{"entity":{"id":"choroid-plexus-carcinoma","kind":"cancer","name":"Choroid plexus carcinoma","aka":["Choroid plexus carcinoma (WHO grade 3)","Malignant choroid plexus tumour","CPC"],"tldr":"Choroid plexus carcinoma is a rare, aggressive brain tumour of infants and young children that grows from the tissue that makes spinal fluid inside the brain's ventricles, causing fluid build-up and pressure. It is strongly linked to an inherited TP53 fault (Li-Fraumeni syndrome), so families are tested; treatment is surgery, then chemotherapy and, in older children, radiotherapy.","summary":"Choroid plexus carcinoma is the grade 3 member of the choroid plexus tumours in the WHO central nervous system classification, distinguished from choroid plexus papilloma and atypical papilloma by frequent mitoses, necrosis and invasion (NCI PDQ). Choroid plexus tumours make up about 2 to 5 percent of paediatric brain tumours and most present with severe hydrocephalus; in 39 patients (31 papillomas, 8 carcinomas) perioperative management and oncological care remained debated (J Neurosurg Pediatrics 2012). Of 42 choroid plexus tumour patients at Children's Hospital Los Angeles, 6 (16.7 percent) had features of Li-Fraumeni syndrome (Pediatric Blood and Cancer 2012), and in southern Brazil, where the low-penetrance TP53 R337H founder mutation is common, 9 of 13 children with choroid plexus carcinoma (69 percent) carried it (Cancer 2011).\n\nHow it differs from its parent: a tumour of infancy arising inside the ventricles with hydrocephalus as the presenting problem, extreme vascularity that makes surgery hazardous, and the strongest association with germline TP53 mutation of any childhood brain tumour, so that germline testing is part of the work-up.\n\nHow common: a minority of the 2 to 5 percent of paediatric brain tumours that are choroid plexus tumours (J Neurosurg Pediatrics 2012).\n\nTreatment: maximal safe resection, often in stages after chemotherapy to shrink and devascularise the tumour, then platinum- and etoposide-based chemotherapy with radiotherapy for older children and residual disease, as the PDQ childhood brain tumour summaries describe; TP53 carriers are treated with radiotherapy sparing where possible because of second cancers.","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Choroid_plexus_carcinoma","links":[{"label":"NCI PDQ: rare cancers of childhood","url":"https://www.cancer.gov/types/childhood-cancers/patient/rare-childhood-cancers-pdq"},{"label":"J Neurosurg Pediatrics 2012: treatment of choroid plexus tumours, 20-year single-institution experience","url":"https://doi.org/10.3171/2012.8.peds12132"},{"label":"Pediatric Blood and Cancer 2012: choroid plexus tumours and Li-Fraumeni syndrome, Children's Hospital Los Angeles 1991 to 2010","url":"https://doi.org/10.1002/pbc.23349"},{"label":"Cancer 2011: TP53 R337H and paediatric choroid plexus carcinoma in southeast Brazil","url":"https://doi.org/10.1002/cncr.25826"}],"tags":["subtype-page","wave4","rare"],"related":["brain-tumours","childhood-cancers","pineoblastoma","atrt","adrenocortical"],"cancers":[],"sections":[],"technologies":["germline-testing"],"targets":["tp53"],"drugs":["cisplatin","carboplatin","etoposide","cyclophosphamide"],"companies":[],"institutions":[],"pathways":[],"terms":["li-fraumeni"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"paediatric","burden":"Choroid plexus tumours are about 2 to 5 percent of paediatric brain tumours, and carcinomas the minority: 8 carcinomas among 39 choroid plexus tumours in one 20-year series (J Neurosurg Pediatrics 2012). No registry figure for the carcinoma alone is in the sources read.","subtypes":[],"biomarkers":["Germline TP53 mutation (Li-Fraumeni syndrome)","Mitotic count, necrosis and brain invasion (grade 3 criteria)","Extent of resection","Hydrocephalus at presentation"],"standardOfCare":[{"setting":"All cases","approach":"Maximal safe resection, staged after chemotherapy where the tumour is too vascular; platinum- and etoposide-based chemotherapy; radiotherapy for older children and residual disease; germline TP53 testing.","refs":["brain-tumours","cisplatin","etoposide","germline-testing","li-fraumeni"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"brain-tumours"},"route":"/cancers/choroid-plexus-carcinoma/","neighbours":{"cancer":[{"id":"adrenocortical","kind":"cancer","name":"Adrenocortical carcinoma","route":"/cancers/adrenocortical/"},{"id":"atrt","kind":"cancer","name":"Atypical teratoid/rhabdoid tumour (ATRT)","route":"/cancers/atrt/"},{"id":"brain-tumours","kind":"cancer","name":"Brain and spinal cord tumours (all types)","route":"/cancers/brain-tumours/"},{"id":"childhood-cancers","kind":"cancer","name":"Childhood cancers (all types)","route":"/cancers/childhood-cancers/"},{"id":"pineoblastoma","kind":"cancer","name":"Pineoblastoma","route":"/cancers/pineoblastoma/"},{"id":"pineal-parenchymal-tumours","kind":"cancer","name":"Pineocytoma and pineal parenchymal tumour of intermediate differentiation","route":"/cancers/pineal-parenchymal-tumours/"}],"technology":[{"id":"germline-testing","kind":"technology","name":"Germline (hereditary) testing","route":"/technologies/germline-testing/"}],"target":[{"id":"tp53","kind":"target","name":"TP53","route":"/targets/tp53/"}],"drug":[{"id":"carboplatin","kind":"drug","name":"Carboplatin","route":"/drugs/carboplatin/"},{"id":"cisplatin","kind":"drug","name":"Cisplatin","route":"/drugs/cisplatin/"},{"id":"cyclophosphamide","kind":"drug","name":"Cyclophosphamide","route":"/drugs/cyclophosphamide/"},{"id":"etoposide","kind":"drug","name":"Etoposide","route":"/drugs/etoposide/"}],"term":[{"id":"li-fraumeni","kind":"term","name":"Li-Fraumeni syndrome (germline TP53)","route":"/terms/li-fraumeni/"}]}}