Ectopic syndrome | Ectopic Cushing Syndrome | Ectopic ACTH Syndrome

 


Ectopic Cushing syndrome is some other form of Cushing syndrome which is a tumor present outside the pituitary gland that produces a hormone called ACTH - adrenocorticotropic hormone.


Cushing’s disease means when the excessive ACTH can be produced by a tumor in the pituitary gland or else if the tumor is located outside the pituitary gland which is typically called ectopic ACTH secretion. Usually these Ectopic ACTH-secreting tumors occur mostly in men than women. These tumors are usually benign or malignant but the risk of malignancy is very low. Generally these ACTH-producing tumors are mostly found in the chest in up to 52% of Ectopic Cushing syndrome patients. These ACTh producing tumors can also produced by other tumors like thymomas in 5% of overall patients,tumor called pancreatic islet tumors 1% of overall patients and tumor called medullary carcinomas of thyroid in almost 2% of overall patients


Causes


Cushing syndrome is a type of disorder which typically occurs when your body has higher than normal levels of the hormone cortisol. Usually these hormones are produced in the adrenal glands. Too much hormone cortisol because of various problems. If there is too much amount of ACTH hormone present in your blood , this is the main cause of it. Usually ACTH is produced by the pituitary gland in small amounts and then it signals the adrenal glands which produces cortisol. In some cases, other types of cells which present outside the pituitary gland make large amounts of ACTH. This is called ectopic Cushing syndrome. Ectopic is generally occurring in an unusual place in your body. 


Ectopic Cushing syndrome is usually caused by tumors which release ACTH hormone. In some rare cases, Tumors can release ACTH which includes :


  • In the lungs - Benign carcinoid tumors 

  • In the pancreas - Islet cell tumors

  • In the thyroid - Medullary carcinoma of the 

  • In the lung - Small cell tumors 

  • In the thymus gland -Tumors


Symptoms


Ectopic Cushing syndrome will cause a lot of various symptoms. Some people have many symptoms but some others will experience a few of them. People mostly with some type of Cushing syndrome have:


  • Round, red, and full face like moon face

  • Slow growth development in children

  • central obesity - Weight gain by the fat accumulation on the trunk, but in some cases, fat loss from the arms, legs, and buttocks 


Skin changes which often seen in:


  • Skin infections

  • Purple stretch marks which usually appear as 1/2 inch to 1 centimeter or more wide called striae on the skin of many areas which includes abdomen, thighs, upper arms, and breasts

  • Thin skin with easy bruising


Changes in Muscle and bone which includes:


  • Backache, which usually occurs with your daily routine activities

  • Bone pain or tenderness

  • Accumulation of fat between your shoulders and above the collar bone areas

  • Rib and spine fractures which is because of thinning of the bones

  • Weak muscles, particularly in the hips and shoulder areas


Systemic Body-wide problems which includes:


  • Type 2 diabetes mellitus

  • High blood pressure

  • High cholesterol and triglycerides


Women might have other symptoms which includes:


  • Excess or abnormal hair growth on the specific areas like face, neck, chest, abdomen, and thighs

  • Periods which become irregular or it stop suddenly


Men might have other symptoms which includes:


  • Decreased or no desire for sexual activities

  • Become Impotence


Other symptoms that may occur include:


  • Changes in your Mental state such as depression, anxiety, or changes in behavior

  • Fatigue

  • Headache

  • Increased thirst and urination


Exams and Tests


Your healthcare professional will do a proper physical examination and they may ask about your symptoms.


Some Tests which may be done include:


  • They take 24-hour urine or pee sample which helps to measure cortisol and creatinine levels in your body

  • They take Blood sample from your body to check ACTH, cortisol, and potassium levels which  will be often very low in ectopic Cushing syndrome

  • Dexamethasone suppression test they may be both high and low dose

  • Inferior petrosal sinus sampling which is a special test that helps to measures ACTH from veins which is near the brain and in the chest

  • Fasting glucose

  • MRI and high resolution CT scans which usually help you to find the tumor and in some cases nuclear medicine scans may be needed


Treatment

Your physical provider will suggest surgery to remove the tumor. Because this is one the best treatment methods. Surgery is especially possible when the tumor is noncancerous called benign.


In some rare cases, if the tumor is cancerous then it spreads to other areas of the body before the healthcare provider can identify the illness with cortisol production. Surgery won't be helpful for these cases. But your provider prescribes some medications which generally block the cortisol production.


If the tumor can’t be found or medications won't fully block your cortisol production, they suggest you remove the both adrenal glands.


Outlook


If you untreated the Cushing’s syndrome, it will lead to fatal, so people with this health condition should follow their regular medical care and also follow the treatment plan. Surgery can be useful, if a tumor is found and cancerous. If a tumor is not found, Cushing syndrome must be treated with medicines or adrenalectomy if it is needed.


Frequently Asked Questions


Q1. What is ectopic hormone syndrome?

Ans: Ectopic or inappropriate hormone production (paraneoplastic endocrine syndrome) is the synthesis of hormones by tumors from tissues that do not normally produce the hormone.


Q2. What is the most common cause of ectopic ACTH secretion?

Ans: Ectopic ACTH secretion typically results from an occult, slow-growing bronchial carcinoid tumor. 


Q3. Does PCOS cause ectopic?

Ans: PCOS is associated with an increase in subfertility, ectopic pregnancy and early pregnancy loss (EPL).


Q4. How is ectopic diagnosed?

Ans: An ectopic pregnancy is usually diagnosed by carrying out a transvaginal ultrasound scan.


Q5. What is ACTH deficiency called?

Ans: Low ACTH and high cortisol levels: This may mean Cushing's syndrome or a tumor of the adrenal gland.

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