Paraneoplastic syndrome

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Paraneoplastic syndrome refers to a group of rare disorders that are triggered by an abnormal immune system response to a cancerous tumor known as a "neoplasm." Paraneoplastic syndromes are thought to happen when cancer-fighting antibodies or white blood cells, known as T cells, mistakenly attack normal cells in the nervous system. These syndromes can affect various systems of the body, including the nervous, endocrine, and immune systems.

Symptoms

The symptoms of paraneoplastic syndromes vary greatly depending on the system affected, and they often precede the diagnosis of a malignancy. Neurological symptoms can include difficulty walking, swallowing, loss of muscle coordination, or loss of sensation in the limbs, a condition known as peripheral neuropathy. Endocrine symptoms can include weight loss, weakness, or fatigue due to adrenal insufficiency, or excessive thirst and urination due to diabetes insipidus.

Causes

Paraneoplastic syndromes are caused by the body's immune response to neoplasms. The exact mechanism is not fully understood, but it is believed that the immune system misidentifies normal proteins in the body as foreign due to their similarity to tumor proteins. This results in an immune response that attacks both the tumor and the normal tissues, leading to the symptoms of the syndrome.

Diagnosis

Diagnosis of paraneoplastic syndromes can be challenging due to their rarity and the wide range of possible symptoms. They are often discovered during the workup for an unrelated condition. Diagnostic tests may include blood tests to detect specific antibodies associated with these syndromes, imaging studies to identify underlying tumors, and neurological tests to assess the extent of neurological damage.

Treatment

Treatment of paraneoplastic syndromes focuses on treating the underlying cancer and managing the symptoms of the syndrome. This may involve chemotherapy, radiation therapy, or surgery to remove the tumor. Medications may also be used to suppress the immune system and reduce the severity of the symptoms.

Prognosis

The prognosis for individuals with paraneoplastic syndromes varies widely and depends on several factors, including the type and stage of the underlying cancer, the severity of the syndrome symptoms, and the individual's overall health.

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Contributors: Bonnu, Prab R. Tumpati, MD