Paraneoplastic Syndrome: Difference between revisions
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'''Original Editors '''-[[User: Brittany Chorley|Brittany Chorley]] &[[User: Seth Chorley|Seth Chorley]][[Pathophysiology of Complex Patient Problems|from Bellarmine University's Pathophysiology of Complex Patient Problems project.]] | '''Original Editors '''-[[User: Brittany Chorley|Brittany Chorley]] &[[User: Seth Chorley|Seth Chorley]][[Pathophysiology of Complex Patient Problems|from Bellarmine University's Pathophysiology of Complex Patient Problems project.]] | ||
'''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}} | '''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}} | ||
</div> | </div> | ||
== Introduction == | == Introduction == | ||
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Paraneoplastic syndromes (PNS) occur due to the systemic effect of a malignancy and occur remotely to the primary malignancy. Symptoms are brought about by [[cytokines]], [[hormones]] or [[Immune System|immune]] cross-reactivity. These syndromes can cause a manifold of symptoms and can affect numerous systems.<ref name=":2">Radiopedia [https://radiopaedia.org/articles/paraneoplastic-syndromes Paraneoplastic syndromes] Available: https://radiopaedia.org/articles/paraneoplastic-syndromes<nowiki/>(accessed 24.1.2023)</ref> PNS can occur concurrently with tumour diagnosis before a tumour is diagnosed and even after tumours have been resected<ref name=":0">Henry K. [https://www.sciencedirect.com/science/article/abs/pii/S0740257019300097 Paraneoplastic syndromes: definitions, classification, pathophysiology and principles of treatment.] InSeminars in diagnostic pathology 2019 Jul 1 (Vol. 36, No. 4, pp. 204-210). WB Saunders.Available from:https://www.sciencedirect.com/science/article/abs/pii/S0740257019300097 (last accessed 15.7.2020)</ref>. The symptoms typically present in the middle-aged to [[Older People - An Introduction|older]] population. Also, it is common in individuals with [[Lung Cancer|lung]], [[Ovarian Cancer|ovarian]], [[Acute Lymphoblastic Leukemia|lymphatic]], or [[Breast Cancer|breast]] cancer. The most common cancer associated with paraneoplastic syndrome is small-cell cancer of the lungs. <ref name="p2">National Institute of Neurological Disorders and Stroke. NINDS Paraneoplastic Syndromes Information Page. http://www.ninds.nih.gov/disorders/paraneoplastic/paraneoplastic.htm. (accessed 18 March 2013).</ref> | Paraneoplastic syndromes (PNS) occur due to the systemic effect of a malignancy and occur remotely to the primary malignancy. Symptoms are brought about by [[cytokines]], [[hormones]] or [[Immune System|immune]] cross-reactivity. These syndromes can cause a manifold of symptoms and can affect numerous systems.<ref name=":2">Radiopedia [https://radiopaedia.org/articles/paraneoplastic-syndromes Paraneoplastic syndromes] Available: https://radiopaedia.org/articles/paraneoplastic-syndromes<nowiki/>(accessed 24.1.2023)</ref> PNS can occur concurrently with tumour diagnosis before a tumour is diagnosed and even after tumours have been resected<ref name=":0">Henry K. [https://www.sciencedirect.com/science/article/abs/pii/S0740257019300097 Paraneoplastic syndromes: definitions, classification, pathophysiology and principles of treatment.] InSeminars in diagnostic pathology 2019 Jul 1 (Vol. 36, No. 4, pp. 204-210). WB Saunders.Available from:https://www.sciencedirect.com/science/article/abs/pii/S0740257019300097 (last accessed 15.7.2020)</ref>. The symptoms typically present in the middle-aged to [[Older People - An Introduction|older]] population. Also, it is common in individuals with [[Lung Cancer|lung]], [[Ovarian Cancer|ovarian]], [[Acute Lymphoblastic Leukemia|lymphatic]], or [[Breast Cancer|breast]] cancer. The most common cancer associated with paraneoplastic syndrome is small-cell cancer of the lungs. <ref name="p2">National Institute of Neurological Disorders and Stroke. NINDS Paraneoplastic Syndromes Information Page. http://www.ninds.nih.gov/disorders/paraneoplastic/paraneoplastic.htm. (accessed 18 March 2013).</ref> | ||
There is no cure for paraneoplastic syndromes and treatment does not stop neurological damage.<ref name=":3">NIH [https://www.ninds.nih.gov/health-information/disorders/paraneoplastic-syndromes Paraneoplastic Syndrome]s Available:https://www.ninds.nih.gov/health-information/disorders/paraneoplastic-syndromes (accessed 24.1.2023)</ref> | There is no cure for paraneoplastic syndromes and the treatment does not stop neurological damage.<ref name=":3">NIH [https://www.ninds.nih.gov/health-information/disorders/paraneoplastic-syndromes Paraneoplastic Syndrome]s Available:https://www.ninds.nih.gov/health-information/disorders/paraneoplastic-syndromes (accessed 24.1.2023)</ref> | ||
== Aetiology == | == Aetiology == | ||
PNS are largely due to two main causes: | PNS are largely due to two main causes: | ||
# Those due to tumour secretions of hormones, functionally active peptides, enzymes cytokines | # Those due to tumour secretions of hormones, functionally active peptides, enzymes cytokines | ||
# Those due to tumours operating through auto-immune/immunological mechanisms with cross-reacting antibodies between neoplastic and normal tissues. Nb Remission of symptoms often follows resection of humoral secretory tumours but not always of tumours due to immunological mechanisms<ref name=":0" />. | # Those due to tumours operating through auto-immune/immunological mechanisms with cross-reacting antibodies between neoplastic and normal tissues. Nb Remission of symptoms often follows resection of humoral secretory tumours but not always of tumours due to immunological mechanisms<ref name=":0" />. | ||
== Types of Paraneoplastic Syndromes == | |||
* Lambert-Eaton Myasthenic Syndrome | |||
* Stiff-Person Syndrome | |||
* Encephalomyelitis | |||
* Myasthenia Gravis | |||
* Cerebellar Degeneration | |||
* Limbic or Brainstem Encephalitis | |||
* Neuromyotonia | |||
* Opsoclonus | |||
* Sensory Neuropathy | |||
==Epidemiology == | ==Epidemiology == |
Revision as of 16:05, 30 September 2023
Original Editors -Brittany Chorley &Seth Chorleyfrom Bellarmine University's Pathophysiology of Complex Patient Problems project.
Top Contributors - Seth Chorley, Brittany Chorley, Lucinda hampton, Kim Jackson, Priya Gulla, Admin, Elaine Lonnemann, WikiSysop, Rujuta Naik, Vidya Acharya, Claire Knott, 127.0.0.1, Wendy Walker, Mariam Hashem and Adam Vallely Farrell
Introduction[edit | edit source]
Paraneoplastic syndromes (PNS) occur due to the systemic effect of a malignancy and occur remotely to the primary malignancy. Symptoms are brought about by cytokines, hormones or immune cross-reactivity. These syndromes can cause a manifold of symptoms and can affect numerous systems.[1] PNS can occur concurrently with tumour diagnosis before a tumour is diagnosed and even after tumours have been resected[2]. The symptoms typically present in the middle-aged to older population. Also, it is common in individuals with lung, ovarian, lymphatic, or breast cancer. The most common cancer associated with paraneoplastic syndrome is small-cell cancer of the lungs. [3]
There is no cure for paraneoplastic syndromes and the treatment does not stop neurological damage.[4]
Aetiology[edit | edit source]
PNS are largely due to two main causes:
- Those due to tumour secretions of hormones, functionally active peptides, enzymes cytokines
- Those due to tumours operating through auto-immune/immunological mechanisms with cross-reacting antibodies between neoplastic and normal tissues. Nb Remission of symptoms often follows resection of humoral secretory tumours but not always of tumours due to immunological mechanisms[2].
Types of Paraneoplastic Syndromes[edit | edit source]
- Lambert-Eaton Myasthenic Syndrome
- Stiff-Person Syndrome
- Encephalomyelitis
- Myasthenia Gravis
- Cerebellar Degeneration
- Limbic or Brainstem Encephalitis
- Neuromyotonia
- Opsoclonus
- Sensory Neuropathy
Epidemiology[edit | edit source]
PNS occur in up to 15% of patients with cancer.[1] Neurological manifestation in the form of neuropathies is common. Males and females are affected equally.[5]
Clinical Presentation[edit | edit source]
PNS can affect multiple systems and have various presentations. Symptoms usually start before a tumour is found, slowly developing over a few days or weeks. Some of the more common symptoms include - Difficulty walking or 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; Vertigo or dizziness.[4]
Examples are given below based on the organ system involved.
- Paraneoplastic neurological syndromes: encephalitis (paraneoplastic rhombencephalitis, limbic encephalitis); Lambert-Eaton myasthenic syndrome; Optic neuropathy; Cerebellar degeneration.
- Endocrine eg Cushing syndrome; Syndrome of inappropriate antidiuretic hormone secretion (SIADH); Hypercalcemia
- Rheumatological eg Paraneoplastic polyarthritis; Polymyalgia rheumatica; Multicentric reticulohistiocytosis; Hypertrophic osteoarthropathy
- Paraneoplastic haematologic syndromes: Good syndrome; Polycythaemia[1]
- Dermatological eg Acanthosis nigricans; Paraneoplastic pemphigus; Sweet syndrome; Leukocytoclastic vasculitis; Dermatomyositis
- Renal eg Electrolyte imbalance (hypokalemia, hypo or hypernatremia, hyperphosphatemia) causing nephropathy and acid-base disturbance due to ectopic hormones produced by tumour cells such as ACTH and ADH. Nephrotic syndrome can also be one of the manifestations of paraneoplastic syndrome.
- Miscellaneous eg Fever, cachexia, anorexia, dysgeusia[5]
Management[edit | edit source]
Management of the patients is based on the type, severity, and location of the paraneoplastic syndrome. First, therapeutic options are to treat underlying malignancy with chemotherapy, radiation, or surgery.
- Other therapeutic options are immunosuppression with corticosteroids or other immunosuppressive drugs, intravenous immunoglobulins, plasma exchange, or plasmapheresis.
Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]
First, a healthcare provider will perform a clinical exam that would include a general physical and neurological screening.
- Neurological screening: reflexes, sensation, myotomes, balance, and coordination.
- Laboratory tests that could be utilized to diagnose paraneoplastic syndrome include: Blood tests, Spinal tap
- Imaging tests that could be utilized to diagnose paraneoplastic syndrome include: CT Scan; MRI; PET Scan; and PET-CT, which may enhance the detection rate of small cancers
If the physicians cannot find a malignant tumour, the syndrome may be the cause of a tumour that is too small to locate. In this instance, the physician will continue to have follow-up imaging conducted every three to six months for several years unless the cause is identified. [6]
Physical Therapy Management[edit | edit source]
People with paraneoplastic syndrome can have difficulty with walking, balance, coordination, muscle tone, sensory of where the body is in space, and vertigo.
- All of these symptoms the physical therapist can treat with traditional therapy.
- Precautions must be taken into account for the cancer or neoplasm that is involved.[3]
Conclusion[edit | edit source]
The diagnosis and management of paraneoplastic syndromes is difficult.
- In most cases, there is an underlying malignancy responsible.
- Due to the numerous causes, the condition is best managed by an interprofessional team (including a pathologist, oncologist, radiologist, haematologist, nurse specialist, and internist).
- Once the cause is discovered, it needs to be treated.
The management of the patients is based on the type, severity, and location of the paraneoplastic syndrome.
- First, therapeutic options are to treat underlying malignancy with chemotherapy, radiation, or surgery.
- Other therapeutic options are immunosuppression with corticosteroids or other immunosuppressive drugs, intravenous immunoglobulins, plasma exchange, or plasmapheresis.[5]
References[edit | edit source]
- ↑ 1.0 1.1 1.2 Radiopedia Paraneoplastic syndromes Available: https://radiopaedia.org/articles/paraneoplastic-syndromes(accessed 24.1.2023)
- ↑ 2.0 2.1 Henry K. Paraneoplastic syndromes: definitions, classification, pathophysiology and principles of treatment. InSeminars in diagnostic pathology 2019 Jul 1 (Vol. 36, No. 4, pp. 204-210). WB Saunders.Available from:https://www.sciencedirect.com/science/article/abs/pii/S0740257019300097 (last accessed 15.7.2020)
- ↑ 3.0 3.1 National Institute of Neurological Disorders and Stroke. NINDS Paraneoplastic Syndromes Information Page. http://www.ninds.nih.gov/disorders/paraneoplastic/paraneoplastic.htm. (accessed 18 March 2013).
- ↑ 4.0 4.1 NIH Paraneoplastic Syndromes Available:https://www.ninds.nih.gov/health-information/disorders/paraneoplastic-syndromes (accessed 24.1.2023)
- ↑ 5.0 5.1 5.2 Thapa B, Ramphul K. Paraneoplastic syndromes. InStatPearls [Internet] 2020 Jun 27. StatPearls Publishing.Available from:https://www.ncbi.nlm.nih.gov/books/NBK507890/ (last accessed 15.7.2020)
- ↑ Mayo Clinic. Paraneoplastic syndromes of the nervous system. http://www.mayoclinic.com/health/paraneoplastic-syndromes/DS00840. (accessed 18 March 2013).