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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

The optimal management of brain metastases from gestational trophoblastic neoplasia.
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
Current management of gestational trophoblastic neoplasia.
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
The management of recurrent and drug-resistant gestational trophoblastic neoplasia (GTN).
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

Ultra High-risk Gestational Trophoblastic Neoplasia.
Elias KM, Berkowitz RS, Horowitz NS
Hematol Oncol Clin North Am 2024 Dec;38(6):1259-1264. Epub 2024 Sep 27 doi: 10.1016/j.hoc.2024.08.015. PMID: 39341707
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
The optimal management of brain metastases from gestational trophoblastic neoplasia.
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
Organ transplantation from donors (cadaveric or living) with a history of malignancy: review of the literature.
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
The management of recurrent and drug-resistant gestational trophoblastic neoplasia (GTN).
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

Ultra High-risk Gestational Trophoblastic Neoplasia.
Elias KM, Berkowitz RS, Horowitz NS
Hematol Oncol Clin North Am 2024 Dec;38(6):1259-1264. Epub 2024 Sep 27 doi: 10.1016/j.hoc.2024.08.015. PMID: 39341707
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
The optimal management of brain metastases from gestational trophoblastic neoplasia.
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
Unusual clinical presentations of choriocarcinoma: A systematic review of case reports.
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
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

Therapy

Ultra High-risk Gestational Trophoblastic Neoplasia.
Elias KM, Berkowitz RS, Horowitz NS
Hematol Oncol Clin North Am 2024 Dec;38(6):1259-1264. Epub 2024 Sep 27 doi: 10.1016/j.hoc.2024.08.015. PMID: 39341707
The optimal management of brain metastases from gestational trophoblastic neoplasia.
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
Organ transplantation from donors (cadaveric or living) with a history of malignancy: review of the literature.
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
The management of recurrent and drug-resistant gestational trophoblastic neoplasia (GTN).
Newlands ES
Best Pract Res Clin Obstet Gynaecol 2003 Dec;17(6):905-23. doi: 10.1016/s1521-6934(03)00092-0. PMID: 14614889
Radiation therapy in the management of gestational choriocarcinoma metastatic to the central nervous system.
Yordan EL Jr, Schlaerth J, Gaddis O, Morrow CP
Obstet Gynecol 1987 Apr;69(4):627-30. PMID: 3822306

Prognosis

Ultra High-risk Gestational Trophoblastic Neoplasia.
Elias KM, Berkowitz RS, Horowitz NS
Hematol Oncol Clin North Am 2024 Dec;38(6):1259-1264. Epub 2024 Sep 27 doi: 10.1016/j.hoc.2024.08.015. PMID: 39341707
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
PD-L1 and PD-1 expression in pediatric central nervous system germ cell tumors.
Woods JK, Lidov HG, Ligon KL, Santagata S, Chi SN, Yeo KK, Alexandrescu S
Mod Pathol 2022 Dec;35(12):1770-1774. Epub 2022 Sep 3 doi: 10.1038/s41379-022-01142-3. PMID: 36057740
Unusual clinical presentations of choriocarcinoma: A systematic review of case reports.
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
The management of recurrent and drug-resistant gestational trophoblastic neoplasia (GTN).
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

Ultra High-risk Gestational Trophoblastic Neoplasia.
Elias KM, Berkowitz RS, Horowitz NS
Hematol Oncol Clin North Am 2024 Dec;38(6):1259-1264. Epub 2024 Sep 27 doi: 10.1016/j.hoc.2024.08.015. PMID: 39341707
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
Primary central nervous system lymphomas with massive intratumoral hemorrhage: Clinical, radiological, pathological, and molecular features of six cases.
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
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

Recent systematic reviews

Discovered cancers at postmortem donor examination: A starting point for quality improvement of donor assessment.
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
Unusual clinical presentations of choriocarcinoma: A systematic review of case reports.
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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