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Choroid plexus papilloma(CPP)

MedGen UID:
64439
Concept ID:
C0205770
Neoplastic Process
Synonyms: CPP; Papilloma of choroid plexus
SNOMED CT: Benign choroid plexus papilloma (1156641000)
 
Gene (location): TP53 (17p13.1)
 
HPO: HP:0200022
Monarch Initiative: MONDO:0009837
OMIM®: 260500
Orphanet: ORPHA2807

Definition

Choroid plexus tumors are of neuroectodermal origin and range from benign choroid plexus papillomas (CPPs) to malignant choroid carcinomas (CPCs). These rare tumors generally occur in childhood, but have also been reported in adults. Patients typically present with signs and symptoms of increased intracranial pressure including headache, hydrocephalus, papilledema, nausea, vomiting, cranial nerve deficits, gait impairment, and seizures (summary by Safaee et al., 2013). [from OMIM]

Clinical features

From HPO
Osteosarcoma
MedGen UID:
10501
Concept ID:
C0029463
Neoplastic Process
A malignant bone tumor that usually develops during adolescence and usually affects the long bones including the tibia, femur, and humerus. The typical symptoms of osteosarcoma comprise bone pain, fracture, limitation of motion, and tenderness or swelling at the site of the tumor.
Nausea
MedGen UID:
10196
Concept ID:
C0027497
Sign or Symptom
A sensation of unease in the stomach together with an urge to vomit.
Vomiting
MedGen UID:
12124
Concept ID:
C0042963
Sign or Symptom
Forceful ejection of the contents of the stomach through the mouth by means of a series of involuntary spasmic contractions.
Headache
MedGen UID:
9149
Concept ID:
C0018681
Sign or Symptom
Cephalgia, or pain sensed in various parts of the head, not confined to the area of distribution of any nerve.
Hydrocephalus
MedGen UID:
9335
Concept ID:
C0020255
Disease or Syndrome
Hydrocephalus is an active distension of the ventricular system of the brain resulting from inadequate passage of CSF from its point of production within the cerebral ventricles to its point of absorption into the systemic circulation.
Seizure
MedGen UID:
20693
Concept ID:
C0036572
Sign or Symptom
A seizure is an intermittent abnormality of nervous system physiology characterised by a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.
Choroid plexus papilloma
MedGen UID:
64439
Concept ID:
C0205770
Neoplastic Process
Choroid plexus tumors are of neuroectodermal origin and range from benign choroid plexus papillomas (CPPs) to malignant choroid carcinomas (CPCs). These rare tumors generally occur in childhood, but have also been reported in adults. Patients typically present with signs and symptoms of increased intracranial pressure including headache, hydrocephalus, papilledema, nausea, vomiting, cranial nerve deficits, gait impairment, and seizures (summary by Safaee et al., 2013).
Papilledema
MedGen UID:
10565
Concept ID:
C0030353
Finding
Papilledema refers to edema (swelling) of the optic disc secondary to any factor which increases cerebral spinal fluid pressure.

Term Hierarchy

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

Conditions with this feature

Choroid plexus papilloma
MedGen UID:
64439
Concept ID:
C0205770
Neoplastic Process
Choroid plexus tumors are of neuroectodermal origin and range from benign choroid plexus papillomas (CPPs) to malignant choroid carcinomas (CPCs). These rare tumors generally occur in childhood, but have also been reported in adults. Patients typically present with signs and symptoms of increased intracranial pressure including headache, hydrocephalus, papilledema, nausea, vomiting, cranial nerve deficits, gait impairment, and seizures (summary by Safaee et al., 2013).
Neurocutaneous melanocytosis
MedGen UID:
154259
Concept ID:
C0544862
Congenital Abnormality
Neurocutaneous melanosis, or neuromelanosis, is characterized by the presence of melanin-producing cells within the brain parenchyma or leptomeninges, which may lead to clinically apparent neurologic signs and symptoms, such as seizures. Other neurologic abnormalities, including hydrocephalus, arachnoid cysts, tumors, and syringomyelia, may also occur. The disorder is a rare but severe manifestation of congenital melanocytic nevus syndrome (CMNS; 137550). Some patients with neurocutaneous melanosis or CMNS may develop malignant melanoma. The incidence of neurologic involvement, development of malignant melanoma, and death is significantly associated with the projected adult size of the largest congenital melanocytic nevus, particularly those greater than 40 cm (summary by Kinsler et al., 2008; Kinsler et al., 2013).

Professional guidelines

PubMed

Chong AW, McAdory LE, Low DCY, Lim EJ, Leong NWL, Ho CL
Clin Imaging 2022 Feb;82:38-52. Epub 2021 Oct 29 doi: 10.1016/j.clinimag.2021.10.008. PMID: 34773811
Thomas C, Metrock K, Kordes U, Hasselblatt M, Dhall G
J Neurooncol 2020 May;148(1):39-45. Epub 2020 Apr 28 doi: 10.1007/s11060-020-03509-5. PMID: 32342334Free PMC Article
Hosmann A, Hinker F, Dorfer C, Slavc I, Haberler C, Dieckmann K, Knosp E, Czech T
Acta Neurochir (Wien) 2019 Apr;161(4):745-754. Epub 2019 Feb 19 doi: 10.1007/s00701-019-03832-5. PMID: 30783805Free PMC Article

Recent clinical studies

Etiology

Chen Y, Zhao R, Shi W, Li H
Clin Neurol Neurosurg 2021 Jan;200:106345. Epub 2020 Nov 4 doi: 10.1016/j.clineuro.2020.106345. PMID: 33203591
Thomas C, Metrock K, Kordes U, Hasselblatt M, Dhall G
J Neurooncol 2020 May;148(1):39-45. Epub 2020 Apr 28 doi: 10.1007/s11060-020-03509-5. PMID: 32342334Free PMC Article
Crawford JR, Isaacs H Jr
Childs Nerv Syst 2019 Jun;35(6):937-944. Epub 2019 Apr 5 doi: 10.1007/s00381-019-04135-x. PMID: 30953158
Shi YZ, Chen MZ, Huang W, Guo LL, Chen X, Kong D, Zhuang YY, Xu YM, Zhang RR, Bo GJ, Wang ZQ
Acta Radiol 2017 Aug;58(8):983-990. Epub 2017 Mar 30 doi: 10.1177/0284185116676651. PMID: 28358248
Li P, Diao X, Bi Z, Hao S, Ren X, Zhang J, Xing J
J Clin Neurosci 2015 Nov;22(11):1776-84. Epub 2015 Aug 29 doi: 10.1016/j.jocn.2015.05.025. PMID: 26455422

Diagnosis

Chen Y, Zhao R, Shi W, Li H
Clin Neurol Neurosurg 2021 Jan;200:106345. Epub 2020 Nov 4 doi: 10.1016/j.clineuro.2020.106345. PMID: 33203591
Thomas C, Metrock K, Kordes U, Hasselblatt M, Dhall G
J Neurooncol 2020 May;148(1):39-45. Epub 2020 Apr 28 doi: 10.1007/s11060-020-03509-5. PMID: 32342334Free PMC Article
Muly S, Liu S, Lee R, Nicolaou S, Rojas R, Khosa F
Clin Imaging 2018 Nov-Dec;52:226-239. Epub 2018 Aug 1 doi: 10.1016/j.clinimag.2018.07.021. PMID: 30138862
Keddie S, Jaunmuktane Z, Brandner S, Shah S, Maddison P, Rees JH, Hanna MG, Lunn MPT, Reilly MM, Rossor AM, Carr AS
Pract Neurol 2018 Apr;18(2):137-142. Epub 2018 Jan 23 doi: 10.1136/practneurol-2017-001801. PMID: 29363556Free PMC Article
Wolburg H, Paulus W
Acta Neuropathol 2010 Jan;119(1):75-88. Epub 2009 Dec 24 doi: 10.1007/s00401-009-0627-8. PMID: 20033190

Therapy

Wolff JE, Van Gool SW, Kutluk T, Diez B, Kebudi R, Timmermann B, Garami M, Sterba J, Fuller GN, Bison B, Kordes UR
J Neurooncol 2022 Feb;156(3):599-613. Epub 2022 Jan 8 doi: 10.1007/s11060-021-03942-0. PMID: 34997889Free PMC Article
Aydin Ozturk P, Sanri O, Yilmaz A, Arpa A, Ozturk U, Ceviz A
Pediatr Neurosurg 2018;53(6):413-415. Epub 2018 Sep 3 doi: 10.1159/000492334. PMID: 30176663
Mayl JJ, Flores MA, Stelzer JW, Liu B, Messina SA, Murray JV
Emerg Radiol 2018 Apr;25(2):215-218. Epub 2018 Feb 3 doi: 10.1007/s10140-018-1582-2. PMID: 29397464
Wrede B, Hasselblatt M, Peters O, Thall PF, Kutluk T, Moghrabi A, Mahajan A, Rutkowski S, Diez B, Wang X, Pietsch T, Kortmann RD, Paulus W, Jeibmann A, Wolff JEA
J Neurooncol 2009 Dec;95(3):383-392. Epub 2009 Jun 20 doi: 10.1007/s11060-009-9936-y. PMID: 19543851Free PMC Article
McCall T, Binning M, Blumenthal DT, Jensen RL
Surg Neurol 2006 Jul;66(1):62-7; discussion 67-8. doi: 10.1016/j.surneu.2005.09.023. PMID: 16793445

Prognosis

Horinouchi S, Watanabe T, Takeshima H
World Neurosurg 2022 Jun;162:74-76. Epub 2022 Mar 18 doi: 10.1016/j.wneu.2022.03.068. PMID: 35314414
Chong AW, McAdory LE, Low DCY, Lim EJ, Leong NWL, Ho CL
Clin Imaging 2022 Feb;82:38-52. Epub 2021 Oct 29 doi: 10.1016/j.clinimag.2021.10.008. PMID: 34773811
Thomas C, Metrock K, Kordes U, Hasselblatt M, Dhall G
J Neurooncol 2020 May;148(1):39-45. Epub 2020 Apr 28 doi: 10.1007/s11060-020-03509-5. PMID: 32342334Free PMC Article
Mayl JJ, Flores MA, Stelzer JW, Liu B, Messina SA, Murray JV
Emerg Radiol 2018 Apr;25(2):215-218. Epub 2018 Feb 3 doi: 10.1007/s10140-018-1582-2. PMID: 29397464
Li P, Diao X, Bi Z, Hao S, Ren X, Zhang J, Xing J
J Clin Neurosci 2015 Nov;22(11):1776-84. Epub 2015 Aug 29 doi: 10.1016/j.jocn.2015.05.025. PMID: 26455422

Clinical prediction guides

Chong AW, McAdory LE, Low DCY, Lim EJ, Leong NWL, Ho CL
Clin Imaging 2022 Feb;82:38-52. Epub 2021 Oct 29 doi: 10.1016/j.clinimag.2021.10.008. PMID: 34773811
Yankelevich M, Finlay JL, Gorsi H, Kupsky W, Boue DR, Koschmann CJ, Kumar-Sinha C, Mody R
Cold Spring Harb Mol Case Stud 2021 Feb;7(1) Epub 2021 Feb 19 doi: 10.1101/mcs.a005272. PMID: 33608379Free PMC Article
Chen Y, Zhao R, Shi W, Li H
Clin Neurol Neurosurg 2021 Jan;200:106345. Epub 2020 Nov 4 doi: 10.1016/j.clineuro.2020.106345. PMID: 33203591
Ruiz-Garcia H, Huayllani MT, Incontri D, Whaley JJ, Marenco-Hillembrand L, Ebot J, Chaichana KL, Sheehan J, Quiñones-Hinojosa A, Trifiletti DM
J Neurooncol 2020 Sep;149(2):283-292. Epub 2020 Sep 8 doi: 10.1007/s11060-020-03603-8. PMID: 32897467
Li P, Diao X, Bi Z, Hao S, Ren X, Zhang J, Xing J
J Clin Neurosci 2015 Nov;22(11):1776-84. Epub 2015 Aug 29 doi: 10.1016/j.jocn.2015.05.025. PMID: 26455422

Recent systematic reviews

Gaddi MJS, Lappay JI, Chan KIP, Pascual JSG, Salonga AEM
Childs Nerv Syst 2021 Mar;37(3):799-807. Epub 2020 Sep 26 doi: 10.1007/s00381-020-04896-w. PMID: 32980905
Bunevicius A, Matukevicius A, Deltuva V, Gudinaviciene I, Pranys D, Tamasauskas A
World Neurosurg 2018 May;113:129-134. Epub 2018 Feb 13 doi: 10.1016/j.wneu.2018.02.012. PMID: 29452330

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