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Cushing's syndrome

Cushing's syndrome or Cushing's disease is a hormonal disorder that typically affects adults in the age group 20 - 50 years. This disease is named after the American surgeon Harvey Cushing. Since Cushing's syndrome is characterized by high levels of cortisol in the body, it is also known as 'hypercortisolism'. Cortisol is one of the hormones produced by the adrenal glands and plays a vital role in regulating blood pressure and maintaining the functioning of the cardiovascular system. Cortisol aids the metabolization of proteins and fats and helps the body's response to stress. Adenomas in the pituitary are responsible for Cushing's syndrome. This is more likely in women. Adrenal tumors are sometimes responsible for increased levels of cortisol in the blood.


Typical symptoms of Cushings syndrome include upper body obesity and thinning limbs. Reduced growth rate is noticed among children suffering from Cushing's syndrome. the skin becomes fragile and easily prone to bruising. The patient will notice purple stretch marks around the abdomen, thighs and arms. Persons suffering from Cushing's syndrome tend to experience severe fatigue, weakness in the muscles and elevated levels of blood pressure and blood sugar. There is extra hair growth on the face, chest and abdomen. A person suffering from Cushing's syndrome may suffer from depression and anxiety. Women may notice irregular or cessation of menstrual cycle. There is decrease in libido in men and women. Excessive cortisol produces a characteristic hump of fat between the shoulders. High doses of corticosteroids taken over a prolonged period can trigger Cushing's syndrome.


If left untreated, Cushing's syndrome is usually fatal. Blood and urine tests help in identifying high cortisol levels. A urine test measures how much cortisol is being produced. It is also essential to identify the cause and location of the abnormality that releases excessive cortisol. Biochemistry tests such as dexamethasone suppression test and corticotropin-releasing hormone (CRH) stimulation test also help in testing for Cushing's syndrome. MRI scans aid in locating tumors that may be the cause for Cushings disease. Treatment for Cushing's syndrome can range from radiation and chemotherapy to surgery to remove tumors. Use of cortisol-inhibiting drugs may also be resorted to.

ACTH Test

ACTH also known as adrenocorticotropic hormone is a hormone produced by the pituitary gland. This hormone in turn regulates the production of another important hormone cortisol, made by adrenal glands. Cortisol, known as a 'stress hormone', controls varied reactions in our body that take place in response to stress. Cortisol regulates blood pressure and blood sugar levels in the body and helps in maintaining immune function and anti-inflammatory processes. ACTH travels through the bloodstream to the adrenal glands and stimulates the adrenals to release cortisol. An ACTH blood test is done to measure the level of the adrenocorticotropic hormone in the blood.


Too much or too little ACTH level reveals problems related to adrenal glands or pituitary glands. High level of ACTH points to problems with adrenal glands and low level of ACTH may imply defective pituitary glands. The results of ACTH test is extremely useful in diagnosing Cushing syndrome and adrenal insufficiency.

Higher values of ACTH test are associated with one of the following conditions:



Lower levels of ACTH could mean adrenal tumor, Exogenous Cushing syndrome or hypopituitarism, a pituitary dysfunction leading to little or no production of hormone.


Preparing for the test

Patient should not eat or drink for 10 hours prior to the test. It is advisable to take a diet low in carbohydrates for two to three days before the test. Exercise and alcohol should be avoided for 12 hours before the test. Few steroid medications result in low levels of ACTH, hence patient should share all the information regarding the current medication with the doctor. Patient should not have undergone any medical test that uses a radioactive tracer for a week before an ACTH test.


ACTH levels do not remain in the same range through the day. The plasma ACTH levels are highest in the morning and start to decline during the waking hours. Therefore, blood is usually collected in the morning hours or multiple blood samples are sought for accurate diagnosis.

The Normal reference range is as follows:

Morning: Less than 80 pg/mL or less than 18 pmol/L

Evening: Less than 50 pg/mL or less than 11 pmol/L

A significant deviation from the normal range could mean defective adrenal glands or pituitary gland and further investigation is ordered for the accurate diagnosis.


Paraneoplastic Syndrome

The first report of Paraneoplastic syndrome is attributed to a French physician, M Auche, who described the involvement in a peripheral nervous system in cancer patients in 1890. This syndrome is a group of signs and symptoms caused by a substance that is produced by a tumor, or in reaction to a tumor. Paraneoplastic syndrome is defined as a rare disorder triggered by a response to neoplasm of an immune system that is altered. These are clinical syndromes produced by tumor although they occur remotely from the tumor itself.


The symptoms may be of any nature – endocrine, neuromuscular or musculoskeletal, cardiovascular, cutaneous, hematologic, gastrointestinal, renal or miscellaneous. Fever is the most common sign. Other clinical symptoms may be benign but syndromes could be varied; from a malignant carcinoid syndrome to Cushing syndrome. In cancer patients, the central nervous system involvement can be detected and it is a prominent manifestation of cancer.

Because of their protean manifestations, Paraneoplastic syndromes are managed by a medical team of physicians, including medical oncologists, surgeons, radiation oncologists, endocrinologists, hematologists, neurologists and dermatologists.


Causes

This syndrome can be due to a number of causes, including hormones or biologically active products, made by tumor, which blocks normal hormone, autoimmunity, immune-complex production and immune suppression. But, this is not caused by the primary tumor itself, or by its metastases, or by compression, infection, nutritional deficiency, or treatment of tumor.

Patients with family history of malignancies are at an increased risk and should be screened for cancer. Paraneoplastic syndromes occur typically among middle-aged to older patients. They are mostly diagnosed with cancers of the lung, breast, ovaries, or lymphatic system or lymphoma.

These symptoms sometimes appear before the diagnosis of a malignancy. When the tumor breaks immune tolerance and begins to attack the normal tissue expressing that protein, this syndrome become prominent.


Types of Paraneoplastic Syndrome

Due to the complexity of this syndrome, its clinical presentations may vary greatly. Hence, Paraneoplastic syndromes is divided into four main categories - endocrine, neurological, mucocutaneous, and hematological syndromes as well as a host of others including rheumatologic, renal, gastrointestinal, Cutaneous and miscellaneous.


Fever, dysgeusia, anorexia, and cachexia are included under miscellaneous category. Fever is associated with lymphomas, acute leukemias, sarcoma and carcinomas.

Rheumatologic syndrome includes polyarthritis, particularly in patients with myelomas, lymphomas, acute leukemia, malignant tumors of the colon, pancreas, prostrate. Scleroderma may precede the evidence of tumor. Widespread form of malignancies of breast, uterus and lung can be observed.

Renal syndrome is characterized by hypokalemic nephropathy, and it occurs in 50% of individuals with ACTH secreting tumors of the lung. Nephrotic syndrome is observed in patients with Hodgkin lymphoma, non Hodgkin lymphoma, malignancies of lung, thyroid, colon, breast, ovary and pancreatic head.

Watery diarrhea and electrolyte imbalance, leads to asthenia, confusion and exhaustion, all characteristic of gastrointestinal syndromes. Severe prostaglandins lead to malabsorption and unavailability of nutrients.


Hematologic syndromes are related to anemia, thrombocytosis, disseminated intravascular coagulation, and leukemoid reactions from several types of cancers. Leukemoid reactions due to immature white blood cells in the blood stream are accompanied by hypereosinophilia and itching.

Cutaneous syndromes are manifested by itching and flushes, alopecia, or hypertrichosis. Blackish pigmentation of the skin usually occurs in patients with metastatic melanomas or pancreatic tumors.

Endocrine syndromes resemble common endocrine disorders. Increased serum and urine cortisol concentrations are common examples of this malignancy.


Neurologic or neuromuscular syndromes relate to cancers and such disorders affect 6% of all patients with cancer. Neuromuscular symptoms mimic common neurological conditions. Myasthenia gravis is a common Paraneoplastic syndrome in patients with thymoma, a malignancy arising from epithelial cells of the thymus.

Paraneoplastic limbic encephalitis is characterized by depression, seizures, irritability and short term memory loss and neurologic symptoms rapidly resemble dementia.

Paraneoplastic cerebellar degeneration causes gait difficulties, dizziness, nausea and diplopia. Sensory neuropathy affects lower and upper extremities and there is progressive memory loss, either symmetric or asymmetric.


Diagnosis

As Paraneoplastic syndromes may evolve over weeks to months, they usually stabilize regardless whether the patient's underlying condition improves or worsens. Since this is the first manifestation of cancer, patients should undergo investigation for cancer. Other diagnoses are membrane disease, dementia, encephalopathy, encephalitis, myelitis, anemia, bone marrow failure, chronic fatigue syndrome, mixed connective tissue disease, polycythemia vera and polymyalgia rheumatica among others.

Complete laboratory tests in blood, urine, cerebrospinal fluid, protein electrophoresis, assays for auto antibodies, Endoscopy and imaging studies including whole body scans and FDG-PET scans are prescribed to detect extremely small tumors especially useful for patients with neurologic diseases.


Treatment of Paraneoplastic Syndrome

Treatment for this syndrome varies depending upon the type and location of tumors. The first option of treatment of the underlying tumor is by therapeutic protocols, a combination of surgery, radiation, chemotherapy. Second therapeutic option is for patients with clearly identifiable antibodies by intravenous immunoglobulins, steroids, or plasma exchange.

Surgical treatment is typically directed toward the underlying neoplasm although some Paraneoplastic disorders may resolve rapidly without surgery on the primary tumor.


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Collection of Pages - Last revised Date: December 11, 2017