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Start free with EleplanCushing syndrome due to ectopic ACTH secretion
ORPHA:99889Disease
Also called Adrenocorticotropic hormone secretion syndrome · Ectopic ACTH secreting tumor · Ectopic Cushing syndrome · Occult ectopic ACTH secretion · Paraneoplastic Cushing syndrome
What it is
A form of adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome, an endogenous Cushing syndrome (CS), characterized by the secretion of ACTH by non-pituitary neuroendocrine tumors (NETs) of various locations and various degrees of histological differentiation and aggressiveness.
Key facts
- Age of onset
- Adult, Elderly
- Inheritance
- Not applicable
- Classified as
- Disease
Recorded for the broader condition
- Prevalence
- 1-9 / 1 000 000 (annual incidence)ACTH-dependent Cushing syndrome
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
Signs and symptoms
Very common80–99%
6Common30–79%
34- Abdominal obesity
- Abnormality of the cardiovascular system
- Abnormal libido
- Acne
- Adrenal hyperplasia
- Amenorrhea
- Anxiety
- Asthenia
- Atypical behavior
- Bruising susceptibility
- Capillary fragility
- Decreased eosinophil count
- Dorsocervical fat pad
- Ecchymosis
- Emotional lability
- Hirsutism
- Hyperpigmentation of the skin
- Hypertension
- Immunodeficiency
- Increased body weight
- Increased circulating androgen concentration
- Increased total leukocyte count
- Insomnia
- Intra-oral hyperpigmentation
- Lymphopenia
- Moon facies
- Muscle weakness
- Osteoporosis
- Plethora
- Poor wound healing
- Proximal amyotrophy
- Striae distensae
- Thin skin
- Truncal obesity
Sometimes5–29%
26- Abnormality of the lymph nodes
- Abnormality of the respiratory system
- Anorexia
- Atypical pulmonary carcinoid tumor
- Depression
- Diarrhea
- Flushing
- Impaired glucose tolerance
and 18 more in this range
Rare1–4%
15- Dementia
- Hypergastrinemia
- Medullary thyroid carcinoma
- Neoplasm of the stomach
- Pancreatic adenocarcinoma
- Pancreatoblastoma
- Panic attack
- Paranoia
and 7 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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