ectopic ACTH secretion syndrome

Summary

ectopic ACTH secretion syndrome (MONDO:0043472) is a disease and 4 clinical trials. Top therapeutic interventions include pasireotide, atumelnant, and clofutriben. A subtype of adrenal gland disorder — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Clinical trials: 4

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	ectopic ACTH secretion syndrome
Mondo ID	MONDO:0043472
MeSH	D000182
ICD-10-CM	E24.3
NCIT	C4387
SNOMED CT	626004
UMLS	C0001231
MedGen	103
GARD	0025861
Is cancer (heuristic)	no

Also known as: ACTH syndromes, ectopic · ectopic ACTH secretion · ectopic ACTH secretion syndrome · ectopic ACTH syndrome · ectopic ACTH syndromes · hypercortisolism due to nonpituitary tumor · hypercortisolism due to nonpituitary tumour · syndrome, ectopic ACTH · syndromes, ectopic ACTH

Disease family

This is a subtype of adrenal gland disorder. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › endocrine system disorder › adrenal gland disorder › ectopic ACTH secretion syndrome

Related subtypes (17): medulloadrenal hyperfunction, adrenal cortex disorder, adrenal medullary hyperplasia, pituitary dwarfism, pseudoleprechaunism syndrome, Patterson type, apparent mineralocorticoid excess, autoimmune polyendocrine syndrome type 1, adrenomyodystrophy, corticosteroid-binding globulin deficiency, Congenital adrenal insufficiency with 46, XY sex reversal OR 46,XY disorder of sex development-adrenal insufficiency due to CYP11A1 deficiency, adrenogenital syndrome, hypoaldosteronism disease, primary pigmented nodular adrenocortical disease, adrenoleukodystrophy, adrenal gland neoplasm, endogenous Cushing syndrome, isolated micronodular adrenocortical disease

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

Drugs indicated for this disease

No approved or late-stage (phase ≥3) drug is indicated for this disease; the following are in earlier-phase trials only.

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Pasireotide.

Clinical trials & evidence

Clinical trials

Clinical trials: 4.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	3
PHASE1/PHASE2	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT05804669	PHASE1/PHASE2	RECRUITING	A Study to Evaluate the Safety and PK of CRN04894 for the Treatment of Cushing’s Syndrome
NCT00958841	PHASE2	TERMINATED	Study of Pasireotide in Patients With Rare Tumors of Neuroendocrine Origin
NCT02780882	PHASE2	WITHDRAWN	SOM230 Ectopic ACTH-producing Tumors
NCT05307328	PHASE2	COMPLETED	SPI-62 as a Treatment for Adrenocorticotropic Hormone-dependent Cushing’s Syndrome

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
PASIREOTIDE	4	1
ATUMELNANT	2	1
CLOFUTRIBEN	2	1