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Cribriform Neuroepithelial Tumor

MedGen UID:
888969
•Concept ID:
C4053528
•
Neoplastic Process

Definition

A very rare, nonrhabdoid, intraventricular tumor with relatively favorable prognosis. It is characterized by the presence of neuroepithelial cells forming cribriform patterns, trabeculae, epithelial membrane antigen immunopositivity on epithelial surfaces, and loss of nuclear INI 1 expression. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCribriform Neuroepithelial Tumor

Professional guidelines

PubMed

Nimotuzumab therapy in the treatment of pediatric central nervous system tumors: single-center experience.
Susam-Sen H, Varan A, Bajin İ, Göçmen R, Aydın B, Yalcin B, Kurucu N, Kutluk T, Bayhan T, Akyuz C
Naunyn Schmiedebergs Arch Pharmacol 2021 Aug;394(8):1769-1777. Epub 2021 Jun 21 doi: 10.1007/s00210-021-02109-y. PMID: 34151393

Recent clinical studies

Etiology

Evaluation and Diagnosis of Central Nervous System Embryonal Tumors (Non-Medulloblastoma).
Cotter JA, Judkins AR
Pediatr Dev Pathol 2022 Jan-Feb;25(1):34-45. doi: 10.1177/10935266211018554. PMID: 35168419
Nimotuzumab therapy in the treatment of pediatric central nervous system tumors: single-center experience.
Susam-Sen H, Varan A, Bajin İ, Göçmen R, Aydın B, Yalcin B, Kurucu N, Kutluk T, Bayhan T, Akyuz C
Naunyn Schmiedebergs Arch Pharmacol 2021 Aug;394(8):1769-1777. Epub 2021 Jun 21 doi: 10.1007/s00210-021-02109-y. PMID: 34151393
The expanding family of SMARCB1(INI1)-deficient neoplasia: implications of phenotypic, biological, and molecular heterogeneity.
Agaimy A
Adv Anat Pathol 2014 Nov;21(6):394-410. doi: 10.1097/PAP.0000000000000038. PMID: 25299309
Choroid plexus carcinomas are characterized by complex chromosomal alterations related to patient age and prognosis.
Ruland V, Hartung S, Kordes U, Wolff JE, Paulus W, Hasselblatt M
Genes Chromosomes Cancer 2014 May;53(5):373-80. Epub 2014 Jan 30 doi: 10.1002/gcc.22148. PMID: 24478045
Genomic aberrations in pediatric gliomas and embryonal tumors.
Dahlback HS, Brandal P, Gorunova L, Widing E, Meling TR, Krossnes BK, Heim S
Genes Chromosomes Cancer 2011 Oct;50(10):788-99. Epub 2011 Jun 29 doi: 10.1002/gcc.20898. PMID: 21717527

Diagnosis

Transcallosal and endoscopic hybrid approach to a rare entity of pediatric intraventricular tumors-cribriform neuroepithelial tumor: a case report and literature review.
Mete EB, Eray HA, Orhan O, Zaimoglu M, Ersoz CC, Dogan I
Childs Nerv Syst 2023 May;39(5):1123-1129. Epub 2023 Mar 8 doi: 10.1007/s00381-023-05897-1. PMID: 36884098
Evaluation and Diagnosis of Central Nervous System Embryonal Tumors (Non-Medulloblastoma).
Cotter JA, Judkins AR
Pediatr Dev Pathol 2022 Jan-Feb;25(1):34-45. doi: 10.1177/10935266211018554. PMID: 35168419
SMARCB1-deficient Tumors of Childhood: A Practical Guide.
Pawel BR
Pediatr Dev Pathol 2018 Jan-Feb;21(1):6-28. Epub 2017 Dec 27 doi: 10.1177/1093526617749671. PMID: 29280680
All things rhabdoid and SMARC: An enigmatic exploration with Dr. Louis P. Dehner.
Fuller CE
Semin Diagn Pathol 2016 Nov;33(6):427-440. Epub 2016 Aug 31 doi: 10.1053/j.semdp.2016.08.003. PMID: 27745735
Uncommon pediatric tumors of the posterior fossa: pathologic and molecular features.
Dunham C
Childs Nerv Syst 2015 Oct;31(10):1729-37. Epub 2015 Sep 9 doi: 10.1007/s00381-015-2735-1. PMID: 26351226

Therapy

Nimotuzumab therapy in the treatment of pediatric central nervous system tumors: single-center experience.
Susam-Sen H, Varan A, Bajin İ, Göçmen R, Aydın B, Yalcin B, Kurucu N, Kutluk T, Bayhan T, Akyuz C
Naunyn Schmiedebergs Arch Pharmacol 2021 Aug;394(8):1769-1777. Epub 2021 Jun 21 doi: 10.1007/s00210-021-02109-y. PMID: 34151393
Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis.
Hasselblatt M, Oyen F, Gesk S, Kordes U, Wrede B, Bergmann M, Schmid H, Frühwald MC, Schneppenheim R, Siebert R, Paulus W
J Neuropathol Exp Neurol 2009 Dec;68(12):1249-55. doi: 10.1097/NEN.0b013e3181c06a51. PMID: 19915490

Prognosis

Evaluation and Diagnosis of Central Nervous System Embryonal Tumors (Non-Medulloblastoma).
Cotter JA, Judkins AR
Pediatr Dev Pathol 2022 Jan-Feb;25(1):34-45. doi: 10.1177/10935266211018554. PMID: 35168419
Nimotuzumab therapy in the treatment of pediatric central nervous system tumors: single-center experience.
Susam-Sen H, Varan A, Bajin İ, Göçmen R, Aydın B, Yalcin B, Kurucu N, Kutluk T, Bayhan T, Akyuz C
Naunyn Schmiedebergs Arch Pharmacol 2021 Aug;394(8):1769-1777. Epub 2021 Jun 21 doi: 10.1007/s00210-021-02109-y. PMID: 34151393
SWI/SNF deficient central nervous system neoplasms.
Cai C
Semin Diagn Pathol 2021 May;38(3):167-174. Epub 2021 Mar 15 doi: 10.1053/j.semdp.2021.03.003. PMID: 33762087
SMARCB1-deficient Tumors of Childhood: A Practical Guide.
Pawel BR
Pediatr Dev Pathol 2018 Jan-Feb;21(1):6-28. Epub 2017 Dec 27 doi: 10.1177/1093526617749671. PMID: 29280680
Cribriform neuroepithelial tumor: molecular characterization of a SMARCB1-deficient non-rhabdoid tumor with favorable long-term outcome.
Johann PD, Hovestadt V, Thomas C, Jeibmann A, Heß K, Bens S, Oyen F, Hawkins C, Pierson CR, Aldape K, Kim SP, Widing E, Sumerauer D, Hauser P, van Landeghem F, Ryzhova M, Korshunov A, Capper D, Jones DTW, Pfister SM, Schneppenheim R, Siebert R, Paulus W, Frühwald MC, Kool M, Hasselblatt M
Brain Pathol 2017 Jul;27(4):411-418. Epub 2016 Aug 11 doi: 10.1111/bpa.12413. PMID: 27380723Free PMC Article

Clinical prediction guides

Nimotuzumab therapy in the treatment of pediatric central nervous system tumors: single-center experience.
Susam-Sen H, Varan A, Bajin İ, Göçmen R, Aydın B, Yalcin B, Kurucu N, Kutluk T, Bayhan T, Akyuz C
Naunyn Schmiedebergs Arch Pharmacol 2021 Aug;394(8):1769-1777. Epub 2021 Jun 21 doi: 10.1007/s00210-021-02109-y. PMID: 34151393
The expanding family of SMARCB1(INI1)-deficient neoplasia: implications of phenotypic, biological, and molecular heterogeneity.
Agaimy A
Adv Anat Pathol 2014 Nov;21(6):394-410. doi: 10.1097/PAP.0000000000000038. PMID: 25299309
Cribriform neuroepithelial tumor arising in the lateral ventricle.
Arnold MA, Stallings-Archer K, Marlin E, Grondin R, Olshefski R, Biegel JA, Pierson CR
Pediatr Dev Pathol 2013 Jul-Aug;16(4):301-7. Epub 2013 Mar 15 doi: 10.2350/12-12-1287-CR.1. PMID: 23495723

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