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a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"

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(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/09/22 18:00:16」(JST)

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A paraneoplastic syndrome is a syndrome (a set of signs and symptoms) that is the consequence of cancer in the body but that, unlike mass effect, is not due to the local presence of cancer cells.^[1] In contrast, these phenomena are mediated by humoral factors (such as 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 (e.g., 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.

The abbreviation PNS is sometimes used for paraneoplastic syndrome, although it is used more often to refer to the peripheral nervous system.

Classification

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]
	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 cancer ovarian cancer breast carcinoma Hodgkin's lymphoma
	Encephalomyelitis		inflammation of the brain and spinal cord
	Limbic encephalitis	small-cell lung carcinoma
	Brainstem encephalitis
	Opsoclonus myoclonus ataxia syndrome	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 cancer bladder cancer
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 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]	Fibroblast growth factor 23
	Stauffer syndrome	Renal cell carcinoma
	Neoplastic fever ^[16]

Neurological

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 tumor. 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

^ 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, doi:10.1016/0140-6736(93)92485-c, 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, doi:10.1038/nrc1255, 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.
^ 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): 96–100. doi:10.1016/S0140-6736(00)03540-6. PMID 11197446.
^ 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.
^ Zell JA, Chang JC (November 2005). "Neoplastic fever: a neglected paraneoplastic syndrome". 13 (11): 870–7. doi:10.1007/s00520-005-0825-4. 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.

UpToDate Contents

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1. 神経系の腫瘍随伴性症候群の概要 overview of paraneoplastic syndromes of the nervous system
2. 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
3. 肺癌の危険因子、病理、および臨床症状の概要 overview of the risk factors pathology and clinical manifestations of lung cancer
4. 腫瘍随伴性および自己免疫性脳炎 paraneoplastic and autoimmune encephalitis
5. 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

English Journal

Is there a paraneoplastic ALS?

Corcia P1, Gordon PH, Camdessanche JP.
Amyotrophic lateral sclerosis & frontotemporal degeneration.Amyotroph Lateral Scler Frontotemporal Degener.2015 Jun;16(3-4):252-7. doi: 10.3109/21678421.2014.965178. Epub 2014 Oct 6.
Our objective was to examine the strength of evidence in support of the paraneoplastic syndrome (PNS) as one cause of ALS and, if the association appears more likely than chance, determine which features of ALS imply concurrent malignancy. We reviewed the literature on concurrent ALS and neoplasia a
PMID 25285651

18F-FDG PET/CT and MRI Findings in a Patient With Anti-GABAB Receptor Encephalitis.

Su M1, Xu D, Tian R.
Clinical nuclear medicine.Clin Nucl Med.2015 Jun;40(6):515-7. doi: 10.1097/RLU.0000000000000676.
Autoimmune synaptic encephalitis can occur as paraneoplastic neurological syndromes, which are dysfunctions of the nervous system in cancer patients. One such rare but treatable form is associated with GABAB (γ-aminobutyric acid-B) receptor antibody. We report a 55-year-old man with small cell lung
PMID 25608169

Tumor-Induced Osteomalacia: an Up-to-Date Review.

Hautmann AH1, Hautmann MG, Kölbl O, Herr W, Fleck M.
Current rheumatology reports.Curr Rheumatol Rep.2015 Jun;17(6):512. doi: 10.1007/s11926-015-0512-5.
Tumor-induced osteomalacia (TIO) is a paraneoplastic syndrome resulting in renal phosphate wasting and decreased bone mineralization. TIO is usually induced by small, slowly growing tumors of mesenchymal origin (phosphaturic mesenchymal tumor mixed connective tissue variant [PMTMCT]). Nonspecific sy
PMID 25900190

Japanese Journal

TおよびNK細胞リンパ腫のdiagnostic clues (特集血液疾患の治療前必須検査とその解釈)

磯部泰司
血液内科 = Hematology 70(5), 569-574, 2015-05
NAID 40020490763

症例報告傍腫瘍性神経症候群を呈した腹膜癌の1例

松野孝幸,市川剛,永田瑞穂 [他]
東京産科婦人科学会会誌 64(1), 70-74, 2015-01
NAID 40020542147

Paraneoplastic Syndrome を合併した Rhabdoid 型有棘細胞癌の 1 例

水柿典子,高橋宏征,加賀谷真起子 [他],高橋博之
西日本皮膚科 77(3), 225-229, 2015
… 経過は順調であったが，化学療法 3 クール施行後に皮下および多臓器転移が急速に出現し，また，再度著明な白血球と血小板増加および高カルシウム血症，副甲状腺ホルモン関連蛋白と GCSF 高値を伴う paraneoplastic syndrome を併発し，初診より 5 カ月後に永眠した。 …
NAID 130005101060

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リンク元	「腫瘍随伴症候群」「傍腫瘍症候群」
拡張検索	「nervous system paraneoplastic syndrome」
関連記事	「paraneoplastic」「syndrome」

「腫瘍随伴症候群」

Paraneoplastic syndrome
Classification and external resources
Specialty	Oncology
DiseasesDB	2064
eMedicine	med/1747
MeSH	D010257
	[edit on Wikidata]

　　[★]

英: paraneoplastic syndrome
同: 傍腫瘍性症候群、腫瘍随伴症状 paraneoplastic symptom
関: 腫瘍

[show details]

傍腫瘍性症状 : 6 件
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傍腫瘍性症候群 : 約 19,800 件
腫瘍随伴症候群 : 約 14,500 件

「傍腫瘍症候群」

　　[★]

英: paraneoplastic syndrome

「nervous system paraneoplastic syndrome」

　　[★]

傍腫瘍性神経症候群、腫瘍随伴神経症候群、神経性腫瘍随伴症候群

関: paraneoplastic encephalomyelitis

「paraneoplastic」

　　[★]

adj.

腫瘍随伴の、傍腫瘍性の、傍腫瘍の

「syndrome」

　　[★]

n.

症候群

[1] Paraneoplastic Syndromes, 2011, Darnell & Posner

[2] NINDS Paraneoplastic Syndromes Information Page National Institute of Neurological Disorders and Stroke

[3] Darnell,R.B.; DeAngelis,L.M. (1993), "Regression of small-cell lung carcinoma in patients with paraneoplastic neuronal antibodies", Lancet, 341 (8836): 21–22, doi:10.1016/0140-6736(93)92485-c, PMID 8093269

[4] 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

[5] Albert,M.A.; Darnell,R.B. (2004), "Paraneoplastic neurological degenerations: keys to tumour immunity", Nature Reviews Cancer, 4 (1): 36–44, doi:10.1038/nrc1255, PMID 14708025

[6] Paraneoplastic endocrine syndromes at the US National Library of Medicine Medical Subject Headings (MeSH)

[Kumar6-5-7] ^ ^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.

[8] 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.

[9] Nervous system paraneoplastic syndromes at the US National Library of Medicine Medical Subject Headings (MeSH)

[10] 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.

[pmid17262855-11] 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.

[pmid9208889-12] Cohen PR, Kurzrock R (1997). "Mucocutaneous paraneoplastic syndromes". Semin. Oncol. 24 (3): 334–59. PMID 9208889.

[Catherine_L_Hill-13] 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): 96–100. doi:10.1016/S0140-6736(00)03540-6. PMID 11197446.

[pmid9208888-14] Staszewski H (1997). "Hematological paraneoplastic syndromes". Semin. Oncol. 24 (3): 329–33. PMID 9208888.

[TIO-15] 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.

[16] Zell JA, Chang JC (November 2005). "Neoplastic fever: a neglected paraneoplastic syndrome". 13 (11): 870–7. doi:10.1007/s00520-005-0825-4. PMID 15864658.

[17] 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.

[18] 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.

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paraneoplastic syndrome