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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.
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).
How 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.
How common: a minority of the 2 to 5 percent of paediatric brain tumours that are choroid plexus tumours (J Neurosurg Pediatrics 2012).
Treatment: 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.
No registry figure for the carcinoma alone is in the sources read.
Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
Gliomas infiltrate along white matter and can cross the corpus callosum, medulloblastoma sits in the cerebellum, and CNS lymphoma favours deep periventricular tissue; none spread through lymph nodes.
No conventional lymphatics: gliomas spread along white matter tracts and, rarely, through cerebrospinal fluid; medulloblastoma can seed the spine.
Same organ: Lactotroph pituitary neuroendocrine tumour (prolactinoma), Somatotroph pituitary neuroendocrine tumour (acromegaly), Corticotroph pituitary neuroendocrine tumour (Cushing disease and silent corticotroph tumour), Gonadotroph pituitary neuroendocrine tumour (non-functioning adenoma), Thyrotroph pituitary neuroendocrine tumour (TSH-secreting), Pineocytoma and pineal parenchymal tumour of intermediate differentiation, Pineoblastoma, Papillary tumour of the pineal region, Glioma & glioblastoma, Primary CNS lymphoma, Medulloblastoma, Paediatric low-grade glioma, Diffuse midline glioma, H3 K27-altered (including DIPG), Atypical teratoid/rhabdoid tumour (ATRT), Ependymoma, Craniopharyngioma, Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma, Brain and spinal cord tumours (all types), Astrocytoma, IDH-mutant (grades 2 to 4), Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, Paediatric high-grade glioma (excluding diffuse midline glioma), Meningioma, Brain metastases (secondary brain tumours), Vestibular schwannoma (acoustic neuroma), Central nervous system germ cell tumours (germinoma and non-germinomatous), Spinal cord tumours (intramedullary and intradural), WNT-activated medulloblastoma, SHH-activated medulloblastoma, Group 3 and group 4 medulloblastoma (non-WNT/non-SHH)
No subtypes recorded beyond the ones named in the family strip above.
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.
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Query for this cancer: (TITLE:"Choroid plexus carcinoma" OR ABSTRACT:"Choroid plexus carcinoma" OR TITLE:"Choroid plexus carcinoma WHO grade 3" OR ABSTRACT:"Choroid plexus carcinoma WHO grade 3" OR TITLE:"Malignant choroid plexus tumour" OR ABSTRACT:"Malignant choroid plexus tumour" OR TITLE:"CPC" OR ABSTRACT:"CPC") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Choroid plexus carcinoma, not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
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Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
Dose reduce or avoid for CrCl below 60 (carboplatin is the alternative).
Reduce to 75% for CrCl 15-50.
See all on the product pages:CisplatinEtoposide·Printable cards in the navigator
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