Ectopic ACTH Syndrome

Introduction to ectopic ACTH syndrome "Ectopic hormone secretion syndrome" means that a tumor originating from a non-endocrine tissue produces a hormone, or a tumor originating from the endocrine gland (such as medullary thyroid carcinoma), which is secreted except when the endocrine gland is normal. In addition to hormones (calcitonin), other hormones are also released. Ectopic ACTH syndrome is the earliest and most widely studied ectopic hormone secretion syndrome. More common in APUD tumors, such as oat cell bronchial lung cancer (about half). Carcinoids in different parts, as well as islet cancer, medullary thyroid carcinoma, pheochromocytoma, neuroblasts, melanoma, etc. Non-APUD tumors, such as lung adenocarcinoma, squamous cell carcinoma. Liver cancer can also be caused. basic knowledge The proportion of illness: 0.005% Susceptible people: no special people Mode of infection: non-infectious Complications: Acne Hypertension Progressive muscular dystrophy

Etiology of ectopic ACTH syndrome

Causes:

The cause is unknown, more common in APUD tumors, such as oat cell bronchial lung cancer (about half), carcinoids in different parts, as well as islet cancer, medullary thyroid carcinoma, pheochromocytoma, neuroblasts, melanoma, etc., non-APUD Tumors, such as lung adenocarcinoma, squamous cell carcinoma, and liver cancer can also be caused.

Ectopic ACTH Syndrome Prevention

Because tumor cells other than the pituitary gland secrete a large amount of ACTH, there is no effective preventive measures, early detection and early treatment is the key to the prevention and treatment of this disease.

Ectopic ACTH syndrome complications Complications Acne Hypertension Progressive Muscular Dystrophy

Clinically, it can be characterized by more typical Cushing's syndrome, such as full moon face, central obesity, purple pattern, hemorrhoids, rapid hypertension, fragility diabetes, muscle weakness, progressive muscular dystrophy, edema and mental disorders.

Symptoms of ectopic ACTH syndrome Common symptoms Hypertension Obesity after meals

The basis for clinical diagnosis of ectopic hormone secretion syndrome is:

(1) tumor and endocrine syndrome coexist, and the tumor has non-occurring and endocrine glands secreting the hormone at normal time;

(2) abnormal increase in hormone levels in the blood or urine;

(3) Hormone secretion is autonomic and cannot be inhibited by normal feedback mechanisms;

(4) Exclude other causes of the syndrome;

(5) After specific treatment of tumor (such as surgery, chemotherapy, radiotherapy, etc.), the hormone level decreases, and the symptoms of endocrine syndrome are relieved;

(6) taking the arteriovenous blood test hormone of the tumor during surgery, which proves that the hormone level in the venous blood is higher than that of the artery, or using the catheter to take the venous blood of the draining tumor and compare it with another venous blood sample far from the tumor to prove the blood in the tumor. Hormone content increased significantly;

(7) Confirmation of the presence of hormones by radiotherapy or biological methods in tumor extracts.

Examination of ectopic ACTH syndrome

Because the high-risk area of ectopic ACTH secretion tumor is the chest, the chest radiograph has become a routine examination item. Most thymoma can be detected by chest radiograph, bronchial carcinoid, medullary thyroid carcinoma, some tumors in the mediastinum are small, chest The film is often negative, it should be chest CT or MRI, even thoracoscopy and mediastinoscopy, abdominal ultrasound, CT is also necessary for the discovery of abdominal cavity, pelvic tumor, pancreas, adrenal gland, liver, retroperitoneal should focus on search, gonads also Should be included in the line of sight, ectopic ACTH secretion of the tumor is ubiquitous, so can not let go of any part.

Diagnosis and diagnosis of ectopic ACTH syndrome

According to the medical history, clinical manifestations and laboratory data is not difficult to make a diagnosis.

It is differentiated from pituitary Cushing syndrome.

The level of ACTH in patients with ectopic adrenocorticotropic hormone (ACTH) syndrome is higher than that of pituitary tumors, and ACTH secretion from pituitary tumors is generally not inhibited by high-dose dexamethasone, and metyrapone is inhibited. During cortisol synthesis, ACTH secretion in pituitary ACTH adenomas is increased, whereas ACTH-secreting ectopic tumors do not respond to this type. Corticotropin-releasing hormone (CRH) stimulates ACTH release in most patients with pituitary adenomas, but Patients with ACTH syndrome do not have this effect. The detection of ectopic secretion of ACTH tumors often depends on CT and MRI of the chest and abdomen. The CTH concentration in the pituitary of Cushing's disease patients with pituitary ACTH is higher than that of the diluted peripheral veins. Therefore, it can also be used. The two ratios (IPS/P) identify the two diseases.

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