Paraneoplastic Syndromes

What are Paraneoplastic Syndromes

Paraneoplastic syndromes are rare disorders triggered by the body’s defense system (immune system) in response to a cancerous tumor.

Paraneoplastic syndromes can affect many areas of the body, including the nervous system, hormone (endocrine) system, skin (dermatologic) system, blood (hematologic) system, and joints (rheumatologic) system.

What are the causes?

The exact cause of these syndromes is not known. However, these syndromes are believed to occur when cancerous tumor cells cause an immune response in the body system where the cancer is located.

The immune system activates white blood cells, also called T-cells, to fight cancer cells. White blood cells normally target cancer cells in the area. Paraneoplastic syndromes may occur when the cancer-fighting cells also target healthy cells, which causes damage.

Do paraneoplastic syndromes cause pain?

Paraneoplastic syndromes are relatively rare clinical symptom complexes due to nonmetastatic effects of cancer.

They may manifest as remote effects on the nervous system (e.g., sensorimotor peripheral neuropathy, mononeuritis multiplex, brachial neuritis, and painful peripheral neuropathy resulting from islet cell tumors or paraproteinuria).

The painful symptoms result from disturbances of the immunologic system and/or from substances (toxins, hormones, electrolyte imbalance) produced by the tumor but occurring remotely from the site of the tumor.

The most common paraneoplastic syndrome is fever, but presentation can vary from dermatomyositis-polymyositis to Cushing syndrome or the malignant carcinoid syndrome.

What increases the risk?

These syndromes may develop in any person who has a diagnosis of cancer. The most common cancers associated with these syndromes include:

  • Small cell lung cancer.
  • Ovarian cancer.
  • Testicular cancer.
  • Breast cancer.
  • Lymphatic system cancer.

What are the symptoms?

Symptoms generally develop over a period of days to weeks. Symptoms vary depending on the body system involved. Symptoms affecting the nervous system are most common and may include:

  • Trouble walking.
  • Trouble swallowing.
  • Loss of muscle tone.
  • Trouble sleeping.
  • Memory loss or problems with thinking.
  • Dizziness (vertigo).
  • Loss of fine motor skills.
  • Numbness or tingling of the hands, feet, or other areas of the body (neuropathy).
  • Slurred speech.
  • Vision problems.
  • Seizures.
  • Seeing, hearing, tasting, smelling, or feeling things that are not real (hallucinations).
  • Loss of feeling in the limbs.
  • Unusual or involuntary body movements.

Other symptoms may include:

  • Skin changes (acanthosis nigricans).
  • Electrolyte imbalance.
  • Joint pain or stiffness.
  • Blood clotting problems.

In many cases, symptoms of the syndromes occur before the cancerous tumor is found.

How is this diagnosed?

Diagnosis will include a medical history and a physical exam. You will also have tests done, which will depend on your symptoms and the specific syndrome your health care provider thinks you have. Tests may include:

  • Blood tests.
  • A procedure in which a small amount of the fluid that surrounds the brain and spinal cord is removed and examined (lumbar puncture or spinal tap).
  • MRI.
  • CT scan.
  • Positron emission tomography (PET scan).
  • Electroencephalogram (EEG).
  • Electromyelogram (EMG).

How is this treated?

There is no cure for these syndromes. Treatment will first focus on treating the cancer you have. Then, treatment will focus on helping your immune system reduce its response to the tumor. This may be done with:

  • Steroids.
  • Medicines to slow the immune response (immunosuppressants).
  • Anti-seizure medicines.
  • Blood cleansing (plasmapheresis).
  • Immunoglobulin antibodies given intravenously (IVIg). These help destroy the cells that are causing harm.
  • Medicines to improve muscle function.

Speech and physical therapy may also be recommended.

Follow these instructions at home:

  • Take over-the-counter and prescription medicines only as told by your health care provider.
  • Do any speech or physical therapy as told by your health care provider.
  • Keep all follow-up visits as told by your health care provider. This is important.
  • Consider joining a support group.

Contact a health care provider if:

  • You have a fever.
  • You have a reaction to a medicine you are taking.
  • Your symptoms change or get worse.

Get help right away if:

  • You have trouble breathing.
  • You have more trouble swallowing.

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