Choroid plexus carcinoma
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-CHOROID-PLEXUS-CARCINOMA |
|---|---|
| Type | Disease |
| Aliases | CPCChoroid plexus papilloma (atypical / malignant variants)Pediatric choroid plexus carcinomaКарцинома хоріоїдного сплетення |
| Status | reviewed 2026-05-18 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-COG SRC-NCCN-CNS-2025 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 9390/3 |
|---|---|
| icd_o_3_topography | ["C71.5"] |
| icd_10 | C71.5 |
| who_classification | WHO CNS Tumours 5th ed (2021): Choroid plexus carcinoma (Grade 3); choroid plexus papilloma (Grade 1) and atypical choroid plexus papilloma (Grade 2) are benign / intermediate counterparts |
Notes
One of the strongest single-tumor signals for germline TP53 — CPC diagnosis in a child should trigger germline TP53 testing regardless of family history (per Chompret + Li-Fraumeni 2015 testing criteria). Treatment: maximal safe surgical resection (gross total resection is prognostic dominant — bleeding-prone tumor with intraventricular drainage challenges); adjuvant chemotherapy (regimens vary — ICE, carboplatin-VP16, CCNU-based); craniospinal RT in age-appropriate patients (typically deferred under age 3 to avoid neurocognitive toll — high-dose chemo + autoSCT bridge sometimes used). Li-Fraumeni carriers require lifelong Toronto-protocol surveillance after treatment (cross-syndrome second-primary risk).
Used By
Algorithms
ALGO-CASCADE-LFS-CARRIER-FAMILY-TESTING- ALGO-CASCADE-LFS-CARRIER-FAMILY-TESTING