Carcinome adrénocortical (CAC)
Vue déterministe de l'entité source de la LJAM. L'autorité clinique demeure avec les ID de la source cités et l'état d'approbation de l ́examinateur.
| ID | DIS-ADRENOCORTICAL-CARCINOMA |
|---|---|
| Type | Maladie |
| Alias | ACCCarcinome cortical adrénalCarcinome adrénocorticalCarcinome du cortex surrénalАдренокортикальна карцинома (АКК) |
| État | reviewed 2026-05-19 | pending_clinical_signoff |
| Maladies | Aucune déclaration |
| Sources | SRC-NCCN-GENETIC-FAMILIAL-BREAST-OVARIAN-2025 SRC-NCCN-NET-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 | 8370/3 |
|---|---|
| icd_o_3_topography | ["C74.0"] |
| icd_10 | C74.0 |
| who_classification | WHO Endocrine and Neuroendocrine Tumours 5th ed (2022): Adrenal cortical carcinoma |
Notes
Adrenocortical carcinoma is an aggressive rare cancer of the adrenal cortex. Bimodal age distribution (pediatric and adult peaks) reflects two distinct biological contexts: pediatric ACC is overwhelmingly hereditary (Li-Fraumeni / Beckwith-Wiedemann), adult ACC is mostly sporadic with ~10% hereditary component. Functional ACC presents with hormonal syndromes: hypercortisolism (Cushing — most common, ~50% of functional ACC), virilization (androgen-secreting; especially in women), feminization (estrogen- secreting; rare; in men), Conn syndrome (aldosterone-secreting; rare). Non-functional ACC presents with mass effect, abdominal pain, or as incidental imaging finding. Staging: ENSAT (European Network for the Study of Adrenal Tumors) staging system; tumor size >6 cm, capsular invasion, regional nodes, distant metastasis are key prognostic factors. Mitotic count (Weiss score / Helsinki score) drives recurrence-risk stratification. Treatment fundamentals (full Indications pending): radical adrenalectomy with curative intent for resectable disease — open approach historically preferred (laparoscopic associated with positive-margin / peritoneal-seeding risk in retrospective series). Mito...
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Algorithmes
ALGO-ACC-1L- ALGO-ACC-1LALGO-CASCADE-LFS-CARRIER-FAMILY-TESTING- ALGO-CASCADE-LFS-CARRIER-FAMILY-TESTINGALGO-MEN1-CARRIER-SURVEILLANCE- ALGO-MEN1-CARRIER-SURVEILLANCEALGO-PEDIATRIC-CANCER-PREDISPOSITION-TRIAGE- ALGO-PEDIATRIC-CANCER-PREDISPOSITION-TRIAGE
Maladie
DIS-CYSTIC-NEPHROMA- Néphrome kystiqueDIS-WILMS- Tumeur Wilms (néphroblastome)
Indication
IND-ACC-1L-MITOTANE-EDP- IND-ACC-1L-MITOTANE-EDPIND-ACC-1L-RESECTION-MITOTANE- IND-ACC-1L-RESECTION-MITOTANE
Signal d’alerte
RF-ACC-HIGH-RECURRENCE-RISK-BIOLOGY- Carcinome adrénocortical post-résection avec des caractéristiques à risque de récidive élevé — stade ENSAT...RF-LI-FRAUMENI-CONFIRMED-CARRIER- Patient has a confirmed germline TP53 pathogenic variant (positive germline test, distinc...
Protocole thérapeutique
REG-EDP-M-ACC- EDP-M (etoposide + doxorubicine + cisplatin + mitotane) — FIRM-ACT traitement thérapeutiqueREG-MITOTANE-ADJUVANT-ACC- Mitotane adjuvant en monothérapie (CAC)
Sources
SRC-ADIUVO-TERZOLO-2023- Mitotane adjuvant versus surveillance chez le carcinome adrénocortical localisé de faible grade (A...SRC-ENSAT-ACC-2018- Société européenne d'endocrinologie Directives de pratique clinique sur la gestion des...SRC-ESMO-ACC-2020- Carcinomes adrénocorticaux et phaéochromocytomes malins: ESMO-EURACAN Clinique Pratique...SRC-FIRM-ACT-FASSNACHT-2012- Chimiothérapie combinée dans un carcinome adrénocortical avancé