ArticleBrain pathology (Zurich, Switzerland)2010
Central nervous system primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases.
Article in Brain pathology (Zurich, Switzerland), 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 66 citations in OpenAlex.
- Clinical management and therapeutic development for the rare disease rhabdomyosarcoma.Journal of Cancer · 2026Review
- Embryonal Tumors of the Central Nervous System with Multilayered Rosettes and Atypical Teratoid/Rhabdoid Tumors.Advances in experimental medicine and biology · 2023Article
- Spectrum of paired-like homeobox 2b immunoexpression in pediatric brain tumors with embryonal morphology.Pathology international · 2022Article
- GEM-IL: A highly responsive fluorescent lactate indicator.Cell reports methods · 2021Article
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- Primary intracranial Ewing's sarcoma with unusual features.International journal of clinical and experimental pathology · 2015Article
- Signals that regulate the oncogenic fate of neural stem cells and progenitors.Experimental neurology · 2014Review
- Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity.Acta neuropathologica · 2014Article
- MYCN amplification predicts poor outcome for patients with supratentorial primitive neuroectodermal tumors of the central nervous system .Neuro-oncology · 2014Article
- Supratentorial primitive neuroectodermal tumor presenting with intracranial hemorrhage in adult.Journal of neurosciences in rural practice · 2014Article
- Adults with CNS primitive neuroectodermal tumors/pineoblastomas: results of multimodal treatment according to the pediatric HIT 2000 protocol.Journal of neuro-oncology · 2014Article
- A supratentorial primitive neuroectodermal tumor presenting with intracranial hemorrhage in a 42-year-old man: a case report and review of the literature.Journal of medical case reports · 2013Article
- H3.3 G34R mutations in pediatric primitive neuroectodermal tumors of central nervous system (CNS-PNET) and pediatric glioblastomas: possible diagnostic and therapeutic implications?Journal of neuro-oncology · 2013Article
- Loss of INI1 protein expression defines a subgroup of aggressive central nervous system primitive neuroectodermal tumors.Brain pathology (Zurich, Switzerland) · 2013Article
- Definition of genetic events directing the development of distinct types of brain tumors from postnatal neural stem/progenitor cells.Cancer research · 2012Article
- Institutional experience of paediatric high-grade central nervous system tumours: an analysis of 74 patients and review of the literature.Contemporary oncology (Poznan, Poland) · 2012Article
- Gone FISHing: clinical lessons learned in brain tumor molecular diagnostics over the last decade.Brain pathology (Zurich, Switzerland) · 2011Review
- Molecular diagnostics in embryonal brain tumors.Brain pathology (Zurich, Switzerland) · 2011Review
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Central nervous system (CNS) primitive neuroectodermal tumors (PNETs) include supratentorial, brain stem, and spinal cord tumors with medulloblastoma-like histopathology. The prognostic impact of various pathologic and genetic features has not been thoroughly investigated. After re-diagnosis of three infantile cases as atypical teratoid/rhabdoid tumor (AT/RT), 33 remaining CNS PNETs were retrieved for clinicopathologic and fluorescence in situ hybridization studies. Anaplastic and/or large cell features were seen in 18 of 33 (55%) examples and survival was decreased in these patients (P = 0.036). MYCN or MYCC gene amplifications were noted in about half, with a trend towards decreased survival (P = 0.112). Polysomies of chromosomes 2 and 8 were each individually associated with decreased survival in children, with an even stronger association when combined (P = 0.013). Neither EWS gene rearrangements, nor AT/RT-like 22q deletions were encountered. We conclude that in CNS PNET: (i) routine application of INI1 immunohistochemistry helps rule out AT/RT, particularly in infants; (ii) MYC gene amplifications (especially MYCN) are common; (iii) involvement of CNS parenchyma by Ewing sarcoma/peripheral PNET is rare enough that EWS gene testing is not necessary unless significant dural involvement is present; and (iv) both anaplastic/large cell features and polysomies of 2 and 8 are associated with more aggressive clinical behavior.
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