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Choriocarcinoma of the central nervous system

MedGen UID:
234100
Concept ID:
C1332876
Neoplastic Process
Synonyms: Central Nervous System Choriocarcinoma; central nervous system choriocarcinoma; central nervous system choriocarcinoma (disease); Choriocarcinoma of CNS; choriocarcinoma of CNS; choriocarcinoma of the central nervous system; Choriocarcinoma of the CNS; choriocarcinoma of the CNS
 
Monarch Initiative: MONDO:0016740
Orphanet: ORPHA252015

Definition

A rare primary germ cell tumor of central nervous system characterized by a lesion typically in the region of the pineal gland and the suprasellar compartment, composed of cytotrophoblastic elements and multinucleated syncytiotrophoblastic giant cells. Ectatic stromal vascular channels, blood lakes, and extensive hemorrhagic necrosis are the rule. The tumor usually arises in the second decade of life and predominantly in males. Clinical presentation depends on location and size and includes signs of increased intracranial pressure, visual disturbances, and endocrine abnormalities. Prognosis is generally poor. [from ORDO]

Professional guidelines

PubMed

Tsai J, Vellayappan B, Venur V, McGranahan T, Gray H, Urban RR, Tseng YD, Palmer J, Foote M, Mayr NA, Combs SE, Sahgal A, Chang EL, Lo SS
Expert Rev Anticancer Ther 2022 Mar;22(3):307-315. Epub 2022 Feb 13 doi: 10.1080/14737140.2022.2038566. PMID: 35114862
Goldstein DP, Berkowitz RS
Hematol Oncol Clin North Am 2012 Feb;26(1):111-31. Epub 2011 Nov 21 doi: 10.1016/j.hoc.2011.10.007. PMID: 22244665
Newlands ES
Best Pract Res Clin Obstet Gynaecol 2003 Dec;17(6):905-23. doi: 10.1016/s1521-6934(03)00092-0. PMID: 14614889

Recent clinical studies

Etiology

Ogiwara H
Childs Nerv Syst 2023 Sep;39(9):2349-2352. Epub 2022 Oct 1 doi: 10.1007/s00381-022-05676-4. PMID: 36181520
Tsai J, Vellayappan B, Venur V, McGranahan T, Gray H, Urban RR, Tseng YD, Palmer J, Foote M, Mayr NA, Combs SE, Sahgal A, Chang EL, Lo SS
Expert Rev Anticancer Ther 2022 Mar;22(3):307-315. Epub 2022 Feb 13 doi: 10.1080/14737140.2022.2038566. PMID: 35114862
Zhang S, Yuan J, Li W, Ye Q
Transplant Rev (Orlando) 2014 Oct;28(4):169-75. Epub 2014 Jul 23 doi: 10.1016/j.trre.2014.06.002. PMID: 25135838
Goldstein DP, Berkowitz RS
Hematol Oncol Clin North Am 2012 Feb;26(1):111-31. Epub 2011 Nov 21 doi: 10.1016/j.hoc.2011.10.007. PMID: 22244665
Newlands ES
Best Pract Res Clin Obstet Gynaecol 2003 Dec;17(6):905-23. doi: 10.1016/s1521-6934(03)00092-0. PMID: 14614889

Diagnosis

Ogiwara H
Childs Nerv Syst 2023 Sep;39(9):2349-2352. Epub 2022 Oct 1 doi: 10.1007/s00381-022-05676-4. PMID: 36181520
Tsai J, Vellayappan B, Venur V, McGranahan T, Gray H, Urban RR, Tseng YD, Palmer J, Foote M, Mayr NA, Combs SE, Sahgal A, Chang EL, Lo SS
Expert Rev Anticancer Ther 2022 Mar;22(3):307-315. Epub 2022 Feb 13 doi: 10.1080/14737140.2022.2038566. PMID: 35114862
Mangla M, Singla D, Kaur H, Sharma S
Taiwan J Obstet Gynecol 2017 Feb;56(1):1-8. doi: 10.1016/j.tjog.2015.05.011. PMID: 28254207
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
Fujimaki T
J Child Neurol 2009 Nov;24(11):1439-45. doi: 10.1177/0883073809342127. PMID: 19841431

Therapy

Tsai J, Vellayappan B, Venur V, McGranahan T, Gray H, Urban RR, Tseng YD, Palmer J, Foote M, Mayr NA, Combs SE, Sahgal A, Chang EL, Lo SS
Expert Rev Anticancer Ther 2022 Mar;22(3):307-315. Epub 2022 Feb 13 doi: 10.1080/14737140.2022.2038566. PMID: 35114862
Spiess D, Winker M, Chauveau A, Abegg VF, Potterat O, Hamburger M, Gründemann C, Simões-Wüst AP
Planta Med 2022 Oct;88(12):1036-1046. Epub 2021 Oct 8 doi: 10.1055/a-1628-8132. PMID: 34624906Free PMC Article
Zhang S, Yuan J, Li W, Ye Q
Transplant Rev (Orlando) 2014 Oct;28(4):169-75. Epub 2014 Jul 23 doi: 10.1016/j.trre.2014.06.002. PMID: 25135838
Newlands ES
Best Pract Res Clin Obstet Gynaecol 2003 Dec;17(6):905-23. doi: 10.1016/s1521-6934(03)00092-0. PMID: 14614889
Yordan EL Jr, Schlaerth J, Gaddis O, Morrow CP
Obstet Gynecol 1987 Apr;69(4):627-30. PMID: 3822306

Prognosis

Ogiwara H
Childs Nerv Syst 2023 Sep;39(9):2349-2352. Epub 2022 Oct 1 doi: 10.1007/s00381-022-05676-4. PMID: 36181520
Mangla M, Singla D, Kaur H, Sharma S
Taiwan J Obstet Gynecol 2017 Feb;56(1):1-8. doi: 10.1016/j.tjog.2015.05.011. PMID: 28254207
Gao Y, Jiang J, Liu Q
Int J Clin Exp Pathol 2014;7(10):6965-72. Epub 2014 Sep 15 PMID: 25400782Free PMC Article
Goldstein DP, Berkowitz RS
Hematol Oncol Clin North Am 2012 Feb;26(1):111-31. Epub 2011 Nov 21 doi: 10.1016/j.hoc.2011.10.007. PMID: 22244665
Newlands ES
Best Pract Res Clin Obstet Gynaecol 2003 Dec;17(6):905-23. doi: 10.1016/s1521-6934(03)00092-0. PMID: 14614889

Clinical prediction guides

Ogiwara H
Childs Nerv Syst 2023 Sep;39(9):2349-2352. Epub 2022 Oct 1 doi: 10.1007/s00381-022-05676-4. PMID: 36181520
Yamada S, Muto J, Iba S, Shiogama K, Tsuyuki Y, Satou A, Ohba S, Murayama K, Sugita Y, Nakamura S, Yokoo H, Tomita A, Hirose Y, Tsukamoto T, Abe M
Neuropathology 2021 Oct;41(5):335-348. Epub 2021 Jul 13 doi: 10.1111/neup.12739. PMID: 34254378
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
Shi H, Cao D, Wei L, Sun L, Guo A
Virchows Arch 2010 Jan;456(1):65-70. Epub 2009 Dec 16 doi: 10.1007/s00428-009-0864-1. PMID: 20013345
Hirato J, Nakazato Y
J Neurooncol 2001 Sep;54(3):239-49. doi: 10.1023/a:1012721723387. PMID: 11767290

Recent systematic reviews

Girolami I, Neil D, Segev DL, Furian L, Zaza G, Boggi U, Gambaro G, De Feo T, Casartelli-Liviero M, Cardillo M, Lombardini L, Zampicinini L, D'Errico A, Eccher A
Transplant Rev (Orlando) 2021 Apr;35(2):100608. Epub 2021 Feb 20 doi: 10.1016/j.trre.2021.100608. PMID: 33647551
Mangla M, Singla D, Kaur H, Sharma S
Taiwan J Obstet Gynecol 2017 Feb;56(1):1-8. doi: 10.1016/j.tjog.2015.05.011. PMID: 28254207

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