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Paraneoplastic syndromes are disorders that are triggered by an abnormal immune system responding to the presence of a tumour. They are non-metastatic effects that bother the entire body system even though the cancer is located in just one area. The symptoms can be haematologic, gastrointestinal, cutaneous, cardiovascular, muscular or renal in nature. Fever is the most common symptom you'll see in paraneoplastic symptoms but you can see many other symptoms as well. Sometimes the only thing you'll see is the paraneoplastic syndrome, which should prompt an evaluation for cancer in the body.

The frequency of paraneoplastic syndrome is only about 10-20 percent of all malignancies. Neurological paraneoplastic syndromes make up about 1 percent of those with cancer. The incidence of morbidity and mortality of these syndromes is not completely known. There is no predilection to certain races, sex or ages of people.

The symptoms occurring in paraneoplastic syndromes vary. Doctors who suspect a paraneoplastic syndrome should ask about a history of cancer. The symptoms include fever, particularly with blood cancers and kidney cell cancer, arthritis or pain in the joints, scleroderma, lupus or secondary amyloidosis, renal disease with high or low sodium and other electrolyte abnormalities, nephrotic syndrome, watery diarrhoea and anaemia or high red blood cell count, and migrating vascular thrombosis. There can be immature white blood cells in the bloodstream, eosinophilia, itching, herpes zoster, endocrine disorders like Cushing's syndrome, and neuromuscular disorders. These can include myasthenia gravis and other myasthenic disorders as well as symptoms of hypotonia, irritability and ataxia.

The causes of paraneoplastic syndromes are not known. Doctors believe that there are pyrogens that trigger fever and alterations in copper and zinc metabolism that trigger a bad taste in the mouth. Weight loss is felt to be due to bioactive molecules secreted by the tumour and osteoarthritic symptoms seem to be related to growth hormone and oestrogen or other sex hormone activity. The autoimmune diseases appear to be related to the production of antinuclear antibodies (ANA) that attack connective tissue. The kidney disease seems to be related to secondary renal amyloidosis and the sedimentation of immune-complexes in the nephrons of the kidney. Elevated erythropoietin levels seem to be related to the development of a high red blood cell count. Anaemia can result from internal hemorrhages and altered uptake of vitamins contributing to red blood cell synthesis.

The diagnosis of a paraneoplastic syndrome can be difficult. It can mimic a lot of other diseases so the index of suspicion must be high for the presence of cancers related to the paraneoplastic syndrome. There should be evaluation of the blood, urine and cerebrospinal fluid. If there is high or low red blood cell count, then you should look for cancer. Certain liver enzymes might be elevated in paraneoplastic syndromes. There can be elevations of albumin in the cerebrospinal fluid and an increase in globulins. Tumour markers can be elevated, such as CEA and CA-125. Autoantibodies may be detected in the blood or in tissue. Most are directed against neural tissue.

Imaging studies can look for the presence of the tumor in a paraneoplastic syndrome, such as CT scanning or MRI scanning of the entire body. Metastases can be found as well. Biopsies of the tissue can show the type of cancer in the body. This can be done with endoscopy or ultrasound guided needle biopsy. Skin biopsy can be done in cases of skin cancer.

Treatment of paraneoplastic syndromes begins by treating the underlying cancer. All the same treatment protocols are identified and used whether or not there is a paraneoplastic syndrome associated with the cancer. Immunosuppressive agents are used whenever there is an autoimmune disease functioning along with the cancer.

Surgical treatment is directed at the underlying tumor. Some may resolve without removing the tumour but this is not common. Removing the adrenal glands takes care of tumours secreting too much ACTH and the thymus gland is removed to regress certain paraneoplastic syndromes, like myasthenia gravis.

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Medical Negligence Solicitors

Our personal injury solicitors operate a specialist medical negligence compensation service. Our Paraneoplastic Syndromes solicitors deal with claims using a no win no fee arrangement which means that if you don�t win then you don�t pay them their professional costs. If you would like legal advice at no cost with no further obligation just complete the contact form or email our lawyers offices or use the helpline and a Paraneoplastic Syndromes solicitor will review your medical negligence compensation claim and phone you immediately.

HELPLINE: ☎ 1800 633 634

The author of the substantive medical writing on this website is Dr. Christine Traxler MD whose biography can be read here