Paraneoplastic syndrome
Paraneoplastic syndrome | |
---|---|
Specialty | Oncology |
Frequency | Rare[1] |
A paraneoplastic syndrome is a
Paraneoplastic syndromes are typical among middle-aged to older people, and they most commonly occur with cancers of the
The abbreviation PNS is sometimes used for paraneoplastic syndrome, although it is used more often to refer to the peripheral nervous system.
Signs and symptoms
Symptomatic features of paraneoplastic syndrome cultivate in four ways:
Endocrine
The following diseases manifest by means of
Neurological
The following diseases manifest by means of
Mucocutaneous
The following diseases manifest by means of
Hematological
The following diseases manifest by means of
Other
The following diseases manifest by means of physiological dysfunction besides the categories above:
Mechanism
The mechanism for a paraneoplastic syndrome varies from case to case. However, pathophysiological outcomes usually arise when a tumor does. Paraneoplastic syndrome often occurs alongside associated cancers as a result of an activated immune system. In this scenario, the body may produce antibodies to fight off the tumor by directly binding and destroying the tumor cell. Paraneoplastic disorders may arise in that antibodies would cross-react with normal tissues and destroy them.[9]
Diagnosis
Diagnostic testing in a possible paraneoplastic syndrome depends on the symptoms and the suspected underlying cancer.[citation needed]
Diagnosis may be difficult in patients in whom paraneoplastic antibodies cannot be detected. In the absence of these antibodies, other tests that may be helpful include MRI, PET, lumbar puncture and electrophysiology.[10]
Types
Syndrome class | Syndrome | Main causal cancers | Causal mechanism |
---|---|---|---|
Endocrine[11] | |||
Cushing syndrome
|
Ectopic ACTH and ACTH-like substance
| ||
Syndrome of inappropriate antidiuretic hormone
|
Antidiuretic hormone[12]
| ||
Hypercalcemia
|
|
IL-1[12]
| |
Hypoglycemia |
|
Insulin or insulin-like substance[12] or "big" IGF-II | |
Carcinoid syndrome | Serotonin, bradykinin[12] | ||
Hyperaldosteronism |
|
Aldosterone[14] | |
Neurological[15] | Lambert–Eaton myasthenic syndrome |
|
Immunologic |
Paraneoplastic cerebellar degeneration |
|
||
Encephalomyelitis | Inflammation of the brain and spinal cord | ||
Limbic encephalitis |
|
||
Brainstem encephalitis |
|
Antineuronal antibodies (anti-Hu, anti-Ri, and anti-Ma2). Some forms are amenable to immunotherapy while others are not.[16] | |
Opsoclonus myoclonus ataxia syndrome
|
|
Autoimmune reaction against the RNA-binding protein Nova-1[17] | |
Anti-NMDA receptor encephalitis | Autoimmune reaction against NMDA-receptor subunits
| ||
Polymyositis |
|
||
Mucocutaneous[19] | Acanthosis nigricans |
| |
Dermatomyositis |
|
Immunologic[12] | |
Leser-Trélat sign
|
|||
Necrolytic migratory erythema | Glucagonoma | ||
Sweet's syndrome
|
|||
Florid cutaneous papillomatosis | |||
Pyoderma gangrenosum | |||
Acquired generalized hypertrichosis
|
|||
Hematological[21] | Granulocytosis | G-CSF | |
Polycythemia |
|
Erythropoietin[12] | |
Trousseau sign | Mucins that activate clotting,[12] others
| ||
Nonbacterial thrombotic endocarditis |
|
Hypercoagulability[12]
| |
Anemia |
|
Unknown[12] | |
Others | Membranous glomerulonephritis |
|
|
Tumor-induced osteomalacia
|
|
||
Stauffer syndrome | |||
Neoplastic fever[23] | |||
Thymoma-associated multiorgan autoimmunity |
|
A specifically devastating form of (neurological) paraneoplastic syndromes is a group of disorders classified as
The most common cancers associated with PNDs are breast, ovarian, and lung cancers, but many other cancers can produce paraneoplastic symptoms, as well.[citation needed]
The root cause is extremely difficult to identify for paraneoplastic syndrome, as there are so many ways the disease can manifest (which may eventually lead to cancer)[citation needed]. Ideas may relate to age-related diseases (unable to handle environmental or physical stress in combination with genetic pre-dispositions), accumulation of damaged biomolecules (damages signaling pathways in various regions of the body), increased oxygen free radicals in the body (alters metabolic processes in various regions of the body), etc. [citation needed].
However, prophylactic efforts include routine checks with physicians (particularly those that specialize in neurology and oncology) especially when a patient notices subtle changes in his or her own body.[citation needed]
Treatment
Treatment options include:[citation needed]
- Therapies to eliminate the underlying cancer, such as chemotherapy, radiation and surgery.
- Therapies to reduce or slow neurological degeneration. In this scenario, 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 paraneoplastic neurological disorders (PNDs). Therefore, PND patients should consult with a specialist with experience in diagnosing and treating paraneoplastic neurological disorders.
A specific prognosis for those with paraneoplastic syndromes links to each unique case presented. Thus, prognosis for paraneoplastic syndromes may vary greatly. For example, paraneoplastic pemphigus often included infection as a major cause of death.[26] Paraneoplastic pemphigus is one of the three major subtypes that affects IgG autoantibodies that are characteristically raised against desmoglein 1 and desmoglein 3 (which are cell-cell adhesion molecules found in desmosomes).[27] Underlying cancer or irreversible system impairment, seen in acute heart failure or kidney failure, may result in death as well.[citation needed]
Research directions
Prostate cancer is the second most common urological malignancy to be associated with paraneoplastic syndromes after renal cell carcinoma. Paraneoplastic syndromes of this nature tend to occur in the setting of late stage and aggressive tumors with poor overall outcomes (endocrine manifestations, neurological entities, dermatological conditions, and other syndromes). A vast majority of prostate cancer cases (over 70%) document paraneoplastic syndrome as a major clinical manifestation of prostate cancer; and (under 20%), the syndrome as an initial sign of disease progression to the castrate-resistant state.[28] Urologist researchers identify serum markers that are associated with the syndrome in order to specific what type of therapies may work most effectively.[citation needed]
Paraneoplastic neurological syndromes may be related
References
- ^ a b "Paraneoplastic Syndromes". National Institute of Neurological Disorders and Stroke. Retrieved 5 September 2023.
- S2CID 247934655.
- ^ Paraneoplastic Syndromes, 2011, Darnell & Posner
- ^ NINDS Paraneoplastic Syndromes Information Page Archived 2015-01-04 at the Wayback Machine National Institute of Neurological Disorders and Stroke
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- ^ a b c d e f Santacroce L, Diomede L, Balducci L (3 February 2019). Talavera F, Movsas B (eds.). "Background of Paraneoplastic Syndromes". Medscape. WebMD LLC.
- S2CID 31651589.
- ^ Dalmau J, Rosenfield MR (6 December 2016). "Overview of paraneoplastic syndromes of the nervous system". UpToDate. Retrieved 23 December 2017.
- ^ Paraneoplastic+endocrine+syndromes at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
- ^ ISBN 978-1-4160-2973-1. 8th edition.
- ^ PMID 27170690.
- PMID 11368052.
- ^ Nervous+system+paraneoplastic+syndromes at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
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