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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/12/18 10:33:38」(JST)

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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.^[1] 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).^[2] 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^[3] in suppressing tumor growth and symptoms.^[4]^[5] 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.

Classification[edit]

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^[6]
	Cushing syndrome	small-cell lung cancer^[7] Pancreatic carcinoma^[7] Neural tumors^[7] Thymoma	Ectopic ACTH and ACTH-like substance
	SIADH	small-cell lung cancer^[7] CNS malignancies^[7]	antidiuretic hormone^[7]
	Hypercalcemia	Lung cancer (typically squamous cell)^[7] Breast carcinoma^[7] Renal carcinoma^[7] Multiple myeloma (may occur independent of osteolytic lesions) Adult T cell leukemia/lymphoma^[7] Ovarian carcinoma^[7]	PTHrP (Parathyroid hormone-related protein), TGF-α, TNF, IL-1^[7]
	Hypoglycemia	Fibrosarcoma^[7] Other mesenchymal sarcomas^[7] Insulinoma Hepatocellular carcinoma^[7]	Insulin or insulin-like substance^[7] or "big" IGF-II
	Carcinoid syndrome	Bronchial adenoma (carcinoid type)^[7] Pancreatic carcinoma^[7] Gastric carcinoma^[7]	Serotonin, bradykinin^[7]
	Polycythemia		See hematological paraneoplastic syndromes
	Hyperaldosteronism	Adrenal adenoma / Conn's syndrome Non-Hodgkin's lymphoma ovarian carcinoma pulmonary	Aldosterone^[8]
Neurological^[9]	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	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^[10]
	Anti-NMDA receptor encephalitis	Teratoma^[11]	Autoimmune reaction against NMDA-receptor subunits
	Polymyositis	Non-Hodgkin lymphoma lung cancers bladder cancers
Mucocutaneous^[12]	Acanthosis nigricans	Gastric carcinoma^[7] Lung carcinoma^[7] Uterine carcinoma^[7]	Immunologic^[7] Secretion of EGF
	Dermatomyositis	Bronchogenic carcinoma^[7] Breast carcinoma^[7] ovarian cancer pancreatic cancer stomach cancer colorectal cancer Non-hodgkin's lymphoma ^[13]	Immunologic^[7]
	Leser-Trélat sign
	Necrolytic migratory erythema	Glucagonoma
	Sweet's syndrome
	Florid cutaneous papillomatosis
	Pyoderma gangrenosum
	Acquired generalized hypertrichosis
Hematological^[14]	Granulocytosis		G-CSF
	Polycythemia	Renal carcinoma^[7] Cerebellar hemangioma^[7] Hepatocellular carcinoma^[7]	Erythropoietin^[7]
	Trousseau sign	Pancreatic carcinoma^[7] Bronchogenic carcinoma^[7]	Mucins that activate clotting,^[7] others
	Nonbacterial thrombotic endocarditis	Advanced cancers^[7]	Hypercoagulability^[7]
	Anemia	Thymic neoplasms^[7]	Unknown^[7]
Others	Membranous glomerulonephritis	Various^[7]	Tumor antigens^[7] Immune complexes^[7]
	Tumor-induced osteomalacia	Hemangiopericytoma Phosphaturic mesenchymal tumor^[15]	FGF-23 (Fibroblast growth factor-23)
	Stauffer syndrome	renal cell carcinoma
	Neoplastic fever ^[16]

Neurological[edit]

A particularly devastating form of paraneoplastic syndromes is a group of disorders classified as paraneoplastic neurological disorders (PNDs).^[17] These paraneoplastic disorders affect the central or peripheral nervous system; some are degenerative,^[18] 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:

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.

References[edit]

^ 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 Opinion 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
^ Paraneoplastic endocrine syndromes at the US National Library of Medicine Medical Subject Headings (MeSH)
^ ^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.
^ Mulatero P, Rabbia F, Veglio F. "Paraneoplastic hyperaldosteronism associated with non-Hodgkin's lymphoma.", New England Journal of Medicine. 2001 May 17;344(20):1558-9. Retrieved on 29 August 2012.
^ Nervous system paraneoplastic syndromes at the US National Library of Medicine Medical Subject Headings (MeSH)
^ 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. Cite uses deprecated parameters (help)
^ Cohen PR, Kurzrock R (1997). "Mucocutaneous paraneoplastic syndromes". Semin. Oncol. 24 (3): 334–59. PMID 9208889.
^ Hill, Catherine L; Zhang, Yuqing; Sigurgeirsson, Bardur; Pukkala, Eero; Mellemkjaer, Lene; Airio, Antti; Evans, Stephen R; Felson, David T (2001). "Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study". The Lancet 357 (9250): 96. doi:10.1016/S0140-6736(00)03540-6.
^ 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 48 (2): e9–10. doi:10.1016/j.oraloncology.2011.09.011. PMID 21985764. Cite uses deprecated parameters (help)
^ Zell JA, Chang JC. Neoplastic fever: a neglected paraneoplastic syndrome. PMID 15864658
^ 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.
^ 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.

External links[edit]

http://www.antibodypatterns.com/hu.php

UpToDate Contents

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1. 末梢神経と筋肉に影響を与える腫瘍随伴性症候群 paraneoplastic syndromes affecting peripheral nerve and muscle
2. 神経系の腫瘍随伴性症候群の概要 overview of paraneoplastic syndromes of the nervous system
3. 脊髄および後根神経節に影響を与える腫瘍随伴性症候群 paraneoplastic syndromes affecting the spinal cord and dorsal root ganglia
4. 免疫誘導性神経障害 immune mediated neuropathies
5. 視神経症 optic neuropathies

English Journal

Efficacy of lenalidomide plus dexamethasone for POEMS syndrome relapsed after autologous peripheral stem-cell transplantation.

Vannata B, Laurenti L, Chiusolo P, Sorà F, Balducci M, Sabatelli M, Luigetti M, Giannotta C, De Stefano V, Leone G, Sica S.SourceDepartment of Hematology, Catholic University of the Sacred Heart, Hospital A. Gemelli, Largo A. Gemelli 8, Rome, Italy.
American journal of hematology.Am J Hematol.2012 Jun;87(6):641-2. doi: 10.1002/ajh.23195. Epub 2012 Apr 10.
POEMS syndrome is a rare paraneoplastic condition associated to an underlying plasmacellular dyscrasia. The pathogenesis of POEMS is poorly understood, but overproduction of VEGF, probably secreted by clonal plasma cells, is thought to be responsible for the signs and symptoms of the syndrome, and i
PMID 22488443

Paraneoplastic Isaacs' syndrome: a case series and review of the literature.

Rana SS, Ramanathan RS, Small G, Adamovich B.SourceAllegheny General Hospital, Department of Neurology, Drexel University College of Medicine, Allegheny Campus, Pittsburgh, PA 15212, USA. srana@wpahs.org
Journal of clinical neuromuscular disease.J Clin Neuromuscul Dis.2012 Jun;13(4):228-33. doi: 10.1097/CND.0b013e318246197d.
OBJECTIVE: Isaacs' syndrome is a rare disease resulting from hyperexcitability of peripheral nerves causing continuous muscle fiber activity characterized by muscle twitching and stiffness at rest and delayed muscle relaxation after voluntary contraction. Our objective was to discuss the relationshi
PMID 22622168

Japanese Journal

四肢感覚障害を呈した傍腫瘍性神経症侯群の 2 症例に対するリハビリテーションの経験

河田理絵子,青柳陽一郎,平岡崇,山中崇,国安勝司,椿原彰夫
The Japanese Journal of Rehabilitation Medicine 46(9), 572-576, 2009
… We report two cases of paraneoplastic syndrome with sensory polyneuropathy. …
NAID 130000145915

神経学的所見と病理所見

小池春樹,祖父江元
末梢神経 = Peripheral nerve 18(2), 130-134, 2007-12-01
NAID 10027267258

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リンク元	「腫瘍随伴性多発ニューロパチー」「傍腫瘍性ポリニューロパチー」「傍腫瘍性多発ニューロパチー」「腫瘍随伴性ポリニューロパチー」「腫瘍随伴性多発ニューロパシー」
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