Spinal cord primitive neuroectodermal tumor

MedGen UID:: 234805
•Concept ID:: C1336048
•: Neoplastic Process

Synonyms:	primitive neuroectodermal neoplasm of spinal cord; primitive neuroectodermal neoplasm of the spinal cord; primitive neuroectodermal tumor of spinal cord; primitive neuroectodermal tumor of the spinal cord; primitive neuroectodermal tumour of spinal cord; primitive neuroectodermal tumour of the spinal cord; Spinal Cord Embryonal Tumor, NOS; Spinal Cord Embryonal Tumor, Not Otherwise Specified; spinal cord PNET; spinal cord primitive neuroectodermal neoplasm; spinal cord primitive neuroectodermal tumor

Monarch Initiative:	MONDO:0006426

Definition

A central nervous system embryonal tumor, not otherwise specified arising from the spinal cord. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVSpinal cord primitive neuroectodermal tumor

Pathological process
- Disease
  - Neoplasm
    - Embryonal neoplasm
      - Central Nervous System Embryonal Tumor
        Central primitive neuroectodermal tumor
        Spinal cord primitive neuroectodermal tumor

Professional guidelines

PubMed

Treatment and outcome of neuroblastoma with intraspinal extension: A systematic review.

Kraal K, Blom T, van Noesel M, Kremer L, Caron H, Tytgat G, van der Pal H
Pediatr Blood Cancer 2017 Aug;64(8) Epub 2017 Feb 2 doi: 10.1002/pbc.26451. PMID: 28150396

The genetic basis of intradural spinal tumors and its impact on clinical treatment.

Karsy M, Guan J, Sivakumar W, Neil JA, Schmidt MH, Mahan MA
Neurosurg Focus 2015 Aug;39(2):E3. doi: 10.3171/2015.5.FOCUS15143. PMID: 26235020

Diagnosis and management of brain and spinal cord tumors in the neonate.

Hwang SW, Su JM, Jea A
Semin Fetal Neonatal Med 2012 Aug;17(4):202-206. Epub 2012 Mar 30 doi: 10.1016/j.siny.2012.03.001. PMID: 22465555

See all (37)

Recent clinical studies

Etiology

Purely intramedullary spinal cord primitive neuroectodermal tumor: case report and review of the literature.

Otero-Rodríguez A, Hinojosa J, Esparza J, Muñoz MJ, Iglesias S, Rodríguez-Gil Y, Ricoy JR
Neurocirugia (Astur) 2009 Aug;20(4):381-6; discussion 386-7. doi: 10.1016/s1130-1473(09)70159-9. PMID: 19688140

See all (1)

Diagnosis

Intramedullary spinal cord primitive neuroectodermal tumor presenting with hydrocephalus.

Alexiou GA, Siozos G, Stefanaki K, Moschovi M, Prodromou N
J Child Neurol 2013 Feb;28(2):246-50. Epub 2012 Apr 24 doi: 10.1177/0883073812441061. PMID: 22532539

Purely intramedullary spinal cord primitive neuroectodermal tumor: case report and review of the literature.

Fanconi anemia and biallelic BRCA2 mutation diagnosed in a young child with an embryonal CNS tumor.

Dewire MD, Ellison DW, Patay Z, McKinnon PJ, Sanders RP, Gajjar A
Pediatr Blood Cancer 2009 Dec;53(6):1140-2. doi: 10.1002/pbc.22139. PMID: 19530235 Free PMC Article

See all (3)