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| 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.[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.
Contents
- 1 Classification
- 2 Neurological
- 3 References
- 4 External links
Classification[edit source | 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) |
|
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 |
|
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 |
|
Hypercoagulability[7] |
| Anemia |
|
Unknown[7] |
| Others |
Membranous glomerulonephritis |
|
- Tumor antigens[7]
- Immune complexes[7]
|
| Tumor-induced osteomalacia |
- Hemangiopericytoma
- Phosphaturic mesenchymal tumor[15]
|
- FGF-23 (Fibroblast growth factor-23)
|
| Stauffer syndrome |
|
|
| Neoplastic fever [16] |
|
|
Neurological[edit source | 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 source | 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 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
- ^ 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. 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.
- ^ 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 source | edit]
- http://www.antibodypatterns.com/hu.php
|
Pathology: Tumor, Neoplasm, Cancer, and Oncology (C00–D48, 140–239)
|
|
| Conditions |
| Benign tumors |
- Hyperplasia
- Cyst
- Pseudocyst
- Hamartoma
|
|
| Malignant progression |
- Dysplasia
- Carcinoma in situ
- Cancer
- Metastasis
|
|
| Topography |
- Head/Neck (Oral, Nasopharyngeal)
- Digestive system
- Respiratory system
- Bone
- Skin
- Blood
- Urogenital
- Nervous system
- Endocrine system
|
|
| Histology |
- Carcinoma
- Sarcoma
- Blastoma
- Papilloma
- Adenoma
|
|
| Other |
- Precancerous condition
- Paraneoplastic syndrome
|
|
|
| Staging/grading |
- TNM
- Ann Arbor
- Prostate cancer staging
- Gleason Grading System
- Dukes classification
|
|
| Carcinogenesis |
- Cancer cell
- Carcinogen
- Tumor suppressor genes/oncogenes
- Clonally transmissible cancer
- Oncovirus
- Cancer bacteria
|
|
| Misc. |
- Research
- List of oncology-related terms
- History
- Cancer pain
- Cancer and nausea
|
|
|
|
|
|
Paraneoplastic syndromes
|
|
| Endocrine |
Hypercalcaemia · SIADH · Zollinger–Ellison syndrome · Cushing's syndrome
|
|
| Hematological |
Granulocytosis · Multicentric reticulohistiocytosis
|
|
| Neurological |
Paraneoplastic cerebellar degeneration · Encephalomyelitis · Limbic encephalitis · Opsoclonus · Polymyositis · Transverse myelitis · Lambert–Eaton myasthenic syndrome · Anti-NMDA receptor encephalitis
|
|
| Musculoskeletal |
Dermatomyositis · Hypertrophic osteoarthropathy
|
|
| Mucocutaneous |
reactive erythema: Erythema gyratum repens · Necrolytic migratory erythema
papulosquamous: Acanthosis nigricans · Acquired ichthyosis · Acrokeratosis paraneoplastica of Bazex · Extramammary Paget's disease · Florid cutaneous papillomatosis · Leser-Trélat sign · Pityriasis rotunda · Tripe palms
other/ungrouped: Febrile neutrophilic dermatosis · Pyoderma gangrenosum · Paraneoplastic pemphigus
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UpToDate Contents
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English Journal
- Paraneoplastic syndromes in rheumatology.
- Manger B, Schett G.
- Nature reviews. Rheumatology.Nat Rev Rheumatol.2014 Nov;10(11):662-670. doi: 10.1038/nrrheum.2014.138. Epub 2014 Aug 19.
- For patients that present with musculoskeletal symptoms, diagnostic procedures carried out by physicians and rheumatologists are primarily aimed at confirming or excluding the occurrence of primary rheumatic diseases. Another important trigger for musculoskeletal disease, however, is the presence of
- PMID 25136782
- Isolated oculomotor nerve palsy as a paraneoplastic manifestation of gastric diffuse large B-cell lymphoma: A case report.
- Yan SY1, Peng YJ2, Lin CS3, Peng GS1, Chang PY4.
- Oncology letters.Oncol Lett.2014 Nov;8(5):1983-1985. Epub 2014 Aug 19.
- Isolated oculomotor nerve palsy (ONP) is rare. The major causes are aneurysm of the posterior communicating artery, microvascular ischemia, neoplasm, inflammation and trauma. The present study reports the case of a 72-year-old female with left isolated pupil-sparing ONP and severe anemia as the init
- PMID 25295082
- Paraneoplastic secondary hypertension due to a renin-secreting desmoplastic small round cell tumor: A case report.
- Lee HJ, Hyun JS, Jang HS, Sul H, Park SG.
- Oncology letters.Oncol Lett.2014 Nov;8(5):1986-1992. Epub 2014 Aug 18.
- Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy with a poor outcome that occurs in adolescents and young adults; <200 cases of DSRCT have been reported. Renin-producing tumors are also rare and cases of extrarenal renin-producing tumors are even rarer. The present
- PMID 25289084
Japanese Journal
- 亀井 潤,京野 陽子,山田 幸央 [他]
- 泌尿器科紀要 58(7), 361-364, 2012-07
- A 32-year-old man, who had been diagnosed with left testicular tumor and treated surgically at 30 years old, was diagnosed with recurrence of the testicular tumor by the elevation of tumor markers and …
- NAID 40019362594
- 症例 抗CV2/CRMP5抗体が陽性であった統合失調症の1例
Related Links
- Paraneoplastic syndromes, defined in this book as "disorders caused by cancer, but not a direct result of cancer invasion of the affected organ or tissue", once believed to be rare and esoteric neurologic disorders have assumed ...
- paraneoplastic /para·neo·plas·tic/ (-ne″o-plas´tik) pertaining to changes produced in tissue remote from a tumor or its metastases. paraneoplastic auxiliary to neoplasia. paraneoplastic syndrome a collective term for disorders arising ...
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