Paraneoplastic syndrome


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[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

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 Ectopic ACTH and ACTH-like substance
SIADH antidiuretic hormone [7]
Hypercalcemia PTHrP (Parathyroid hormone-related protein), TGF-α, TNF, IL-1 [7]
Hypoglycemia Insulin or insulin-like substance[7] or "big" IGF-II
Carcinoid syndrome Serotonin, bradykinin [7]
Polycythemia See hematological paraneoplastic syndromes
Neurological [8] 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[9]
Anti-NMDA receptor encephalitis Autoimmune reaction against NMDA-receptor subunits
Polymyositis
Mucocutaneous [11] Acanthosis nigricans
  • Gastric carcinoma [7]
  • Lung carcinoma [7]
  • Uterine carcinoma [7]
  • Immunologic[7]
  • Secretion of EGF
Dermatomyositis
  • Bronchogenic carcinoma [7]
  • Breast carcinoma [7]
Immunologic [7]
Leser-Trélat sign
Necrolytic migratory erythema Glucagonoma
Sweet's syndrome
Florid cutaneous papillomatosis
Pyoderma gangrenosum
Acquired generalized hypertrichosis
Hematological [12] Granulocytosis G-CSF
Polycythemia 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
Tumor-induced osteomalacia
  • Hemangiopericytoma
  • Phosphaturic mesenchymal tumor[13]
Stauffer syndrome

Neurological

A particularly devastating form of paraneoplastic syndromes is a group of disorders classified as paraneoplastic neurological disorders (PNDs).[14] These paraneoplastic disorders affect the central or peripheral nervous system; some are degenerative [15], 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 (IPA) is an organization dedicated to providing support and information to those affected by paraneoplastic neurological disorders.

References

  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, PMID 8093269 
  4. ^ 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 
  5. ^ Albert,M.A., Darnell,R.B. (2004), "Paraneoplastic neurological degenerations: keys to tumour immunity", Nature Reviews Cancer 4 (1): 36-44, PMID 14708025 
  6. ^ MeSH Paraneoplastic+endocrine+syndromes
  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. ^ MeSH Nervous+system+paraneoplastic+syndromes
  9. ^ 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. 
  10. ^ 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. 
  11. ^ Cohen PR, Kurzrock R (1997). "Mucocutaneous paraneoplastic syndromes". Semin. Oncol. 24 (3): 334–59. PMID 9208889. 
  12. ^ Staszewski H (1997). "Hematological paraneoplastic syndromes". Semin. Oncol. 24 (3): 329–33. PMID 9208888. 
  13. ^ 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. 
  14. ^ 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. 
  15. ^ 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


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