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Yolk sac tumor of central nervous system

MedGen UID:
234492
•Concept ID:
C1337040
•
Neoplastic Process
Synonyms: Central Nervous System Yolk Sac Tumor; central nervous system yolk Sac tumor; central nervous system yolk Sac tumour; CNS Yolk Sac Tumor; CNS yolk Sac tumor; CNS yolk Sac tumour; Endodermal sinus neoplasm of central nervous system; Endodermal sinus tumor of central nervous system; endodermal sinus tumor of central nervous system; endodermal sinus tumor of CNS; Endodermal sinus tumor of CNS; endodermal sinus tumour of central nervous system; endodermal sinus tumour of CNS; intracranial endodermal sinus tumor; Intracranial endodermal sinus tumor; intracranial endodermal sinus tumour; Intracranial yolk sac tumor; intracranial yolk sac tumor; intracranial yolk sac tumour; Yolk Sac Tumor of Central Nervous System; yolk sac tumor of central nervous system; yolk Sac tumor of central nervous system; Yolk Sac Tumor of CNS; Yolk sac tumor of CNS; yolk Sac tumor of CNS; yolk sac tumor of CNS; Yolk Sac Tumor of the Central Nervous System; yolk Sac tumor of the central nervous system; Yolk Sac Tumor of the CNS; yolk Sac tumor of the CNS; yolk Sac tumour of central nervous system; yolk Sac tumour of CNS; yolk sac tumour of CNS; yolk Sac tumour of the central nervous system; yolk Sac tumour of the CNS
SNOMED CT: Endodermal sinus tumor of central nervous system (1197280002); Endodermal sinus neoplasm of central nervous system (1197280002); Yolk sac tumor of central nervous system (1197280002)
 
Monarch Initiative: MONDO:0016739
Orphanet: ORPHA252006

Definition

A malignant germ cell tumor of the central nervous system composed of primitive-appearing epithelial cells - putatively representing yolk sac endoderm - set in a loose, variably cellular, and often conspicuously myxoid matrix, resembling extra-embryonic mesoblast. Eosinophilic hyaline globules immunoreactive for AFP are a diagnostic feature. (WHO) [from NCI]

Professional guidelines

PubMed

Regulation of Microglia for the Treatment of Glioma.
Zhou S, Cisse B
World Neurosurg 2021 Oct;154:222-227. doi: 10.1016/j.wneu.2021.06.107. PMID: 34583499
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

Recent clinical studies

Etiology

Second-look surgery for pineal region tumors.
Ogiwara H
Childs Nerv Syst 2023 Sep;39(9):2349-2352. Epub 2022 Oct 1 doi: 10.1007/s00381-022-05676-4. PMID: 36181520
Clinical characteristics and prognostic factors of male yolk sac tumor: a Surveillance, Epidemiology, and End Results program study.
Geng R, Zheng Z, Lin Y, Li Y, Ge G, Zhang J, Wang C, Dai X, Ye Q, Zhen J, Zhang Y, Fu C, Li Y
World J Urol 2021 Apr;39(4):1211-1217. Epub 2020 Jun 19 doi: 10.1007/s00345-020-03311-y. PMID: 32562046
Central Nervous System-type Neuroepithelial Tumors and Tumor-like Proliferations Developing in the Gynecologic Tract and Pelvis: Clinicopathologic Analysis of 23 Cases.
Murdock T, Orr B, Allen S, Ibrahim J, Sharma R, Ronnett BM, Rodriguez FJ
Am J Surg Pathol 2018 Nov;42(11):1429-1444. doi: 10.1097/PAS.0000000000001131. PMID: 30074494
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
Pineal tumors.
Linggood RM, Chapman PH
J Neurooncol 1992 Jan;12(1):85-91. doi: 10.1007/BF00172460. PMID: 1541982

Diagnosis

Second-look surgery for pineal region tumors.
Ogiwara H
Childs Nerv Syst 2023 Sep;39(9):2349-2352. Epub 2022 Oct 1 doi: 10.1007/s00381-022-05676-4. PMID: 36181520
Multidisciplinary treatment of primary intracranial yolk sac tumor: A case report and literature review.
Xu ZN, Yue XY, Cao XC, Liu YD, Fang BS, Zhao WH, Li C, Xu S, Zhang M
Medicine (Baltimore) 2021 May 14;100(19):e25778. doi: 10.1097/MD.0000000000025778. PMID: 34106610Free PMC Article
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 tumors.
Linggood RM, Chapman PH
J Neurooncol 1992 Jan;12(1):85-91. doi: 10.1007/BF00172460. PMID: 1541982

Therapy

Cystic Trophoblastic Tumor in a Primary Central Nervous System Post-Chemotherapy Germ Cell Tumor: The First Case Report.
Gadde R, Arora K, Felicella MM, Arora S, Cheng L, Park H, Gupta NS, Salamat MS, Williamson SR
Int J Surg Pathol 2020 Dec;28(8):925-928. Epub 2020 Jun 4 doi: 10.1177/1066896920929751. PMID: 32498578
Successful treatment of mixed yolk sac tumor and mature teratoma in the spinal cord: case report.
Mukasa A, Yanagisawa S, Saito K, Tanaka S, Takai K, Shibahara J, Ikegami M, Nakao Y, Takeshita K, Matsutani M, Saito N
J Neurosurg Spine 2017 Mar;26(3):319-324. Epub 2016 Dec 2 doi: 10.3171/2016.8.SPINE16465. PMID: 27911225
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
Yolk sac tumor of the bilateral basal ganglia in a patient with Down syndrome.
Sugimoto K, Ideguchi M, Sadahiro H, Yoshikawa K, Goto H, Nomura S, Fujii M, Suzuki M
Brain Tumor Pathol 2013 Oct;30(4):247-52. Epub 2013 Jan 11 doi: 10.1007/s10014-012-0134-9. PMID: 23306965
Intracranial yolk sac tumor in a patient with Down syndrome.
Maeda Y, Yoshikawa K, Kajiwara K, Ideguchi M, Amano T, Saka M, Nomura S, Fujii M, Suzuki M
J Neurosurg Pediatr 2011 Jun;7(6):604-8. doi: 10.3171/2011.3.PEDS10500. PMID: 21631196

Prognosis

Second-look surgery for pineal region tumors.
Ogiwara H
Childs Nerv Syst 2023 Sep;39(9):2349-2352. Epub 2022 Oct 1 doi: 10.1007/s00381-022-05676-4. PMID: 36181520
12p gain is predominantly observed in non-germinomatous germ cell tumors and identifies an unfavorable subgroup of central nervous system germ cell tumors.
Satomi K, Takami H, Fukushima S, Yamashita S, Matsushita Y, Nakazato Y, Suzuki T, Tanaka S, Mukasa A, Saito N, Kanamori M, Kumabe T, Tominaga T, Kobayashi K, Nagane M, Iuchi T, Yoshimoto K, Tamura K, Maehara T, Sakai K, Sugiyama K, Yokogami K, Takeshima H, Nonaka M, Asai A, Ushijima T, Matsutani M, Nishikawa R, Ichimura K
Neuro Oncol 2022 May 4;24(5):834-846. doi: 10.1093/neuonc/noab246. PMID: 34698864Free PMC Article
Multidisciplinary treatment of primary intracranial yolk sac tumor: A case report and literature review.
Xu ZN, Yue XY, Cao XC, Liu YD, Fang BS, Zhao WH, Li C, Xu S, Zhang M
Medicine (Baltimore) 2021 May 14;100(19):e25778. doi: 10.1097/MD.0000000000025778. PMID: 34106610Free PMC Article
Clinical characteristics and prognostic factors of male yolk sac tumor: a Surveillance, Epidemiology, and End Results program study.
Geng R, Zheng Z, Lin Y, Li Y, Ge G, Zhang J, Wang C, Dai X, Ye Q, Zhen J, Zhang Y, Fu C, Li Y
World J Urol 2021 Apr;39(4):1211-1217. Epub 2020 Jun 19 doi: 10.1007/s00345-020-03311-y. PMID: 32562046
Pineal tumors.
Linggood RM, Chapman PH
J Neurooncol 1992 Jan;12(1):85-91. doi: 10.1007/BF00172460. PMID: 1541982

Clinical prediction guides

Second-look surgery for pineal region tumors.
Ogiwara H
Childs Nerv Syst 2023 Sep;39(9):2349-2352. Epub 2022 Oct 1 doi: 10.1007/s00381-022-05676-4. PMID: 36181520
Loss of PIGK function causes severe infantile encephalopathy and extensive neuronal apoptosis.
Chen X, Yin W, Chen S, Zhang W, Li H, Kuang H, Zhou M, Teng Y, Zhang J, Shen G, Liang D, Li Z, Hu B, Wu L
Hum Genet 2021 May;140(5):791-803. Epub 2021 Jan 4 doi: 10.1007/s00439-020-02243-2. PMID: 33392778
Yolk sac tumor of the fourth ventricle in a 3-year-old boy: imaging features.
Ji Q, Ding C, Liu C, Li S
Childs Nerv Syst 2020 Dec;36(12):3123-3128. Epub 2020 Apr 6 doi: 10.1007/s00381-020-04600-y. PMID: 32253490
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
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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