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5 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
| Setting | Approach | Guideline |
|---|---|---|
| All cases | 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. | not mapped |