Paraneoplastic syndrome
Classification and external resources
DiseasesDB	2064
eMedicine	med/1747
MeSH	D010257

A paraneoplastic syndrome is a disease or symptom that is the consequence of the presence of cancer in the body, but is not due to the local presence of cancer cells. These phenomena are mediated by humoral factors (by hormones or cytokines) excreted by tumor cells or by an immune response against the tumor. Paraneoplastic syndromes are typical among middle aged to older patients (as are all cancers), and they most commonly present with cancers of the lung, breast, ovaries or lymphatic system (a lymphoma).^[1] Sometimes the symptoms of paraneoplastic syndromes show even before the diagnosis of a malignancy.

Contents 1 Classification 2 Neurological 3 Organizations 4 References 5 External links

[edit] 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[2]
	Cushing syndrome	small-cell lung cancer [3] Pancreatic carcinoma [3] Neural tumors [3] thymoma	Ectopic ACTH and ACTH-like substance
	SIADH	small-cell lung cancer [3] CNS malignancies [3]	Atrial natriuretic hormone or atrial natriuretic hormones [3]
	Hypercalcemia	lung cancer (typically squamous cell) [3] breast carcinoma [3] Renal carcinoma [3] Adult T cell leukemia/lymphoma [3] Ovarian carcinoma [3]	PTHrP (Parathyroid hormone-related protein), TGF-α, TNF, IL-1 [3]
	Hypoglycemia	Fibrosarcoma [3] Other mesenchymal sarcomas [3] Hepatocellular carcinoma [3]	Insulin or insulin-like substance[3]
	Carcinoid syndrome	Bronchial adenoma (carcinoid type) [3] Pancreatic carcinoma [3] Gastric carcinoma [3]	Serotonin, bradykinin [3]
	Polycythemia	See hematological paraneoplastic syndromes
Neurological [4]	Lambert-Eaton myasthenic syndrome (LEMS)	small-cell lung cancer	Immunologic
	Paraneoplastic cerebellar degeneration	lung ovarian cancer breast carcinoma
	Encephalomyelitis		inflammation of the brain and spinal cord
	Limbic encephalitis
	Brainstem encephalitis
	Opsoclonus (involving eye movement)
	Encephalitis	Teratoma[5]
	Polymyositis
Mucocutaneous [6]	Acanthosis nigricans	Gastric carcinoma [3] Lung carcinoma [3] Uterine carcinoma [3]	Immunologic[3] Secretion of EGF
	Dermatomyositis	Bronchogenic carcinoma [3] Breast carcinoma [3]	Immunologic [3]
	Leser-Trélat sign
	Necrolytic migratory erythema	Glucagonoma
	Sweet's syndrome
	Florid cutaneous papillomatosis
	Pyoderma gangrenosum
	Acquired generalized hypertrichosis
Hematological [7]	Granulocytosis		G-CSF
	Polycythemia	Renal carcinoma [3] Cerebellar hemangioma [3] Hepatocellular carcinoma [3]	Erythropoietin [3]
	Trousseau sign	Pancreatic carcinoma [3] Bronchogenic carcinoma [3]	Mucins that activate clotting[3], others
	Nonbacterial thrombotic endocarditis	Advanced cancers [3]	Hypercoagulability [3]
	Anemia	Thymic neoplasms [3]	Unknown[3]
Others	Membranous glomerulonephritis	Various [3]	Tumor antigens [3] Immune complexes [3]
	Tumor-induced osteomalacia	Hemangiopericytoma	FGF-23 (Fibroblast growth factor-23)

[edit] Neurological

A particularly devastating form of paraneoplastic syndromes is a group of disorders classified as paraneoplastic neurological disorders (PNDs).^[8] These paraneoplastic disorders affect the central or peripheral nervous system; some are degenerative ^[9], though others (such as LEMS) may improve with treatment of the condition or the tumour. Symptoms of paraneoplastic neurological disorders may include ataxia (difficulty with walking and balance), dizziness, nystagmus (rapid uncontrolled eye movements), difficulty swallowing, loss of muscle tone, loss of fine motor coordination, slurred speech, memory loss, vision problems, sleep disturbances, dementia, seizures, sensory loss in the limbs.

The most common cancers associated with paraneoplastic neurological disorders are breast, ovarian and lung cancer, but many other cancers can produce paraneoplastic symptoms as well.

Treatment options include:

1. Therapies to eliminate the underlying cancer such as chemotherapy, radiation and surgery, and
2. 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.

[edit] Organizations

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

[edit] References

[edit] External links

• Neuroimmunology, The Medical School, Birmingham University - Paraneoplastic neurological autoantibodies, Birmingham UK
• http://www.antibodypatterns.com/hu.php
• IPA website
• Rockefeller University clinical research laboratory devoted to the paraneoplastic neurologic disorders

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v • d • e Paraneoplastic syndromes Endocrine Hypercalcaemia · SIADH · Zollinger-Ellison syndrome  · Cushing's syndrome Hematological Granulocytosis Neurological Paraneoplastic cerebellar degeneration  · Encephalomyelitis · Limbic encephalitis · Opsoclonus · Polymyositis · Transverse myelitis · Lambert-Eaton myasthenic syndrome Mucocutaneous Dermatomyositis  · Leser-Trélat sign · Acanthosis nigricans · Florid cutaneous papillomatosis · Necrolytic migratory erythema · Sweet's syndrome · Pyoderma gangrenosum