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<div class="noeditbox">Welcome to [[Pathophysiology of Complex Patient Problems|PT 635 Pathophysiology of Complex Patient Problems]] This is a wiki created by and for the students in the School of Physical Therapy at Bellarmine University in Louisville KY. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!</div><div class="editorbox">
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== Definition/Description  ==
== Introduction  ==
[[File:Paraneoplastic syndrome.png|400x400px|alt=|thumb|Paraneoplastic syndrome]]
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 syndrome is a group of rare disorders that are triggered by an abnormal immune system response a neoplasm. It is hypothesisized that Paraneoplastic syndrome occurs when cancer-fighting antibodies or white blood cells (known as T cells) mistakenly attack normal cells in the nervous system.&nbsp; Neurologic symptoms can develop over a period of days to weeks and usually occur prior to the tumor being discovered. Paraneoplastic syndrome presents with symptoms that include difficulty in 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, and vertigo or dizziness. Paraneoplastic syndromes include Lambert-Eaton myasthenic syndrome, stiff-person syndrome, encephalomyelitis, myasthenia gravis, cerebellar degeneration, limbic or brainstem encephalitis, neuromyotonia, opsoclonus, and sensory neuropathy. <br>
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 ==
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<ref name=":0" />.


== Prevalence  ==
== Types of Paraneoplastic Syndrome ==
The Paraneoplastic Syndromes are classified based on the organ systems involved or on the etiological groups and are as follows<ref>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.</ref> :
* Lambert-Eaton Myasthenic Syndrome
* Stiff-Person Syndrome
* Encephalomyelitis
* Myasthenia Gravis
* Cerebellar Degeneration
* Limbic or Brainstem Encephalitis
* Neuromyotonia
* Opsoclonus
* Sensory Neuropathy


You will see symptoms of this disorder typically in the middle-aged to older population. Specifically, it is most common in individuals with lung, ovarian, lymphatic, or breast cancer.&nbsp; The most common cancer associated with paraneoplastic syndrome is small cell cancer of the lungs.<br>
==Epidemiology  ==
PNS occur in up to 15% of patients with cancer.<ref name=":2" /> Neurological manifestation in the form of neuropathies is common. Males and females are affected equally.<ref name=":1">Thapa B, Ramphul K. [https://www.ncbi.nlm.nih.gov/books/NBK507890/ Paraneoplastic syndromes.] InStatPearls [Internet] 2020 Jun 27. StatPearls Publishing.Available from:https://www.ncbi.nlm.nih.gov/books/NBK507890/ (last accessed 15.7.2020)</ref>


== '''<u></u>'''Characteristics/Clinical Presentation  ==
==<u></u>Clinical Presentation  ==
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.<ref name=":3" />


Paraneoplastic syndrome presents with symptoms that include difficulty in 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, and vertigo or dizziness. Paraneoplastic syndromes include Lambert-Eaton myasthenic syndrome, stiff-person syndrome, encephalomyelitis, myasthenia gravis, cerebellar degeneration, limbic or brainstem encephalitis, neuromyotonia, opsoclonus, and sensory neuropathy. <br>
Examples are given below based on the organ system involved.
 
# Paraneoplastic neurological syndromes: [[encephalitis]] (paraneoplastic rhombencephalitis, [[Limbic System|limbic]] encephalitis); [[Lambert-Eaton Myasthenic Syndrome|Lambert-Eaton myasthenic syndrome]]; [[Optic Nerve|Optic]] neuropathy; [[Cerebellum|Cerebellar]] degeneration.
Paraneoplastic syndromes most commonly occur in patients not known to have cancer, as well as in those with active cancer and those in remission after treatment. A complete history and physical examination findings can suggest neoplasia. Persons with a family history of malignancies such as breast or colon cancer may be at an increased risk and should be screened for cancer.
#Endocrine eg [[Cushing's Syndrome|Cushing]] syndrome; Syndrome of inappropriate antidiuretic hormone secretion (SIADH); Hypercalcemia
 
#Rheumatologica'''l''' eg Paraneoplastic polyarthritis; [[Polymyalgia Rheumatica|Polymyalgia rheumatica]]; Multicentric reticulohistiocytosis; Hypertrophic osteoarthropathy
Because of their complexity and variety, the clinical presentations of Paraneoplastic syndrome may vary greatly. In general, paraneoplastic syndromes are divided into the following categories: (1) miscellaneous (nonspecific), (2) rheumatologic, (3) renal, (4) gastrointestinal, (5) hematologic, (6) cutaneous, (7) endocrine, and (8) neuromuscular.
#Paraneoplastic haematologic syndromes: Good syndrome; Polycythaemia<ref name=":2" />
 
#Dermatological eg Acanthosis nigricans; Paraneoplastic pemphigus; Sweet syndrome; Leukocytoclastic vasculitis; [[Dermatomyositis]]
<u>'''Miscellaneous (nonspecific)'''</u><br>Fever, dysgeusia, anorexia, and cachexia are included in this category.<br>Fever is frequently associated with lymphomas, acute leukemias, sarcomas, renal cell carcinomas (Grawitz tumors), and digestive malignancies (including the liver).
#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 Nervosa|anorexia]], dysgeusia<ref name=":1" /><sup></sup><sup></sup><sup></sup>
<br><u>'''Rheumatologic'''</u><br>Paraneoplastic arthropathies arise as rheumatic polyarthritis or polymyalgia, particularly in patients with myelomas; lymphomas; acute leukemia; malignant histiocytosis; and tumors of the colon, pancreas, prostate, and CNS.<br>Hypertrophic osteoarthropathy may be observed in patients with lung cancers, pleural mesothelioma, or phrenic neurilemmoma.<br>Scleroderma may precede direct evidence of tumor<br>The widespread form is typical of malignancies of the breast, uterus, and lung (both alveolar and bronchial forms).<br>The localized form is characteristic of carcinoids and of lung tumors (bronchoalveolar forms).<br>Systemic lupus erythematosus (SLE) may develop in patients with lymphomas or cancers of the lung, breast, or gonads.<br>Secondary amyloidosis of the connective tissues is a rare presentation in patients with myeloma, renal carcinoma, and lymphomas.
== Management ==
 
[[File:National-cancer-institute-0YBIMOqQzt0-unsplash.jpg|right|frameless]]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 Side Effects and Syndromes|chemotherapy]], [[Radiation Side Effects and Syndromes|radiation]], or surgery.
<u>'''Renal'''</u><br>Hypokalemic nephropathy, which is characterized by urinary potassium leakage of more than 20 mEq per 24 hours, may develop in patients with tumors that secrete adrenocorticotropic hormone (ACTH) or ACTH-like substances. It occurs in 50% of individuals with ACTH-secreting tumors of the lung (ie, small cell lung cancer ).<br>Hypokalemia, hyponatremia or hypernatremia, hyperphosphatemia, and alkalosis or acidosis may result from other types of tumors that produce ACTH, antidiuretic hormone (ADH), or gut hormones (see Endocrine and neuromuscular, below).<br>Nephrotic syndrome is observed, although infrequently, in patients who have Hodgkin lymphoma (HL); non-Hodgkin lymphoma (NHL); leukemias; melanomas; or malignancies of lung, thyroid, colon, breast, ovary, or pancreatic head.<br>Secondary amyloidosis of the kidneys, heart, or CNS may rarely be a presenting feature in patients with myeloma, renal carcinoma, or lymphomas. The clinical picture of secondary amyloidosis is related to renal and cardiac injuries.  
* Other therapeutic options are immunosuppression with [[Corticosteroids in the Management of Rheumatoid Arthritis|corticosteroids]] or other immunosuppressive drugs, intravenous immunoglobulins, plasma exchange, or plasmapheresis.
 
<u>'''Gastrointestinal'''</u><br>Watery diarrhea accompanied by an electrolyte imbalance leads to asthenia, confusion, and exhaustion.<br>These problems are typical of patients with proctosigmoid tumors (both benign and malignant) and of medullary thyroid carcinomas (MTCs) that produce several prostaglandins (PGs; especially PG E2 and F2) that lead to malabsorption and, consequently, unavailability of nutrients.<br>These alterations also can be observed in patients with melanomas, myelomas, ovarian tumors, pineal body tumors, and lung metastases.
 
<u>'''Hematologic'''</u><br>Symptoms related to erythrocytosis or anemia,[12] thrombocytosis, disseminated intravascular coagulation (DIC), and leukemoid reactions may result from many types of cancers.<br>In some cases, symptoms result from migrating vascular thrombosis (ie, Trousseau syndrome)[13] occurring in at least 2 sites.<br>Leukemoid reactions, characterized by the presence of immature WBCs in the bloodstream, are usually accompanied by hypereosinophilia and itching. These reactions are typically observed in patients with lymphomas or cancers of the lung, breast, or stomach.<br>Cryoglobulinemia may occur in patients with lung cancer or pleural mesothelioma.
 
<u>'''Cutaneous'''</u><br>Itching is the most frequent cutaneous manifestation in patients with cancer.<br>Herpes zoster, ichthyosis, flushes, alopecia, or hypertrichosis also may be observed.<br>Acanthosis nigricans and dermic melanosis are characterized by a blackish pigmentation of the skin and usually occur in patients with metastatic melanomas or pancreatic tumors.
 
<br><u>'''Endocrine'''</u><br>Endocrine symptoms related to paraneoplastic syndromes usually resemble the more common endocrine disorders (eg, Cushing syndrome). Neuromuscular symptoms may mimic common neurological conditions (eg, dementia).<br>Cushing syndrome, accompanied by hypokalemia, very high plasma ACTH levels, and increased serum and urine cortisol concentrations, is the most common example of an endocrine disorder linked to a malignancy. This is related to the ectopic production of ACTH or ACTH-like molecules from many tumors (eg, small cell cancer of the lung).
 
<u>'''Neuromuscular'''</u><br>Neuromuscular disorders related to cancers are now included among the paraneoplastic syndromes. Such disorders affect 6% of all patients with cancer and are prevalent in ovarian and pulmonary cancers. Examples include the following:<br>Myasthenia gravis is the most common paraneoplastic syndrome in patients with thymoma,[20] a malignancy arising from epithelial cells of the thymus. Indeed, thymoma is the underlying cause in approximately 10% to 15% of cases of myasthenia gravis.[21] Rarely, hypogammaglobulinemia and pure red cell aplasia occur as paraneoplastic syndromes in patients with thymoma.<br>Lambert-Eaton myasthenic syndrome (LEMS), which manifests as asthenia of the scapular and pelvic girdles and a reduction of tendon reflexes. LEMS sometimes can be accompanied by xerostomia, sexual impotence, myopathy, and peripheral neuropathy. It is associated with cancer 40-70% of the time, most commonly small cell lung cancer (SCLC). It seems to result from interference with the release of acetylcholine due to immunologic attack against the presynaptic voltage-gated calcium channel.<br>Opsoclonus-myoclonus syndrome usually affects children younger than 4 years. It is associated with hypotonia, ataxia, and irritability. One in two patients has neuroblastoma.<br>Paraneoplastic limbic encephalitis is characterized by depression, seizures, irritability, and short-term memory loss. The neurologic symptoms develop rapidly and can resesmble dementia. Paraneoplastic limbic encephalitis is most commonly associated with SCLC.<br>Paraneoplastic encephalomyelitis is characterized by a complex of symptoms derived from brainstem encephalitis, limbic encephalitis, cerebellar degeneration, myelitis, and autonomic dysfunction. Such neurologic deficits and signs seem to be related to an inflammatory process involving multiple areas of the nervous system.<br>Paraneoplastic cerebellar degeneration causes gait difficulties, dizziness, nausea, and diplopia, followed by ataxia, dysarthria, and dysphagia. Paraneoplastic cerebellar degeneration is frequently associated with Hodgkin lymphoma, breast cancer, SCLC, and ovarian cancer; it may occur in association with prostate carcinoma.<br>Paraneoplastic sensory neuropathy affects lower and upper extremities and is characterized by progressive sensory loss, either symmetric or asymmetric. It seems to be related to the loss of the dorsal root ganglia with early involvement of major fibers responsible for detecting vibration and position. <br>
 
== Associated Co-morbidities  ==
 
-HTN<br>
 
-Endocarditis
 
-Anorexia
 
-Thrombophlebitis
 
-Polyarthritis
 
-Anemia
 
== Medications  ==
 
Medications will depend on what type of paraneoplastic syndrome the patient has.&nbsp; In addition to chemotherapy for the underlying cancer, medications could include:<br>
 
<br>
 
Corticosteroids, such as prednisone, inhibit inflammation. Long-term use of corticosteroids may cause weakening of the bones (osteoporosis), diabetes, high blood pressure, high cholesterol and other serious side effects.<br>
 
<br> Immunosuppressants inhibit the production of disease-fighting white blood cells. Side effects include an increased risk of infections. Drugs with immunosuppressant properties that may be used include azathioprine (Imuran) and cyclophosphamide (Cytoxan).<br><br>
 
Anti-seizure medications may help control seizures associated with syndromes affecting nerve cells in the brain. A number of anti-seizure medications are available, including carbamazepine (Tegretol, Carbatrol, others) and valproic acid (Depakene, Stavzor).
 
<br> Medications to enhance nerve to muscle transmission may improve symptoms of syndromes affecting muscle function. Some drugs, such as 3,4-diaminopyridine, enhance the release of a chemical messenger that transmits a signal from nerve cells to muscles. Other drugs, such as pyridostigmine (Mestinon, Regonol), prevent the breakdown of these chemical messengers.
 
 
 
[[Image:Paraneoplastic_Syndromes_Treatment_Pros_and_Cons.gif]]<br><br>


== Diagnostic Tests/Lab Tests/Lab Values  ==
== Diagnostic Tests/Lab Tests/Lab Values  ==


First, a health care provider will perform a clinical exam that would include a general physical and neurological screening. Tests that would be involved in the neurological screening could include reflexes, sensation, myotomes, balance, and coordination.
First, a healthcare provider will perform a clinical exam that would include a general physical and neurological screening.   
 
Laboratory tests that could be utilized to diagnose paraneoplastic syndrome include:
 
Blood tests: This may identify antibodies typically associated with paraneoplastic syndrome. However, some people who have the syndrome do not have the antibody, and some people who do not have the syndrome actually have the antibody. Blood tests can also identify an infection, disorder of nutrient processing, or hormone disorder.
 
Spinal tap: A neurologist or nurse will insert a needle into your lumbar spine to extract a small amount of cerebrospinal fluid (CSF). At times, paraneoplastic antibodies may be present in the CSF but not in the blood.
 
Imagine tests that could be utilized to diagnose paraneoplastic syndrome include:
 
CT Scan
 
MRI
 
PET Scan
 
PET-CT, which may enhance the detection rate of small cancers
 
"If no malignant tumor is located or no other cause identified, the problem may be a tumor that's still too small to find. You'll likely have follow-up imaging tests every three to six months for a few years or until a cause is identified. " (Mayo Clinic)<br>
 
== Etiology/Causes  ==
 
"Paraneoplastic syndromes occur among a small percentage of people who have cancer, but these syndromes aren't caused by cancer cells directly disrupting nerve function.
 
Instead, they're believed to be caused by cancer-fighting antibodies or certain white blood cells, known as T cells, that the body activates to combat the cancer. Instead of attacking only the cancer cells, these immune system agents also attack normal cells of the nervous system."<br>
 
<br>
 
QUOTE&nbsp;FROM&nbsp;MAYO&nbsp;CLINIC
 
== Systemic Involvement ==
 
-Renal
 
-Gastrointestinal
 
-Hematologic
 
-Cutaneous
 
-Endocrine
 
-Neuromuscular
 
== Medical Management (current best evidence)  ==
 
Treatment varies with the type and location of the paraneoplastic disorder.
 
Treatment of the underlying tumor<br>In general, the therapeutic protocols used are those that are applied in patients without paraneoplastic syndromes (ie, surgery, radiation, or chemotherapy, alone or in combination).
 
<br>Treatment of the presumptive immune-mediated disorder<br>The second therapeutic option is based on immunosuppression (by intravenous immunoglobulins, steroids or other immunosuppressive drugs, or by plasma exchange).<br>This treatment should be reserved for patients with clearly identifiable antibodies in their serum.<br>Some patients with paraneoplastic pemphigus seem to derive some benefit from rituximab, but further studies are necessary to confirm this observation.
 
Plasmapheresis
 
This process — also known as plasma exchange — separates the fluid part of the blood, called plasma, from your blood cells with a device known as a cell separator. The red and white blood cells, along with your platelets, are returned to your body, while the plasma, which contains unwanted antibodies, is discarded and replaced with other fluids.<br>Intravenous immune globulin (IVIg). Immune globulin contains healthy antibodies from blood donors. High doses of immune globulin accelerate the destruction of damaging antibodies in your blood.
 
Other therapies may be helpful if a paraneoplastic syndrome has caused impairments that disrupt functional abilities:
 
Physical therapy
 
Speech Therapy<br>
 
== Physical Therapy Management (current best evidence)  ==
 
People with Paraneoplastic syndrome can have difficulty with walking, 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 to account for the cancer or neoplasm that is involved.
 
== Alternative/Holistic Management (current best evidence)  ==
 
add text here
 
== Differential Diagnosis  ==
 
Abdominal Aortic Aneurysm<br> Anemia<br> Antithrombin Deficiency<br> Attention Deficit Hyperactivity Disorder<br> Bone Marrow Failure<br> Chronic Fatigue Syndrome<br> Dermatomyositis<br> Diabetes Mellitus, Type 1<br> Glomerulonephritis, Acute<br> Mixed Connective-Tissue Disease<br> Myelodysplastic Syndrome<br> Nephrotic Syndrome<br> Personality Disorders<br> Polycythemia Vera<br> Polymyalgia Rheumatica<br> Scleroderma<br> Superficial Thrombophlebitis<br> Systemic Lupus Erythematosus<br> Undifferentiated Connective-Tissue Disease
 
== Case Reports/ Case Studies  ==
 
add links to case studies here (case studies should be added on new pages using the [[Template:Case Study|case study template]])<br>
 
== Resources <br>  ==
 
add appropriate resources here
 
== Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed])  ==
 
see tutorial on [[Adding PubMed Feed|Adding PubMed Feed]]
<div class="researchbox">
<rss>http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1NGmwZeh8JwVIzrKgHG1LrDm0izTr7ViJiDkSYAY2BW5hiXsx0|charset=UTF-8|short|max=10</rss>


&lt;div class="researchbox"&gt;&lt;rss&gt;http://www.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1z_pxc-9fhPQGO3O_wUPIHn9g0TvgRt5zhdAuv7LxIMLxABktq|charset=UTF-8|short|max=10&lt;/rss&gt;&lt;/div&gt;<br><br>
# Neurological screening: reflexes, sensation, myotomes, balance, and coordination.
</div>
# Laboratory tests that could be utilized to diagnose paraneoplastic syndrome include: [[Blood Tests|Blood tests]], Spinal tap
# Imaging tests that could be utilized to diagnose paraneoplastic syndrome include: [[CT Scans|CT]] Scan; [[MRI Scans|MRI]]; PET Scan; and PET-CT, which may enhance the detection rate of small cancers


== References  ==
If the physicians cannot find a malignant tumour, the syndrome may be the cause of a tumour that is too small to locate.&nbsp;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.&nbsp; <ref name="p1">Mayo Clinic. Paraneoplastic syndromes of the nervous system. http://www.mayoclinic.com/health/paraneoplastic-syndromes/DS00840. (accessed 18 March 2013).</ref>
== Physical Therapy Management  ==
People with paraneoplastic syndrome can have difficulty with walking, [[balance]], [[Coordination Exercises|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.<ref name="p2" />
== Conclusion    ==
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.<ref name=":1" /><br>


see [[Adding References|adding references tutorial]].
== References ==


<references />&nbsp;  
<references />&nbsp;  


[[Category:Bellarmine_Student_Project]]
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[[Category:Syndromes]] 
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[[Category:Chronic Respiratory Disease - Conditions]]
[[Category:Oncology]]

Latest revision as of 16:08, 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, Admin, Kim Jackson, Priya Gulla, Elaine Lonnemann, Rujuta Naik, WikiSysop, 127.0.0.1, Vidya Acharya, Claire Knott, Wendy Walker, Mariam Hashem and Adam Vallely Farrell

Introduction[edit | edit source]

Paraneoplastic syndrome

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:

  1. Those due to tumour secretions of hormones, functionally active peptides, enzymes cytokines
  2. 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 Syndrome[edit | edit source]

The Paraneoplastic Syndromes are classified based on the organ systems involved or on the etiological groups and are as follows[5] :

  • 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.[6]

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.

  1. Paraneoplastic neurological syndromes: encephalitis (paraneoplastic rhombencephalitis, limbic encephalitis); Lambert-Eaton myasthenic syndrome; Optic neuropathy; Cerebellar degeneration.
  2. Endocrine eg Cushing syndrome; Syndrome of inappropriate antidiuretic hormone secretion (SIADH); Hypercalcemia
  3. Rheumatological eg Paraneoplastic polyarthritis; Polymyalgia rheumatica; Multicentric reticulohistiocytosis; Hypertrophic osteoarthropathy
  4. Paraneoplastic haematologic syndromes: Good syndrome; Polycythaemia[1]
  5. Dermatological eg Acanthosis nigricans; Paraneoplastic pemphigus; Sweet syndrome; Leukocytoclastic vasculitis; Dermatomyositis
  6. 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.
  7. Miscellaneous eg Fever, cachexia, anorexia, dysgeusia[6]

Management[edit | edit source]

National-cancer-institute-0YBIMOqQzt0-unsplash.jpg

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.

  1. Neurological screening: reflexes, sensation, myotomes, balance, and coordination.
  2. Laboratory tests that could be utilized to diagnose paraneoplastic syndrome include: Blood tests, Spinal tap
  3. 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.  [7]

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.[6]

References[edit | edit source]

  1. ↑ 1.0 1.1 1.2 Radiopedia Paraneoplastic syndromes Available: https://radiopaedia.org/articles/paraneoplastic-syndromes(accessed 24.1.2023)
  2. ↑ 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. ↑ 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. ↑ 4.0 4.1 NIH Paraneoplastic Syndromes Available:https://www.ninds.nih.gov/health-information/disorders/paraneoplastic-syndromes (accessed 24.1.2023)
  5. ↑ 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.
  6. ↑ 6.0 6.1 6.2 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)
  7. ↑ Mayo Clinic. Paraneoplastic syndromes of the nervous system. http://www.mayoclinic.com/health/paraneoplastic-syndromes/DS00840. (accessed 18 March 2013).

 

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