Paraneoplastic syndrome 1

Paraneoplastic syndrome
Paraneoplastic
syndrome
Classification and external resources

DiseasesDB [1]
2064

eMedicine [2]
med/1747

MeSH [3]
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).[4] Sometimes the symptoms of paraneoplastic syndromes
show even before the diagnosis of a malignancy.

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

[5]
Endocrine Cushing syndrome [6] Ectopic ACTH and ACTH-like substance
• small-cell lung cancer
[6]
• Pancreatic carcinoma
[6]
• Neural tumors
• thymoma

SIADH [6] [6]

• small-cell lung cancer antidiuretic hormone
[6]
• CNS malignancies

Hypercalcemia • lung cancer (typically squamous PTHrP (Parathyroid hormone-related protein),

[6] [6]
cell) TGF-α, TNF, IL-1
[6]
• breast carcinoma
[6]
• Renal carcinoma
• Adult T cell
[6]
leukemia/lymphoma
[6]
• Ovarian carcinoma

Hypoglycemia [6] [6]

• Fibrosarcoma Insulin or insulin-like substance
• Other mesenchymal sarcomas
[6]
[6]
• Hepatocellular carcinoma

Carcinoid syndrome [6]

• Bronchial adenoma (carcinoid Serotonin, bradykinin
[6]
type)
[6]
• Pancreatic carcinoma
[6]
• Gastric carcinoma

Polycythemia See hematological paraneoplastic syndromes

Paraneoplastic syndrome 2

[7] Lambert-Eaton myasthenic • small-cell lung cancer Immunologic

Neurological
syndrome (LEMS)

Paraneoplastic cerebellar • lung

degeneration • ovarian cancer
• breast carcinoma

Encephalomyelitis inflammation of the brain and spinal cord

Limbic encephalitis • small-cell lung carcinoma

Brainstem encephalitis

Opsoclonus (involving eye

movement)

Anti-NMDA receptor encephalitis [8] Autoimmune reaction against NMDA-receptor

• Teratoma
subunits

Polymyositis

Acanthosis nigricans [6] [6]

Mucocutaneous • Gastric carcinoma • Immunologic
[9] [6]
• Lung carcinoma • Secretion of EGF
[6]
• Uterine carcinoma

Dermatomyositis [6] [6]

• Bronchogenic carcinoma Immunologic
[6]
• Breast carcinoma

Leser-Trélat sign

Necrolytic migratory erythema Glucagonoma

Sweet's syndrome

Florid cutaneous papillomatosis

Pyoderma gangrenosum

Acquired generalized
hypertrichosis

Granulocytosis G-CSF
Hematological
[10]
Polycythemia [6] [6]
• Renal carcinoma Erythropoietin
[6]
• Cerebellar hemangioma
[6]
• Hepatocellular carcinoma

Trousseau sign [6] [6]

• Pancreatic carcinoma Mucins that activate clotting , others
[6]
• Bronchogenic carcinoma

Nonbacterial thrombotic [6] [6]

• Advanced cancers Hypercoagulability
endocarditis

Anemia [6] [6]

• Thymic neoplasms Unknown

Others Membranous glomerulonephritis [6] [6]

• Various • Tumor antigens
[6]
• Immune complexes

Tumor-induced osteomalacia • Hemangiopericytoma • FGF-23 (Fibroblast growth factor-23)

Stauffer syndrome • renal cell carcinoma

Paraneoplastic syndrome 3

Neurological
A particularly devastating form of paraneoplastic syndromes is a group of disorders classified as paraneoplastic
neurological disorders (PNDs).[11] These paraneoplastic disorders affect the central or peripheral nervous system;
some are degenerative [12] , 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.

Organizations
The International Paraneoplastic Association [13] (IPA) is an organization dedicated to providing support and
information to those affected by paraneoplastic neurological disorders.

External links
• Neuroimmunology, The Medical School, Birmingham University [14] - Paraneoplastic neurological
autoantibodies, Birmingham UK
• http://www.antibodypatterns.com/hu.php
• IPA website [15]
• Rockefeller University clinical research laboratory [16] devoted to the paraneoplastic neurologic disorders

References
[1] http:/ / www. diseasesdatabase. com/ ddb2064. htm
[2] http:/ / www. emedicine. com/ med/ topic1747. htm
[3] http:/ / www. nlm. nih. gov/ cgi/ mesh/ 2010/ MB_cgi?field=uid& term=D010257
[4] NINDS Paraneoplastic Syndromes Information Page (http:/ / www. ninds. nih. gov/ disorders/ paraneoplastic/ paraneoplastic. htm) National
Institute of Neurological Disorders and Stroke
[5] MeSH Paraneoplastic+endocrine+syndromes (http:/ / www. nlm. nih. gov/ cgi/ mesh/ 2009/ MB_cgi?mode=& term=Paraneoplastic+
endocrine+ syndromes)
[6] 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.
[7] MeSH Nervous+system+paraneoplastic+syndromes (http:/ / www. nlm. nih. gov/ cgi/ mesh/ 2009/ MB_cgi?mode=& term=Nervous+
system+ paraneoplastic+ syndromes)
[8] Dalmau J, Tüzün E, Wu HY, et al. (January 2007). "Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian
teratoma" (http:/ / www. pubmedcentral. nih. gov/ articlerender. fcgi?tool=pmcentrez& artid=2430743). Ann. Neurol. 61 (1): 25–36.
doi:10.1002/ana.21050. PMID 17262855. PMC 2430743.
[9] Cohen PR, Kurzrock R (1997). "Mucocutaneous paraneoplastic syndromes". Semin. Oncol. 24 (3): 334–59. PMID 9208889.
[10] Staszewski H (1997). "Hematological paraneoplastic syndromes". Semin. Oncol. 24 (3): 329–33. PMID 9208888.
[11] Rees JH (2004). "Paraneoplastic syndromes: when to suspect, how to confirm, and how to manage" (http:/ / www. pubmedcentral. nih. gov/
articlerender. fcgi?tool=pmcentrez& artid=1765657). J. Neurol. Neurosurg. Psychiatr. 75 Suppl 2: ii43–50. doi:10.1136/jnnp.2004.040378.
PMID 15146039. PMC 1765657.
Paraneoplastic syndrome 4

[12] 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.
[13] http:/ / www. paraneoplastic. org/
[14] http:/ / www. ii. bham. ac. uk/ clinicalimmunology/ Neuroimmunology/
[15] http:/ / www. paraneoplastic. org
[16] http:/ / www. rockefeller. edu/ labheads/ darnellr/
Article Sources and Contributors 5

Article Sources and Contributors

Paraneoplastic syndrome  Source: http://en.wikipedia.org/w/index.php?oldid=373131508  Contributors: Ais523, Altenmann, Arcadian, Carlifenkm, Cogpf, CopperKettle, Cyberaward,
DO11.10, Darnelr, Dbenbenn, De Hoo, Emilymg, Freak in the bunnysuit, Fuzbaby, Galaxiaad, Gweslock, J04n, Jfdwolff, Kauczuk, Madhero88, Mikael Häggström, Neurotip, PFHLai, Rich
Farmbrough, Rmenikoff, SUSHRUTA, Sbmehta, Shaktal, Szalzala, Wendell, WhatamIdoing, Wouterstomp, Zach99998, 41 anonymous edits

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