U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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
Molecular Classification and Management of Rare Pediatric Embryonal Brain Tumors.
Sin-Chan P, Li BK, Ho B, Fonseca A, Huang A
Curr Oncol Rep 2018 Jul 11;20(9):69. doi: 10.1007/s11912-018-0717-7. PMID: 29995179
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

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

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
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

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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...