Paraneoplastic syndrome

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Paraneoplastic syndrome

A paraneoplastic syndrome is a disease

Disease

A disease or medical condition is an abnormal condition of an organism that impairs bodily functions, associated with specific symptoms and Medical signs....
or symptom

Symptom

A symptom is a departure from normal function or feeling which is noticed by a patient, indicating the presence of disease or abnormality. A symptom is subjective, observed by the patient, and not measured....
that is the consequence of the presence of cancer

Cancer

Cancer is a class of diseases in which a group of cell display uncontrolled growth , invasion , and sometimes metastasis . These three malignant properties of cancers differentiate them from benign tumors, which are self-limited, do not invade or metastasize....
in the body, but is not due to the local presence of cancer cells. These phenomena are mediated by humoral factors (by hormones

Hormone

Hormones are chemicals released by cells that affect cells in other parts of the body. Only a small amount of hormone is required to alter cell metabolism....
or cytokines

Cytokine

Cytokines are a category of signaling molecules that, like hormones and neurotransmitters, are used extensively in cell communication. They are proteins, peptides or glycoproteins....
) excreted by tumor cells or by an immune response against the tumor

Tumor

A tumor or tumour is the name for a swelling or lesion formed by an abnormal growth of cells . Tumor is not synonymous with cancer. A tumor can be Benign neoplasm, Carcinoma in situ or malignant, whereas cancer is by definition malignant....
. Paraneoplastic syndromes are typical among middle aged to older patients and they most commonly present with cancers of the lung

Lung cancer

Lung cancer is a disease of uncontrolled cell growth in tissue of the lung. This growth may lead to metastasis, which is the invasion of adjacent tissue and infiltration beyond the lungs....
, breast

Breast cancer

Breast cancer is a cancer that starts in the Cell of the breast in women and men. Worldwide, breast cancer is the second most common type of cancer after lung cancer and the fifth most common cause of cancer death....
, ovaries

Ovarian cancer

Ovarian cancer is a malignant tumor arising from an ovary. Although ovarian cancer is known to occur in many species, the majority of the medical literature and the focus of this article is on ovarian cancer in humans....
or lymphatic system (a lymphoma

Lymphoma

Lymphoma is a type of cancer that originates in lymphocytes of the immune system. They often originate in lymph nodes, presenting as an enlargement of the node ....
).

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Encyclopedia

A paraneoplastic syndrome is a disease

Disease

A disease or medical condition is an abnormal condition of an organism that impairs bodily functions, associated with specific symptoms and Medical signs....
or symptom

Symptom

Cancer

Hormone

Hormones are chemicals released by cells that affect cells in other parts of the body. Only a small amount of hormone is required to alter cell metabolism....
or cytokines

Cytokine

Tumor

Lung cancer

Breast cancer

Ovarian cancer

Lymphoma

Lymphoma is a type of cancer that originates in lymphocytes of the immune system. They often originate in lymph nodes, presenting as an enlargement of the node ....
). Sometimes the symptoms of paraneoplastic syndromes show even before the diagnosis of a malignancy

Malignant

Malignant is a medical term used to describe a severe and progressively worsening disease. The term is most familiar as a description of cancer....
.

Classification

Paraneoplastic syndromes can be divided into 4 main categories: Endocrine, neurological, mucocutaneous and hematological paraneoplastic syndromes, as well as others that may not fit into any of the above categories:

Syndrome class	Syndrome	Main causal cancers	Causal mechanism
Endocrine
	Cushing syndrome	small-cell lung cancer Small cell carcinoma Small cell carcinoma is a type of highly malignant carcinoma usually associated with the lung, though it can be associated with other topographies, such as in cervical cancer.... Pancreatic carcinoma Neural tumors thymoma Thymoma In medicine , thymoma is a tumor originating from the thymus. It consists of any type of thymic epithelial cell as well as lymphocytes that are usually abundant and probably not neoplastic....	Ectopic ACTH and ACTH-like substance
	SIADH Syndrome of inappropriate antidiuretic hormone The syndrome of inappropriate antidiuretic hormone is a condition commonly found in the hospital population, especially in patients being hospitalized for central nervous system injury....	small-cell lung cancer Small cell carcinoma Small cell carcinoma is a type of highly malignant carcinoma usually associated with the lung, though it can be associated with other topographies, such as in cervical cancer.... CNS malignancies	Atrial natriuretic hormone or atrial natriuretic hormones
	Hypercalcemia	lung cancer Lung cancer Lung cancer is a disease of uncontrolled cell growth in tissue of the lung. This growth may lead to metastasis, which is the invasion of adjacent tissue and infiltration beyond the lungs.... (typically squamous cell) breast carcinoma Renal carcinoma Adult T cell leukemia/lymphoma Ovarian carcinoma	PTHrP Parathyroid hormone-related protein Parathyroid hormone-related protein is a protein member of the parathyroid hormone family. It is occasionally secreted by cancer cells . However, it also has normal functions.... (Parathyroid hormone-related protein), TGF-a TGF alpha Transforming growth factor alpha is upregulated in some human cancers. It is produced in macrophages, brain cells, and keratinocytes, and induces epithelium development.... , TNF Tumor necrosis factor Tumor necrosis factor is a cytokine involved in systemic inflammation and is a member of a group of cytokines that stimulate the acute phase reaction.... , IL-1 Interleukin 1 Interleukin-1 is one of the first cytokines ever described. Its initial discovery was as a factor that could induce fever, control lymphocytes, increase the number of bone marrow cells and cause degeneration of bone joints....
	Hypoglycemia Hypoglycemia Hypoglycaemia or hypoglycemia is the medical term for a Pathology state produced by a lower than normal level of Blood glucose. The term hypoglycemia literally means "under-sweet blood" ....	Fibrosarcoma Fibrosarcoma Fibrosarcoma is a malignant tumor derived from fibrous connective tissue and characterized by immature proliferation fibroblasts or undifferentiated anaplastic spindle cells.... Other mesenchymal sarcomas Hepatocellular carcinoma Hepatocellular carcinoma Hepatocellular carcinoma is a primary cancer of the liver. Most cases of HCC are secondary to either a viral hepatitis infection or cirrhosis ....	Insulin Insulin Insulin is a hormone with extensive effects on both metabolism and several other body systems . Insulin causes most of the body's cells to take up glucose from the blood , storing it as glycogen in the liver and muscle, and stops use of fat as an energy source.... or insulin-like substance
	Carcinoid syndrome Carcinoid syndrome Carcinoid syndrome refers to the array of symptoms that occur secondary to carcinoid.Carcinoid tumors are discrete, yellow, well-circumscribed tumors that can occur anywhere along the gastrointestinal tract and in the lung....	Bronchial adenoma (carcinoid Carcinoid Carcinoid is a slow-growing but malignant type of neuroendocrine tumour, originating in the cells of the neuroendocrine system.In 2000, the World Health Organization redefined "carcinoid", but this new definition has not been accepted by all practitioners.... type) Pancreatic carcinoma Gastric carcinoma	Serotonin Serotonin Serotonin is a monoamine neurotransmitter synthesized in serotonergic neurons in the central nervous system and enterochromaffin cells in the gastrointestinal tract of animals including humans.... , bradykinin Bradykinin Bradykinin is a nonapeptide that causes blood vessels to enlarge , and therefore causes blood pressure to lower. A class of drugs called ACE inhibitors, which are used to lower blood pressure, increase bradykinin further lowering blood pressure....
	Polycythemia Polycythemia Polycythemia is a condition in which there is a net increase in the total number of blood cells, primarily red blood cells, in the body. The overproduction of red blood cells may be due to a primary process in the bone marrow , or it may be a reaction to chronically Hypoxia or, rarely, a malignancy....	See hematological paraneoplastic syndromes
Neurological	Lambert-Eaton myasthenic syndrome Lambert-Eaton myasthenic syndrome Lambert-Eaton myasthenic syndrome is a rare autoimmune disorder which affects calcium channels of the nerve-muscle junction. The etiology of LEMS may resemble myasthenia gravis, but there are substantial differences between the clinical presentation and pathogenetic features of the two disorders.... (LEMS)	small-cell lung cancer Lung cancer Lung cancer is a disease of uncontrolled cell growth in tissue of the lung. This growth may lead to metastasis, which is the invasion of adjacent tissue and infiltration beyond the lungs....	Immunologic
	Paraneoplastic cerebellar degeneration Paraneoplastic cerebellar degeneration Paraneoplastic cerebellar degeneration is a paraneoplastic phenomenon associated with lung, ovarian, breast, and other cancers.It is believed to be due to an autoimmune reaction targeted against components of the central nervous system ....	lung ovarian cancer breast carcinoma
	Encephalomyelitis Encephalomyelitis Encephalomyelitis is a general term for inflammation of the brain and spinal cord, describing a number of disorders:* acute disseminated encephalomyelitis or postinfectious encephalomyelitis, a demyelinating disease of the brain and spinal cord, possibly triggered by vaccination or virus infection;...		inflammation of the brain and spinal cord
	Limbic encephalitis Limbic encephalitis Limbic encephalitis is a form of encephalitis.In a small number of cases, the pathogens responsible for encephalitis attack primarily the limbic system , often causing memory deficits similar to those observed in Alzheimer's disease or Creutzfeldt-Jakob disease ...
	Brainstem encephalitis
	Opsoclonus Opsoclonus Opsoclonus refers to uncontrolled eye movements.Opsoclonus consists of rapid, involuntary, multivectorial , unpredictable, conjugate fast eye movements without interSaccade intervals.... (involving eye movement)
	Encephalitis Encephalitis Not to be confused with syphilis, although that can cause encephalitis as well.Encephalitis is an Acute inflammation of the brain.Encephalitis with meningitis is known as meningoencephalitis....	Teratoma Teratoma A teratoma is a kind of tumor . Definitive diagnosis of a teratoma is based on its histology: a teratoma is a tumor with biological tissue or organ components resembling normal derivatives of all three germ layers....
	Polymyositis Polymyositis Polymyositis is a type of chronic inflammatory myopathy, related to dermatomyositis and inclusion body myositis. Polymyositis means 'many muscle inflammation'....
Mucocutaneous	Acanthosis nigricans Acanthosis nigricans Acanthosis nigricans is a brown to black, poorly defined, velvety hyperpigmentation of the skin. It is usually found in body folds, such as the posterior and lateral folds of the neck, the axilla, groin, navel, and other areas....	Gastric carcinoma Lung carcinoma Uterine carcinoma	Immunologic Secretion of EGF Epidermal growth factor Epidermal growth factor or EGF is a growth factor that plays an important role in the regulation of cell growth, proliferation, and Cellular differentiation by binding to its receptor Epidermal growth factor receptor....
	Dermatomyositis Dermatomyositis Dermatomyositis is a connective-tissue disease related to Polymyositis that is characterized by inflammation of the muscles and the skin....	Bronchogenic carcinoma Breast carcinoma	Immunologic
	Leser-Trélat sign Leser-Trelat sign The Leser-Tr?lat sign is the explosive onset of multiple seborrheic keratoses , often with an inflammatory base. This can be an ominous sign of internal malignancy as part of a paraneoplastic syndrome....
	Necrolytic migratory erythema Necrolytic migratory erythema Necrolytic migratory erythema is a classical symptom observed in patients with glucagonoma and is present in 80% of cases....
	Sweet's syndrome
	Florid cutaneous papillomatosis Florid cutaneous papillomatosis Florid cutaneous papillomatosis is the sudden onset of numerous cutaneous papillomas that are indistinguishable from viral warts, and associated with underlying malignancy....
	Pyoderma gangrenosum Pyoderma gangrenosum Pyoderma gangrenosum is a disease that causes tissue to become necrosis, causing deep Skin ulcer that usually occur on the legs. When they occur, they can lead to chronic wounds....
Acquired generalized hypertrichosis Acquired generalized hypertrichosis Acquired generalized hypertrichosis is a generalized increase of hair density and length, an ominous sign in dermatology when there is no obvious drug-related explanation, being almost always a sign of an underlying malignancy or is a harbinger of one to develop in the near future....
Hematological	Granulocytosis Granulocytosis In medicine, granulocytosis is the presence in peripheral blood of an increased number of granulocytes, a category of white blood cells. Often, the word refers to an increased neutrophil granulocyte count, as neutrophils are the main granulocytes....		G-CSF Granulocyte colony-stimulating factor Granulocyte colony-stimulating factor is a colony-stimulating factor hormone. It is a glycoprotein, growth factor or cytokine produced by a number of different tissue to stimulate the bone marrow to produce granulocytes and stem cells....
	Polycythemia Polycythemia Polycythemia is a condition in which there is a net increase in the total number of blood cells, primarily red blood cells, in the body. The overproduction of red blood cells may be due to a primary process in the bone marrow , or it may be a reaction to chronically Hypoxia or, rarely, a malignancy....	Renal carcinoma Cerebellar hemangioma Hepatocellular carcinoma Hepatocellular carcinoma Hepatocellular carcinoma is a primary cancer of the liver. Most cases of HCC are secondary to either a viral hepatitis infection or cirrhosis ....	Erythropoietin Erythropoietin Erythropoietin, or its alternative erythropoetin or EPO, is a glycoprotein hormone that controls erythropoiesis, or red blood cell production....
	Trousseau sign Trousseau sign of malignancy The Trousseau sign of malignancy is a medical sign found in certain cancers that is associated with venous thrombosis and coagulation. It is also referred to as Trousseau syndrome and is distinct from the Trousseau sign of latent tetany....	Pancreatic carcinoma Bronchogenic carcinoma	Mucins that activate clotting, others
	Nonbacterial thrombotic endocarditis	Advanced cancers	Hypercoagulability
	Anemia Anemia Anemia or an?mia/anaemia is defined as a qualitative or quantitative deficiency of hemoglobin, a protein found inside red blood cells ....	Thymic neoplasms	Unknown
Others	Membranous glomerulonephritis Membranous glomerulonephritis Membranous glomerulonephritis is a slowly progressive disease of the kidney affecting mostly patients between ages of 30 and 50 years, usually Caucasian....	Various	Tumor antigen Tumor antigen Tumor antigen is a substance produced in tumor Cell that triggers an immune response in the Host . Tumor antigens are useful in identifying tumor cells and are potential candidates for use in cancer therapy.... s Immune complexes
Others	Tumor induced osteomalacia Tumor induced osteomalacia Tumor induced osteomalacia is an uncommon disorder resulting in increased renal phosphate excretion, hypophosphatemia and osteomalacia....

Neurological

A particularly devastating form of paraneoplastic syndromes is a group of disorders classified as paraneoplastic neurological disorders (PNDs). These paraneoplastic disorders affect the central or peripheral nervous system and they are degenerative. Symptoms of paraneoplastic neurological disorders may include ataxia

Ataxia

Ataxia is a neurology sign and symptom consisting of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of parts of the nervous system that coordinate movement, such as the cerebellum....
(difficulty with walking and balance), dizziness

Dizziness

Dizziness describes a number of subjective symptoms, which the patient may describe as feelings of lightheadedness, floating, wooziness, giddiness, confusion, disorientation or loss of balance....
, nystagmus

Nystagmus

Pathologic nystagmus is a form of involuntary eye movement. It is characterized by alternating smooth pursuit in one direction and saccadic movement in the other direction....
(rapid uncontrolled eye movements), difficulty swallowing, loss of muscle tone

Muscle tone

In physiology, medicine, and anatomy, muscle tone is the continuous and passive partial contraction of the muscles. It helps maintain neutral spine, and it declines during REM sleep....
, loss of fine motor coordination

Motor coordination

Motor coordination is among the most fundamental aspects of everyday life, seen in reaching for the morning cup of coffee to hitting the buttons on a clock to set your morning alarm....
, slurred speech, memory loss, vision problems, sleep disturbances, dementia

Dementia

Dementia is the progressive decline in cognition due to damage or disease in the body beyond what might be expected from normal aging. Although dementia is far more common in the geriatric population, it may occur in any stage of adulthood....
, seizures

Seizure

An epileptic seizure is a transient symptom of abnormal, excessive or synchronous neuronal activity in the brain. It can manifest as an alteration in mental state, tonic or clonic movements, convulsions, and various other psychic symptoms ....
, sensory loss in the limbs.

The most common cancers associated with paraneoplastic neurological disorders are breast

Breast cancer

Ovarian cancer

Lung cancer

Therapies to eliminate the underlying cancer such as chemotherapy, radiation and surgery, and
Therapies to reduce or slow neurological degeneration. Rapid diagnosis and treatment are critical for the patient to have the best chance of recovery. Since these disorders are relatively rare, few doctors have seen or treated PNDs. Therefore, it is important that PND patients consult with a specialist with experience in diagnosing and treating paraneoplastic neurological disorders.

Organizations

The (IPA) is an organization dedicated to providing support and information to those affected by paraneoplastic neurological disorders.

External links

- Paraneoplastic neurological autoantibodies, Birmingham UK

devoted to the paraneoplastic neurologic disorders