},{"key":"b2-peds11316","series-title":"J Neurooncol","doi-asserted-by":"publisher","first-page":"75","DOI":"10.1007\/s11060-010-0224-7","article-title":"Post mortem examinations in diffuse intrinsic pontine glioma: challenges and chances","volume":"101","author":"Angelini","year":"2011"},{"key":"b3-peds11316","series-title":"J Clin Oncol","doi-asserted-by":"publisher","first-page":"1266","DOI":"10.1200\/JCO.2005.04.6599","article-title":"Advances toward an understanding of brainstem gliomas","volume":"24","author":"Donaldson","year":"2006"},{"key":"b4-peds11316","series-title":"Acta Neuropathol","doi-asserted-by":"publisher","first-page":"381","DOI":"10.1007\/s00401-011-0800-8","article-title":"Medulloblastoma: clinicopathological correlates of SHH, WNT, and non-SHH\/WNT molecular subgroups","volume":"121","author":"Ellison","year":"2011"},{"key":"b5-peds11316","series-title":"Pediatr Neurosurg","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1159\/000121011","article-title":"Practical decisions in the treatment of pediatric brain stem tumors","volume":"24","author":"Epstein","year":"1996"},{"key":"b6-peds11316","series-title":"J Neurosurg Pediatr","first-page":"140","article-title":"Pontine gliomas","volume":"5","author":"Foreman","year":"2005"},{"key":"b7-peds11316","series-title":"J Neurosurg Pediatr","doi-asserted-by":"publisher","first-page":"97","DOI":"10.3171\/2011.4.PEDS1180","article-title":"Interpretation of magnetic resonance images in diffuse intrinsic pontine glioma: a survey of pediatric neurosurgeons. 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