- Paraneoplastic syndrome
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, and they most commonly present with cancers of the lung, breast, ovaries or lymphatic system (a lymphoma). Sometimes the symptoms of paraneoplastic syndromes show before the diagnosis of a malignancy, which has been hypothesized to relate to the disease pathogenesis. In this paradigm, tumor cells express tissue-restricted antigens (such as neuronal proteins), triggering an anti-tumor immune response which may be partially or, rarely, completely effective in suppressing tumor growth and symptoms. Patients then come to clinical attention when this tumor immune response breaks immune tolerance and begins to attack the normal tissue expressing that (e.g. neuronal) protein.
Paraneoplastic syndromes can be divided into four 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 Ectopic ACTH and ACTH-like substance SIADH antidiuretic hormone  Hypercalcemia PTHrP (Parathyroid hormone-related protein), TGF-α, TNF, IL-1  Hypoglycemia Insulin or insulin-like substance or "big" IGF-II Carcinoid syndrome Serotonin, bradykinin  Polycythemia See hematological paraneoplastic syndromes Neurological  Lambert-Eaton myasthenic syndrome (LEMS)
- Small-cell lung cancer
Immunologic Paraneoplastic cerebellar degeneration
- ovarian cancer
- breast carcinoma
Encephalomyelitis inflammation of the brain and spinal cord Limbic encephalitis
- small-cell lung carcinoma
Brainstem encephalitis Paraneoplastic Opsoclonus (involving eye movement)-Myoclonus
- breast carcinoma
- ovarian carcinoma
- small-cell lung carcinoma
- neuroblastoma (in children)
Autoimmune reaction against the RNA-binding protein Nova-1 Anti-NMDA receptor encephalitis Autoimmune reaction against NMDA-receptor subunits Polymyositis Mucocutaneous  Acanthosis nigricans Dermatomyositis Immunologic  Leser-Trélat sign Necrolytic migratory erythema Glucagonoma Sweet's syndrome Florid cutaneous papillomatosis Pyoderma gangrenosum Acquired generalized hypertrichosis Hematological  Granulocytosis G-CSF Polycythemia Erythropoietin  Trousseau sign Mucins that activate clotting, others Nonbacterial thrombotic endocarditis
- Advanced cancers 
Hypercoagulability  Anemia
- Thymic neoplasms 
Unknown Others Membranous glomerulonephritis
- Various 
- Phosphaturic mesenchymal tumor
- FGF-23 (Fibroblast growth factor-23)
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; some are degenerative , 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.
Treatment options include:
- 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.
The International Paraneoplastic Association (IPA) is an organization dedicated to providing support and information to those affected by paraneoplastic neurological disorders.
- ^ Paraneoplastic Syndromes, 2011, Darnell & Posner
- ^ NINDS Paraneoplastic Syndromes Information Page National Institute of Neurological Disorders and Stroke
- ^ Darnell,R.B., DeAngelis,L.M. (1993), "Regression of small-cell lung carcinoma in patients with paraneoplastic neuronal antibodies", Lancet 341 (8836): 21-22, PMID 8093269
- ^ Roberts,W.K., Darnell,R.B. (2004), "Neuroimmunology of the paraneoplastic neurological degenerations", Current Opinions in Immunology 16 (5): 616-622, doi:10.1016/j.coi.2004.07.009, PMID 15342008
- ^ Albert,M.A., Darnell,R.B. (2004), "Paraneoplastic neurological degenerations: keys to tumour immunity", Nature Reviews Cancer 4 (1): 36-44, PMID 14708025
- ^ MeSH Paraneoplastic+endocrine+syndromes
- ^ a b c d e f g h i j k l m n o p q r s t u v w x y z aa ab ac ad ae af ag ah ai aj ak al am an ao Table 6-5 in: Mitchell, Richard Sheppard; Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson (2007). Robbins Basic Pathology. Philadelphia: Saunders. ISBN 1-4160-2973-7. 8th edition.
- ^ MeSH Nervous+system+paraneoplastic+syndromes
- ^ Buckanovich RJ, Posner JB, Darnell RB (1993). "Nova, the paraneoplastic Ri antigen, is homologous to an RNA-binding protein and is specifically expressed in the developing motor system". Neuron 11 (4): 657–72. doi:10.1016/0896-6273(93)90077-5. PMID 8398153.
- ^ Dalmau J, Tüzün E, Wu HY et al. (January 2007). "Paraneoplastic Anti–N-methyl-D-aspartate Receptor Encephalitis Associated with Ovarian Teratoma". Ann. Neurol. 61 (1): 25–36. doi:10.1002/ana.21050. PMC 2430743. PMID 17262855. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2430743.
- ^ Cohen PR, Kurzrock R (1997). "Mucocutaneous paraneoplastic syndromes". Semin. Oncol. 24 (3): 334–59. PMID 9208889.
- ^ Staszewski H (1997). "Hematological paraneoplastic syndromes". Semin. Oncol. 24 (3): 329–33. PMID 9208888.
- ^ Zadik Y, Nitzan DW (October 2011). "Tumor induced osteomalacia: A forgotten paraneoplastic syndrome?". Oral Oncol. doi:10.1016/j.joms.2011.02.124. PMID 21985764. http://www.sciencedirect.com/science/article/pii/S1368837511008268.
- ^ Rees JH (2004). "PARANEOPLASTIC SYNDROMES: WHEN TO SUSPECT, HOW TO CONFIRM, AND HOW TO MANAGE". J. Neurol. Neurosurg. Psychiatr. 75 Suppl 2 (Suppl 2): ii43–50. doi:10.1136/jnnp.2004.040378. PMC 1765657. PMID 15146039. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1765657.
- ^ Darnell RB, Posner JB (2006). "Paraneoplastic syndromes affecting the nervous system". Semin Oncol 33 (3): 270–98. doi:10.1053/j.seminoncol.2006.03.008. PMID 16769417.
- Neuroimmunology, The Medical School, Birmingham University - Paraneoplastic neurological autoantibodies, Birmingham UK
- IPA website
- Rockefeller University clinical research laboratory devoted to the paraneoplastic neurologic disorders
Pathology: Tumor, Neoplasm, Cancer, and Oncology (C00–D48, 140–239) ConditionsMalignant progressionTopographyHistologyOtherPrecancerous condition · Paraneoplastic syndrome Staging/grading Carcinogenesis Misc.
tumr, epon, para
Paraneoplastic syndromes Endocrine Hematological Neurological MusculoskeletalDermatomyositis · Hypertrophic osteoarthropathy Mucocutaneous
papulosquamous: Acanthosis nigricans · Acquired ichthyosis · Acrokeratosis paraneoplastica of Bazex · Extramammary Paget's disease · Florid cutaneous papillomatosis · Leser-Trélat sign · Pityriasis rotunda · Tripe palmsother/ungrouped: Febrile neutrophilic dermatosis · Pyoderma gangrenosum · Paraneoplastic pemphigus
tumr, epon, para
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