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Embryonal carcinoma of the central nervous system

MedGen UID:: 272566
•Concept ID:: C1333377
•: Neoplastic Process

Synonyms:	Central Nervous System Embryonal Carcinoma; central nervous system embryonal carcinoma; embryonal carcinoma; Embryonal Carcinoma of CNS; embryonal carcinoma of CNS; Embryonal Carcinoma of the Central Nervous System; embryonal carcinoma of the central nervous system; Embryonal carcinoma of the CNS; embryonal carcinoma of the CNS

Monarch Initiative:	MONDO:0018843
Orphanet:	ORPHA48736

Definition

A malignant germ cell tumor of the central nervous system composed of large cells that proliferate in cohesive nests and sheets. In rare occasions, tumor cells may replicate the structure of the early embryo, forming ""embryoid bodies"" replete with germ discs and miniature amniotic cavities. Additional morphologic characteristics include enlarged nucleoli, abundant clear to somewhat violet-hued cytoplasm, a high mitotic rate and zones of coagulative necrosis. (Adapted from WHO) [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVEmbryonal carcinoma of the central nervous system

Neoplasm
- Neoplasm by Morphology
  - Germ cell tumor
    - Extragonadal germ cell tumor
      - Central nervous system germ cell tumor
        Central nervous system nongerminomatous germ cell tumor
        Embryonal carcinoma of the central nervous system
        Adult central nervous system embryonal carcinoma
        Childhood central nervous system embryonal carcinoma
        Sellar Embryonal Carcinoma

Professional guidelines

PubMed

The Role of Immunotherapy in the Treatment of Rare Central Nervous System Tumors.

Rodriguez A, Kamiya-Matsuoka C, Majd NK
Curr Oncol 2023 May 25;30(6):5279-5298. doi: 10.3390/curroncol30060401. PMID: 37366884 Free PMC Article

Central nervous system germ cell tumors: classification, clinical features, and treatment with a historical overview.

Fujimaki T
J Child Neurol 2009 Nov;24(11):1439-45. doi: 10.1177/0883073809342127. PMID: 19841431

The treatment of cranial germ cell tumours.

Brandes AA, Pasetto LM, Monfardini S
Cancer Treat Rev 2000 Aug;26(4):233-42. doi: 10.1053/ctrv.2000.0169. PMID: 10913379

See all (10)

Recent clinical studies

Etiology

Loss of BRG1 (SMARCA4) Immunoexpression in a Pediatric Non-Central Nervous System Tumor Cohort.

Saunders J, Ingley K, Wang XQ, Harvey M, Armstrong L, Ng T, Dunham C, Bush J
Pediatr Dev Pathol 2020 Mar-Apr;23(2):132-138. Epub 2019 Aug 12 doi: 10.1177/1093526619869154. PMID: 31403913

Diseases Predisposing to Adrenocortical Malignancy (Li-Fraumeni Syndrome, Beckwith-Wiedemann Syndrome, and Carney Complex).

Jouinot A, Bertherat J
Exp Suppl 2019;111:149-169. doi: 10.1007/978-3-030-25905-1_9. PMID: 31588532

Molecular diagnostics in embryonal brain tumors.

Eberhart CG
Brain Pathol 2011 Jan;21(1):96-104. doi: 10.1111/j.1750-3639.2010.00455.x. PMID: 21129063 Free PMC Article

Gastrointestinal and retroperitoneal manifestations of type 1 neurofibromatosis.

Basile U, Cavallaro G, Polistena A, Giustini S, Orlando G, Cotesta D, Petramala L, Letizia C, Calvieri S, De Toma G
J Gastrointest Surg 2010 Jan;14(1):186-94. doi: 10.1007/s11605-009-0940-5. PMID: 19495890

Immunohistochemistry of central nervous system tumors. Its contributions to neurosurgical diagnosis.

Bonnin JM, Rubinstein LJ
J Neurosurg 1984 Jun;60(6):1121-33. doi: 10.3171/jns.1984.60.6.1121. PMID: 6202856

See all (50)

Diagnosis

Histopathology of pineal germ cell tumors.

Vasiljevic A, Szathmari A, Champier J, Fèvre-Montange M, Jouvet A
Neurochirurgie 2015 Apr-Jun;61(2-3):130-7. Epub 2014 Apr 13 doi: 10.1016/j.neuchi.2013.06.006. PMID: 24726316

Gastrointestinal and retroperitoneal manifestations of type 1 neurofibromatosis.

Central nervous system germ cell tumors: classification, clinical features, and treatment with a historical overview.

Fujimaki T
J Child Neurol 2009 Nov;24(11):1439-45. doi: 10.1177/0883073809342127. PMID: 19841431

Central nervous system tumors.

Scheithauer BW, Bruner JM
Clin Lab Med 1987 Mar;7(1):157-79. PMID: 3030612

Immunohistochemistry of central nervous system tumors. Its contributions to neurosurgical diagnosis.

Bonnin JM, Rubinstein LJ
J Neurosurg 1984 Jun;60(6):1121-33. doi: 10.3171/jns.1984.60.6.1121. PMID: 6202856

See all (91)

Therapy

Clinical and Radiologic Features of Pediatric Basal Ganglia Germ Cell Tumors.

Zhang S, Liang G, Ju Y, You C
World Neurosurg 2016 Nov;95:516-524.e1. Epub 2016 Aug 24 doi: 10.1016/j.wneu.2016.08.072. PMID: 27567571

Tumorigenicity issues of embryonic carcinoma-derived stem cells: relevance to surgical trials using NT2 and hNT neural cells.

Newman MB, Misiuta I, Willing AE, Zigova T, Karl RC, Borlongan CV, Sanberg PR
Stem Cells Dev 2005 Feb;14(1):29-43. doi: 10.1089/scd.2005.14.29. PMID: 15725742

Germ cell tumours of the central nervous system: treatment consideration based on 111 cases and their long-term clinical outcomes.

Sawamura Y, Ikeda J, Shirato H, Tada M, Abe H
Eur J Cancer 1998 Jan;34(1):104-10. doi: 10.1016/s0959-8049(97)10045-4. PMID: 9624246

Treatment of nongerminomatous germ-cell tumors of the pineal region.

Herrmann HD, Westphal M, Winkler K, Laas RW, Schulte FJ
Neurosurgery 1994 Mar;34(3):524-9; discussion 529. doi: 10.1227/00006123-199403000-00021. PMID: 7514765

Management of extragonadal germ-cell tumors and the significance of bilateral testicular biopsies.

Daugaard G, Rørth M, von der Maase H, Skakkebaek NE
Ann Oncol 1992 Apr;3(4):283-9. doi: 10.1093/oxfordjournals.annonc.a058180. PMID: 1327076

See all (25)

Prognosis

Co-fractionation-mass spectrometry to characterize native mitochondrial protein assemblies in mammalian neurons and brain.

Zilocchi M, Rahmatbakhsh M, Moutaoufik MT, Broderick K, Gagarinova A, Jessulat M, Phanse S, Aoki H, Aly KA, Babu M
Nat Protoc 2023 Dec;18(12):3918-3973. Epub 2023 Nov 20 doi: 10.1038/s41596-023-00901-z. PMID: 37985878

MALIGNANT TUMORS IN PEDIATRIC POPULATION OF UKRAINE: TRENDS AND STRUCTURAL FEATURES.

Shipko AF, Fedorenko ZP, Ryzhov AY
Exp Oncol 2022 Dec;44(4):314-319. doi: 10.32471/exp-oncology.2312-8852.vol-44-no-4.19109. PMID: 36811535

DICER1 tumor predisposition syndrome: an evolving story initiated with the pleuropulmonary blastoma.

González IA, Stewart DR, Schultz KAP, Field AP, Hill DA, Dehner LP
Mod Pathol 2022 Jan;35(1):4-22. Epub 2021 Oct 1 doi: 10.1038/s41379-021-00905-8. PMID: 34599283 Free PMC Article

Loss of BRG1 (SMARCA4) Immunoexpression in a Pediatric Non-Central Nervous System Tumor Cohort.

Saunders J, Ingley K, Wang XQ, Harvey M, Armstrong L, Ng T, Dunham C, Bush J
Pediatr Dev Pathol 2020 Mar-Apr;23(2):132-138. Epub 2019 Aug 12 doi: 10.1177/1093526619869154. PMID: 31403913

The MYCN oncogene as a specific and selective drug target for peripheral and central nervous system tumors.

Pession A, Tonelli R
Curr Cancer Drug Targets 2005 Jun;5(4):273-83. doi: 10.2174/1568009054064606. PMID: 15975048

See all (69)