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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]

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: 37366884Free 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

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
RNA Sequencing Reveals the Alteration of the Expression of Novel Genes in Ethanol-Treated Embryoid Bodies.
Mandal C, Kim SH, Chai JC, Oh SM, Lee YS, Jung KH, Chai YG
PLoS One 2016;11(3):e0149976. Epub 2016 Mar 1 doi: 10.1371/journal.pone.0149976. PMID: 26930486Free 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

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
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
Pineal region masses: differential diagnosis.
Smirniotopoulos JG, Rushing EJ, Mena H
Radiographics 1992 May;12(3):577-96. doi: 10.1148/radiographics.12.3.1609147. PMID: 1609147
Intracranial embryonal cell carcinoma.
Packer RJ, Sutton LN, Rorke LB, Rosenstock JG, Zimmerman RA, Littman P, Bilaniuk LT, Bruce DA, Schut L
Cancer 1984 Aug 1;54(3):520-4. doi: 10.1002/1097-0142(19840801)54:3<520::aid-cncr2820540323>3.0.co;2-e. PMID: 6733683
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

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
Isolated central nervous system relapse of non-seminomatous germ cell tumour of the testis. A case report and review of the literature.
Cohn DA, Stuart-Harris R
Oncology 2001;61(3):184-8. doi: 10.1159/000055372. PMID: 11574772
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

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
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: 34599283Free 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
INSM1 Expression Is Frequent in Primary Central Nervous System Neoplasms but Not in the Adult Brain Parenchyma.
Ames HM, Rooper LM, Laterra JJ, Eberhart CG, Rodriguez FJ
J Neuropathol Exp Neurol 2018 May 1;77(5):374-382. doi: 10.1093/jnen/nly014. PMID: 29490065Free PMC Article
Primary atypical teratoid/rhabdoid tumor of central nervous system in children: a clinicopathological analysis and review of literature in China.
Yang M, Chen X, Wang N, Zhu K, Hu YZ, Zhao Y, Shu Y, Zhao ML, Gu WZ, Tang HF
Int J Clin Exp Pathol 2014;7(5):2411-20. Epub 2014 Apr 15 PMID: 24966951Free PMC Article

Clinical prediction guides

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
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
Clinicopathological and immunohistochemical features of primary central nervous system germ cell tumors: a 24-years experience.
Gao Y, Jiang J, Liu Q
Int J Clin Exp Pathol 2014;7(10):6965-72. Epub 2014 Sep 15 PMID: 25400782Free PMC Article
Primary atypical teratoid/rhabdoid tumor of central nervous system in children: a clinicopathological analysis and review of literature in China.
Yang M, Chen X, Wang N, Zhu K, Hu YZ, Zhao Y, Shu Y, Zhao ML, Gu WZ, Tang HF
Int J Clin Exp Pathol 2014;7(5):2411-20. Epub 2014 Apr 15 PMID: 24966951Free PMC Article
Pathology of pineal region tumors.
Hirato J, Nakazato Y
J Neurooncol 2001 Sep;54(3):239-49. doi: 10.1023/a:1012721723387. PMID: 11767290

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