Genomic coordinate (human 11:1,995,176 H19) & (mouse 7:142,129,267 H19).
Cytoband (human 11p15.5 H19) & (mouse 7qF3 H19).
Here I 🎁 present: “Adrenocortical Carcinoma”, Victor McKusick, Mendelian Inheritance in Man’, 1966. (H19) icd10=C74.0
Adrenocortical carcinoma (ACC) is an aggressive cancer developing in the outer layer of the adrenal glands, often causing excessive hormone production (Cushing’s syndrome) or abdominal pain. It occurs in a bimodal pattern (young children and adults 40–60). Treatment, primarily surgical removal (adrenalectomy), is often combined with medication or chemotherapy. ICD-10 Code: C74.0 — Adrenocortical Carcinoma
Code: C74.0
Category: Malignant neoplasm of adrenal gland
Specific site: Cortex of adrenal gland
What it Means
Adrenocortical carcinoma (ACC) is an aggressive cancer arising from the adrenal cortex, the outer layer of the adrenal gland responsible for producing steroid hormones (cortisol, aldosterone, and androgens).
Clinical Features
ACC may present as:
Hormone-secreting (functional) tumors
Excess cortisol → Cushing syndrome
Excess aldosterone → hypertension, hypokalemia
Excess androgens → virilization
Nonfunctional tumors
Abdominal or flank pain
Palpable mass
Incidental finding on imaging
Related ICD-10 Codes
C74.1 – Malignant neoplasm of adrenal medulla
C74.9 – Malignant neoplasm of adrenal gland, unspecified
Notes
C74.0 specifically excludes tumors of the adrenal medulla (e.g., pheochromocytoma).
For metastatic disease to the adrenal gland, coding differs (secondary malignant neoplasm codes).
There is evidence that adrenocortical carcinoma is caused by mutation in the H19 gene-RNA encoded on genomic coordinate 11:1,995,176 and cytoband 11p15.5 in humans.
H19 is a gene for a long noncoding RNA, found in humans and elsewhere. H19 has a role in the negative regulation (or limiting) of body weight and cell proliferation. The H19 gene also has a role in the formation of adrenocortical carcinoma.
