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Central primitive neuroectodermal tumor

MedGen UID:
854517
•Concept ID:
C3887678
•
Neoplastic Process
Synonym: Central nervous system primitive neuroectodermal neoplasm
SNOMED CT: Central primitive neuroectodermal tumor (39781001); CPNET (39781001)
 
HPO: HP:0030070
Monarch Initiative: MONDO:0000640

Definition

A primitive neuroectodermal neoplasm that occurs in the central nervous system. [from HPO]

Professional guidelines

PubMed

Medulloblastoma: Molecular understanding, treatment evolution, and new developments.
Liu X, Ding C, Tan W, Zhang A
Pharmacol Ther 2020 Jun;210:107516. Epub 2020 Feb 24 doi: 10.1016/j.pharmthera.2020.107516. PMID: 32105673
Disrupting the CD47-SIRPα anti-phagocytic axis by a humanized anti-CD47 antibody is an efficacious treatment for malignant pediatric brain tumors.
Gholamin S, Mitra SS, Feroze AH, Liu J, Kahn SA, Zhang M, Esparza R, Richard C, Ramaswamy V, Remke M, Volkmer AK, Willingham S, Ponnuswami A, McCarty A, Lovelace P, Storm TA, Schubert S, Hutter G, Narayanan C, Chu P, Raabe EH, Harsh G 4th, Taylor MD, Monje M, Cho YJ, Majeti R, Volkmer JP, Fisher PG, Grant G, Steinberg GK, Vogel H, Edwards M, Weissman IL, Cheshier SH
Sci Transl Med 2017 Mar 15;9(381) doi: 10.1126/scitranslmed.aaf2968. PMID: 28298418
Guidelines for imaging and staging of neuroblastic tumors: consensus report from the International Neuroblastoma Risk Group Project.
Brisse HJ, McCarville MB, Granata C, Krug KB, Wootton-Gorges SL, Kanegawa K, Giammarile F, Schmidt M, Shulkin BL, Matthay KK, Lewington VJ, Sarnacki S, Hero B, Kaneko M, London WB, Pearson AD, Cohn SL, Monclair T; International Neuroblastoma Risk Group Project
Radiology 2011 Oct;261(1):243-57. Epub 2011 May 17 doi: 10.1148/radiol.11101352. PMID: 21586679

Recent clinical studies

Etiology

Two Tumors in 1: What Should be the Therapeutic Target? Pediatric Germ Cell Tumor With Somatic Malignant Transformation.
Faure Conter C, Fresneau B, Thebaud E, Bertrand A, Dijoud F, Rome A, Dumesnil C, Castex MP, Ghanem A, Orbach D
J Pediatr Hematol Oncol 2017 Jul;39(5):388-394. doi: 10.1097/MPH.0000000000000823. PMID: 28375941

Diagnosis

Clinical management of a unique case of PNET of the uterus during pregnancy, and review of the literature.
De Nola R, Di Naro E, Schonauer LM, Lucarelli G, Battaglia M, Fiore MG, Mastrolia SA, Loverro G
Medicine (Baltimore) 2018 Jan;97(2):e9505. doi: 10.1097/MD.0000000000009505. PMID: 29480840Free PMC Article
Childhood pheochromocytoma in a survivor of central primitive neuroectodermal tumor.
Nakano Y, Fujimaru R, Ishii K, Sakamoto H, Inoue T, Sako M, Yamada H
Pediatr Int 2013 Aug;55(4):e100-2. doi: 10.1111/ped.12074. PMID: 23910809
Primary intradural extraosseous Ewing sarcoma of the spine: case report and literature review.
Karikari IO, Mehta AI, Nimjee S, Hodges TR, Tibaleka J, Montgomery C, Simpson L, Cummings TJ, Bagley CA
Neurosurgery 2011 Oct;69(4):E995-9. doi: 10.1227/NEU.0b013e318223b7c7. PMID: 21572359
Intracranial Ewing sarcoma.
Mazur MA, Gururangan S, Bridge JA, Cummings TJ, Mukundan S, Fuchs H, Larrier N, Halperin EC
Pediatr Blood Cancer 2005 Nov;45(6):850-6. doi: 10.1002/pbc.20430. PMID: 15929128
Ovarian neoplasms composed of small round cells: a review.
McCluggage WG
Adv Anat Pathol 2004 Nov;11(6):288-96. doi: 10.1097/01.pap.0000138146.357376.1e. PMID: 15505529

Therapy

Primary intradural extraosseous Ewing sarcoma of the spine: case report and literature review.
Karikari IO, Mehta AI, Nimjee S, Hodges TR, Tibaleka J, Montgomery C, Simpson L, Cummings TJ, Bagley CA
Neurosurgery 2011 Oct;69(4):E995-9. doi: 10.1227/NEU.0b013e318223b7c7. PMID: 21572359

Prognosis

Two Tumors in 1: What Should be the Therapeutic Target? Pediatric Germ Cell Tumor With Somatic Malignant Transformation.
Faure Conter C, Fresneau B, Thebaud E, Bertrand A, Dijoud F, Rome A, Dumesnil C, Castex MP, Ghanem A, Orbach D
J Pediatr Hematol Oncol 2017 Jul;39(5):388-394. doi: 10.1097/MPH.0000000000000823. PMID: 28375941
Childhood pheochromocytoma in a survivor of central primitive neuroectodermal tumor.
Nakano Y, Fujimaru R, Ishii K, Sakamoto H, Inoue T, Sako M, Yamada H
Pediatr Int 2013 Aug;55(4):e100-2. doi: 10.1111/ped.12074. PMID: 23910809
Ewing's family of tumors involving structures related to the central nervous system: a review.
Hadfield MG, Quezado MM, Williams RL, Luo VY
Pediatr Dev Pathol 2000 May-Jun;3(3):203-10. doi: 10.1007/s100249910026. PMID: 10742406
Are clinical parameters valuable prognostic factors in childhood primitive neuroectodermal tumors? A multivariate analysis of 105 cases.
Michiels EM, Heikens J, Jansen MJ, Oldenburger F, Voûte T
Radiother Oncol 2000 Mar;54(3):229-38. doi: 10.1016/s0167-8140(00)00155-9. PMID: 10738081

Clinical prediction guides

Clinical management of a unique case of PNET of the uterus during pregnancy, and review of the literature.
De Nola R, Di Naro E, Schonauer LM, Lucarelli G, Battaglia M, Fiore MG, Mastrolia SA, Loverro G
Medicine (Baltimore) 2018 Jan;97(2):e9505. doi: 10.1097/MD.0000000000009505. PMID: 29480840Free PMC Article
Two Tumors in 1: What Should be the Therapeutic Target? Pediatric Germ Cell Tumor With Somatic Malignant Transformation.
Faure Conter C, Fresneau B, Thebaud E, Bertrand A, Dijoud F, Rome A, Dumesnil C, Castex MP, Ghanem A, Orbach D
J Pediatr Hematol Oncol 2017 Jul;39(5):388-394. doi: 10.1097/MPH.0000000000000823. PMID: 28375941
Are clinical parameters valuable prognostic factors in childhood primitive neuroectodermal tumors? A multivariate analysis of 105 cases.
Michiels EM, Heikens J, Jansen MJ, Oldenburger F, Voûte T
Radiother Oncol 2000 Mar;54(3):229-38. doi: 10.1016/s0167-8140(00)00155-9. PMID: 10738081
Constitutional mutations of the hSNF5/INI1 gene predispose to a variety of cancers.
Sévenet N, Sheridan E, Amram D, Schneider P, Handgretinger R, Delattre O
Am J Hum Genet 1999 Nov;65(5):1342-8. doi: 10.1086/302639. PMID: 10521299Free PMC Article

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