Paraneoplastic syndrome

Paraneoplastic syndrome
SpecialtyOncology
FrequencyRare[1]

A paraneoplastic syndrome is a syndrome (a set of signs and symptoms) that is the consequence of a tumor in the body (usually a cancerous one).[1] It is specifically due to the production of chemical signaling molecules (such as hormones or cytokines) by tumor cells or by an immune response against the tumor.[2] Unlike a mass effect, it is not due to the local presence of cancer cells.[3]

Paraneoplastic syndromes are typical among middle-aged to older people, and they most commonly occur with cancers of the lung, breast, ovaries or lymphatic system (a lymphoma).[4] 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[5] in suppressing tumor growth and symptoms.[6][7] 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.

  1. ^ a b "Paraneoplastic Syndromes". National Institute of Neurological Disorders and Stroke. Retrieved 5 September 2023.
  2. ^ Sardiña González, Cristina; Martínez Vivero, Clara; López Castro, José (June 2022). "Paraneoplastic syndromes review: The great forgotten ones". Critical Reviews in Oncology/Hematology. 174: 103676. doi:10.1016/j.critrevonc.2022.103676. ISSN 1879-0461. PMID 35378267. S2CID 247934655.
  3. ^ Paraneoplastic Syndromes, 2011, Darnell & Posner
  4. ^ NINDS Paraneoplastic Syndromes Information Page Archived 2015-01-04 at the Wayback Machine National Institute of Neurological Disorders and Stroke
  5. ^ Darnell RB, DeAngelis LM (January 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. S2CID 205040647.
  6. ^ Roberts WK, Darnell RB (October 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.
  7. ^ Albert ML, Darnell RB (January 2004). "Paraneoplastic neurological degenerations: keys to tumour immunity". Nature Reviews. Cancer. 4 (1): 36–44. doi:10.1038/nrc1255. PMID 14708025. S2CID 7319871.