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Choroid plexus carcinoma(CPC)

MedGen UID:
96557
Concept ID:
C0431109
Neoplastic Process
Synonyms: CPC
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Sources: HPO, OMIM, Orphanet
Autosomal dominant inheritance refers to genetic conditions that occur when a mutation is present in one copy of a given gene (i.e., the person is heterozygous).
Autosomal dominant inheritance (HPO, OMIM, Orphanet)
SNOMED CT: Choroid plexus papilloma, anaplastic (88252006); Choroid plexus carcinoma (88252006)
 
OMIM®: 191170; 260500
HPO: HP:0030392
Orphanet: ORPHA251899

Definition

Intraventricular papillary neoplasm derived from choroid plexus epithelium. Plexus tumors are most common in the lateral and fourth ventricles; while 80% of lateral ventricle tumors present in children, fourth ventricle tumors are evenly distributed in all age groups. Clinically, choroid plexus tumors tend to cause hydrocephalus and increased intracranial pressure. Histologically, choroid plexus papillomas correspond to WHO grade I, choroid plexus carcinomas to WHO grade III. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChoroid plexus carcinoma
Follow this link to review classifications for Choroid plexus carcinoma in Orphanet.

Conditions with this feature

Rhabdoid tumor predisposition syndrome 1
MedGen UID:
322892
Concept ID:
C1836327
Neoplastic Process
The rhabdoid tumor predisposition syndrome is an autosomal dominant cancer syndrome predisposing to renal or extrarenal malignant rhabdoid tumors and to a variety of tumors of the central nervous system, including choroid plexus carcinoma, medulloblastoma, and central primitive neuroectodermal tumors (Sevenet et al., 1999). Rhabdoid tumors are a highly malignant group of neoplasms that usually occur in children less than 2 years of age. Malignant rhabdoid tumors (MRTs) of the kidney were first described as a sarcomatous variant of Wilms tumors (Beckwith and Palmer, 1978). Later, extrarenal rhabdoid tumor was reported in numerous locations, including the central nervous system (CNS) (Parham et al., 1994). Classification has been difficult because of considerable variation in the histologic and immunologic characteristics within and between rhabdoid tumors of the liver, soft tissues, and CNS. In the CNS, rhabdoid tumors may be pure rhabdoid tumors or a variant that has been designated atypical teratoid tumor (AT/RT).

Professional guidelines

PubMed

Lu KH, Wood ME, Daniels M, Burke C, Ford J, Kauff ND, Kohlmann W, Lindor NM, Mulvey TM, Robinson L, Rubinstein WS, Stoffel EM, Snyder C, Syngal S, Merrill JK, Wollins DS, Hughes KS; American Society of Clinical Oncology.
J Clin Oncol 2014 Mar 10;32(8):833-40. Epub 2014 Feb 3 doi: 10.1200/JCO.2013.50.9257. PMID: 24493721Free PMC Article

Recent clinical studies

Etiology

Ozdogan S, Gergin YE, Gergin S, Senol O, Tiryaki M, Tatarli N, Hicdonmez T
Pan Afr Med J 2015;20:302. Epub 2015 Mar 30 doi: 10.11604/pamj.2015.20.302.5854. PMID: 26161225Free PMC Article
Tong Y, Merino D, Nimmervoll B, Gupta K, Wang YD, Finkelstein D, Dalton J, Ellison DW, Ma X, Zhang J, Malkin D, Gilbertson RJ
Cancer Cell 2015 May 11;27(5):712-27. doi: 10.1016/j.ccell.2015.04.005. PMID: 25965574Free PMC Article
Zaky W, Dhall G, Khatua S, Brown RJ, Ginn KF, Gardner SL, Yildiz VO, Yankelevich M, Finlay JL
Pediatr Blood Cancer 2015 May;62(5):784-9. Epub 2015 Feb 8 doi: 10.1002/pbc.25436. PMID: 25662896
Sun MZ, Ivan ME, Oh MC, Delance AR, Clark AJ, Safaee M, Oh T, Kaur G, Molinaro A, Gupta N, Parsa AT
J Neurooncol 2014 Nov;120(2):353-60. Epub 2014 Aug 9 doi: 10.1007/s11060-014-1559-2. PMID: 25106865
Sun MZ, Ivan ME, Clark AJ, Oh MC, Delance AR, Oh T, Safaee M, Kaur G, Bloch O, Molinaro A, Gupta N, Parsa AT
J Neurooncol 2014 Jan;116(1):179-85. Epub 2013 Oct 29 doi: 10.1007/s11060-013-1281-5. PMID: 24166581

Diagnosis

Ozdogan S, Gergin YE, Gergin S, Senol O, Tiryaki M, Tatarli N, Hicdonmez T
Pan Afr Med J 2015;20:302. Epub 2015 Mar 30 doi: 10.11604/pamj.2015.20.302.5854. PMID: 26161225Free PMC Article
Zaky W, Dhall G, Khatua S, Brown RJ, Ginn KF, Gardner SL, Yildiz VO, Yankelevich M, Finlay JL
Pediatr Blood Cancer 2015 May;62(5):784-9. Epub 2015 Feb 8 doi: 10.1002/pbc.25436. PMID: 25662896
Joó GJ, Reiniger L, Papp C, Csaba A, Komáromy H, Rigó J Jr
Pathol Res Pract 2014 Dec;210(12):1156-9. Epub 2014 Feb 12 doi: 10.1016/j.prp.2014.01.009. PMID: 25034609
Yoshida K, Sato K, Kitai R, Hashimoto N, Kubota T, Kikuta K
Brain Tumor Pathol 2013 Apr;30(2):104-8. Epub 2012 Jul 1 doi: 10.1007/s10014-012-0112-2. PMID: 22752623Free PMC Article
Wilhelm M, Hirsch W, Merkenschlager A, Stepan H, Geyer C, Kiess W
Pediatr Hematol Oncol 2012 Oct;29(7):643-6. Epub 2012 Aug 10 doi: 10.3109/08880018.2012.710298. PMID: 22881717

Therapy

Vesel AS, Novak-Jankovič V, Maldini B, Lukanović NP, Denić K, Markovič-Božić J, Šoštarič M
Acta Clin Croat 2015 Sep;54(3):381-7. PMID: 26666113
Zaky W, Dhall G, Khatua S, Brown RJ, Ginn KF, Gardner SL, Yildiz VO, Yankelevich M, Finlay JL
Pediatr Blood Cancer 2015 May;62(5):784-9. Epub 2015 Feb 8 doi: 10.1002/pbc.25436. PMID: 25662896
Sun MZ, Ivan ME, Oh MC, Delance AR, Clark AJ, Safaee M, Oh T, Kaur G, Molinaro A, Gupta N, Parsa AT
J Neurooncol 2014 Nov;120(2):353-60. Epub 2014 Aug 9 doi: 10.1007/s11060-014-1559-2. PMID: 25106865
Samuel TA, Parikh J, Sharma S, Giller CA, Sterling K, Kapoor S, Pirkle C, Jillella A
J Neurol Surg A Cent Eur Neurosurg 2013 Dec;74 Suppl 1:e149-54. Epub 2013 Feb 20 doi: 10.1055/s-0032-1333419. PMID: 23427033
Mosleh O, Tabori U, Bartels U, Huang A, Schechter T, Bouffet E
Pediatr Hematol Oncol 2013 Aug;30(5):386-91. Epub 2013 Jan 9 doi: 10.3109/08880018.2012.756089. PMID: 23301664

Prognosis

Zaky W, Dhall G, Khatua S, Brown RJ, Ginn KF, Gardner SL, Yildiz VO, Yankelevich M, Finlay JL
Pediatr Blood Cancer 2015 May;62(5):784-9. Epub 2015 Feb 8 doi: 10.1002/pbc.25436. PMID: 25662896
Sun MZ, Ivan ME, Oh MC, Delance AR, Clark AJ, Safaee M, Oh T, Kaur G, Molinaro A, Gupta N, Parsa AT
J Neurooncol 2014 Nov;120(2):353-60. Epub 2014 Aug 9 doi: 10.1007/s11060-014-1559-2. PMID: 25106865
Sun MZ, Ivan ME, Clark AJ, Oh MC, Delance AR, Oh T, Safaee M, Kaur G, Bloch O, Molinaro A, Gupta N, Parsa AT
J Neurooncol 2014 Jan;116(1):179-85. Epub 2013 Oct 29 doi: 10.1007/s11060-013-1281-5. PMID: 24166581
Yoshida K, Sato K, Kitai R, Hashimoto N, Kubota T, Kikuta K
Brain Tumor Pathol 2013 Apr;30(2):104-8. Epub 2012 Jul 1 doi: 10.1007/s10014-012-0112-2. PMID: 22752623Free PMC Article
Wilhelm M, Hirsch W, Merkenschlager A, Stepan H, Geyer C, Kiess W
Pediatr Hematol Oncol 2012 Oct;29(7):643-6. Epub 2012 Aug 10 doi: 10.3109/08880018.2012.710298. PMID: 22881717

Clinical prediction guides

Vesel AS, Novak-Jankovič V, Maldini B, Lukanović NP, Denić K, Markovič-Božić J, Šoštarič M
Acta Clin Croat 2015 Sep;54(3):381-7. PMID: 26666113
Custodio G, Taques GR, Figueiredo BC, Gugelmin ES, Oliveira Figueiredo MM, Watanabe F, Pontarolo R, Lalli E, Torres LF
PLoS One 2011 Mar 22;6(3):e18015. doi: 10.1371/journal.pone.0018015. PMID: 21445348Free PMC Article
Lafay-Cousin L, Mabbott DJ, Halliday W, Taylor MD, Tabori U, Kamaly-Asl ID, Kulkarni AV, Bartels U, Greenberg M, Bouffet E
J Neurosurg Pediatr 2010 Jun;5(6):615-21. doi: 10.3171/2010.3.PEDS09354. PMID: 20515336
Russell-Swetek A, West AN, Mintern JE, Jenkins J, Rodriguez-Galindo C, Ribeiro R, Zambetti GP
J Med Genet 2008 Sep;45(9):603-6. doi: 10.1136/jmg.2008.059568. PMID: 18762572Free PMC Article
Fitzpatrick LK, Aronson LJ, Cohen KJ
J Neurooncol 2002 Apr;57(2):123-6. PMID: 12125972

Recent systematic reviews

Sun MZ, Ivan ME, Oh MC, Delance AR, Clark AJ, Safaee M, Oh T, Kaur G, Molinaro A, Gupta N, Parsa AT
J Neurooncol 2014 Nov;120(2):353-60. Epub 2014 Aug 9 doi: 10.1007/s11060-014-1559-2. PMID: 25106865
Sun MZ, Ivan ME, Clark AJ, Oh MC, Delance AR, Oh T, Safaee M, Kaur G, Bloch O, Molinaro A, Gupta N, Parsa AT
J Neurooncol 2014 Jan;116(1):179-85. Epub 2013 Oct 29 doi: 10.1007/s11060-013-1281-5. PMID: 24166581
Wrede B, Liu P, Wolff JE
J Neurooncol 2007 Dec;85(3):345-51. Epub 2007 Jun 19 doi: 10.1007/s11060-007-9428-x. PMID: 17576522
Wrede B, Liu P, Ater J, Wolff JE
Anticancer Res 2005 Nov-Dec;25(6C):4429-33. PMID: 16334121
Wolff JE, Sajedi M, Brant R, Coppes MJ, Egeler RM
Br J Cancer 2002 Nov 4;87(10):1086-91. doi: 10.1038/sj.bjc.6600609. PMID: 12402146Free PMC Article

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