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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 characterized 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).
Optic papillitis
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
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
Rashidi M, DaSilva VR, Minagar A, Rutka JT
Curr Neurol Neurosci Rep 2003 May;3(3):200-5. doi: 10.1007/s11910-003-0079-9. PMID: 12691624

Diagnosis

Patnaik A, Sekar A, Bansal S, Narayan Sahu R
World Neurosurg 2023 Nov;179:43-44. Epub 2023 Aug 9 doi: 10.1016/j.wneu.2023.08.012. PMID: 37562679
Wendt SP, Raviskanthan S, Mortensen PW, Lee AG
J Neuroophthalmol 2023 Dec 1;43(4):e328-e330. Epub 2022 Feb 25 doi: 10.1097/WNO.0000000000001492. PMID: 35234681
Yesilyurt M, Koksal A, Ogul H
Br J Hosp Med (Lond) 2022 Sep 2;83(9):1. Epub 2022 Sep 7 doi: 10.12968/hmed.2022.0017. PMID: 36193912
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
Rashidi M, DaSilva VR, Minagar A, Rutka JT
Curr Neurol Neurosci Rep 2003 May;3(3):200-5. doi: 10.1007/s11910-003-0079-9. PMID: 12691624

Therapy

Liu J, Luo M, Lv S, Tao S, Wu Z, Yu L, Lin D, Huang L, Wu L, Liao X, Zi J, Lai X, Yuan Y, Zhang W, Yang L
Front Endocrinol (Lausanne) 2022;13:865913. Epub 2022 Jul 5 doi: 10.3389/fendo.2022.865913. PMID: 35865311Free 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
Sufianov AA, Gaibov SS, Sufianov RA
J Neurosurg Pediatr 2015 Jul;16(1):107-11. Epub 2015 Apr 24 doi: 10.3171/2014.12.PEDS14306. PMID: 25910036
Addo NK, Kamaly-Asl ID, Josan VA, Kelsey AM, Estlin EJ
J Neurosurg Pediatr 2011 Aug;8(2):149-53. doi: 10.3171/2011.5.PEDS1187. PMID: 21806355
Stafrace S, Molloy J
Pediatr Radiol 2008 May;38(5):593. Epub 2008 Jan 22 doi: 10.1007/s00247-007-0737-1. PMID: 18210099

Prognosis

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
Rashidi M, DaSilva VR, Minagar A, Rutka JT
Curr Neurol Neurosci Rep 2003 May;3(3):200-5. doi: 10.1007/s11910-003-0079-9. PMID: 12691624
Patanakar T, Prasad S, Mukherji SK, Armao D
Clin Imaging 2000 May-Jun;24(3):130-1. doi: 10.1016/s0899-7071(00)00177-7. PMID: 11150677

Clinical prediction guides

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
Wiatr M, Figueiredo R, Stump-Guthier C, Winter P, Ishikawa H, Adams O, Schwerk C, Schroten H, Rudolph H, Tenenbaum T
Int J Mol Sci 2020 Aug 29;21(17) doi: 10.3390/ijms21176268. PMID: 32872518Free PMC Article
Banerjee G, Adams ME, Jaunmuktane Z, Alistair Lammie G, Turner B, Wani M, Sawhney IMS, Houlden H, Mead S, Brandner S, Werring DJ
Ann Neurol 2019 Feb;85(2):284-290. Epub 2019 Jan 17 doi: 10.1002/ana.25407. PMID: 30597599Free PMC Article
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
Raisanen JM, Davis RL
Pathology (Phila) 1993;2(1):103-16. PMID: 9420933

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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