Onco Wiki / Disease

Adrenocortical carcinoma (ACC)

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDDIS-ADRENOCORTICAL-CARCINOMA
TypeDisease
Aliases
ACCAdrenal cortical carcinomaAdrenocortical carcinomaCarcinoma of adrenal cortexАдренокортикальна карцинома (АКК)
Statusreviewed 2026-05-19 | pending_clinical_signoff
DiseasesNone declared
SourcesSRC-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.

Source YAML

icd_o_3_morphology8370/3
icd_o_3_topography["C74.0"]
icd_10C74.0
who_classificationWHO Endocrine and Neuroendocrine Tumours 5th ed (2022): Adrenal cortical carcinoma

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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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