Materiały źródłowe

• #1 Paraneoplastic Syndromes – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK507890/

  Paraneoplastic syndromes are rare disorders with complex systemic clinical manifestations due to underlying malignancy. […] The precise incidence and prevalence of paraneoplastic syndrome are unknown because of the rarity of the disease; however, it can occur with any malignancy. A literature review suggests that paraneoplastic syndrome occurs in up to 8% of cancer patients. […] Paraneoplastic syndromes may evolve from immunologic or non-immunologic mechanisms. […] An international neurologist panel developed criteria for paraneoplastic syndrome affecting the nervous system into definite and possible categories based on the classical neurological syndrome, presence of paraneoplastic antibodies, and timing of diagnosis of the occult malignancy. […] Paraneoplastic syndromes have diverse clinical manifestations, and hence prognosis may vary.

• #1 Epidemiology of paraneoplastic neurological syndromes: a population-based study – PubMed

  https://pubmed.ncbi.nlm.nih.gov/31552550/

  Background: The epidemiology of paraneoplastic neurological syndromes (PNS) remains to be defined. We present here the first population-based incidence study and report the clinical spectrum and antibody profile of PNS in a large area in Northeastern Italy. […] We performed a 9-year (2009-2017) population-based epidemiological study of PNS in the provinces of Udine, Pordenone and Gorizia, in the Friuli-Venezia Giulia region (983,190 people as of January 1, 2017). PNS diagnosis and subgroups were defined by the 2004 diagnostic criteria. Age- and sex-adjusted incidence rates were calculated. […] We identified 89 patients with a diagnosis of definite PNS. Median age was 68 years (range 26-90), 52% were female. The incidence of PNS was 0.89/100,000 person-years. PNS incidence rates increased over time from 0.62/100,000 person-years (2009-2011), 0.81/100,000 person-years (2012-2014) to 1.22/100,000 person-years (2015-2017). The prevalence of PNS was 4.37 per 100,000. […] This first population-based study found an incidence of PNS that approximates 1/100,000 person-years and a prevalence of 4/100,000. Moreover, the incidence of PNS is increasing over time, probably due to increased awareness and improved detection techniques.

• #1 Paraneoplastic syndromes of the nervous system – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/paraneoplastic-syndromes/symptoms-causes/syc-20355687

  Paraneoplastic syndromes of the nervous system are a group of rare conditions that develop in some people who have cancer. […] Paraneoplastic syndromes of the nervous system occur when cancer-fighting agents of the immune system also attack parts of the brain, spinal cord, peripheral nerves or muscle. […] Symptoms of paraneoplastic syndromes of the nervous system can develop relatively quickly, often over days to weeks. […] Symptoms vary depending on the body part being injured, and may include: […] Any cancer may be associated with a paraneoplastic syndrome of the nervous system. However, it occurs more often in people with cancers of the lung, ovary, breast, testis or lymphatic system. […] Paraneoplastic syndromes of the nervous system are not caused by cancer cells directly or by the cancer spreading, known as metastasis. […] Researchers believe that paraneoplastic syndromes of the nervous system are caused by cancer-fighting abilities of the immune system.

• #1 Paraneoplastic syndromes | MedLink Neurology

  https://www.medlink.com/articles/paraneoplastic-syndromes

  The incidence of paraneoplastic neurologic syndromes is estimated at around one case per 100,000 person-years, with a prevalence of four cases per 100,000. […] Many types of tumors have been associated with paraneoplastic disorders, but for most paraneoplastic neurologic syndromes, an overrepresentation of one or a few particular neoplasms is seen. […] Neuroblastoma and small-cell lung carcinoma (a type of neuroendocrine tumor) are the tumors most often associated with paraneoplastic phenomena in children and adults, respectively. […] Approximately 1% to 3% of patients with small cell lung carcinoma develop Lambert-Eaton myasthenic syndrome or another paraneoplastic syndrome. […] Other tumors overrepresented among patients with paraneoplastic syndromes include breast carcinoma, ovarian carcinoma, ovarian teratoma, Hodgkin lymphoma, thymoma, and testicular germ cell tumors.

• #1 Paraneoplastic neurological syndromes: a practical approach to diagnosis and management | Practical Neurology

  https://pn.bmj.com/content/22/1/19

  Overall, the clinical, serological and oncological features in an individual patient help to determine the clinical relevance of the syndrome and hence guide its management. […] Expert guidelines in 2021 have redefined aspects of these disorders in light of the description of novel antibodies and the most robust emergent clinical-serological-oncological associations. […] Tumour identification and appropriate oncological management are key to medical and neurological stabilisation, especially as many PNS associated with cancers display a limited immunotherapy response. […] The risk of death from the paraneoplastic syndrome was highest for patients with dysautonomic features; in this subgroup, nearly 60% died of neurological disease. […] Overall, these authors found PNS coexisted with 1 in 334 cancers, most commonly lung (17%), breast (16%) and lymphoma (12%).

• #1 Paraneoplastic Neurological Disorders | PM&R KnowledgeNow

  https://now.aapmr.org/neuromuscular-manifestations-of-neoplasms-and-paraneoplastic-syndromes/

  Population-based epidemiology studies have indicated that autoimmune disorders affecting the nervous system are not as rare as previously considered. Incidence rates are increasing over time, likely due to increased awareness and improved detection techniques. The incidence of paraneoplastic disorders varies with the neurologic syndrome and type of tumor. The more common syndromes are Lambert-Eaton myasthenic syndrome (LEMS), which affects approximately 3% of patients with small-cell lung cancer (SCLC), and myasthenia gravis (MG), which affects 15% of all patients with thymoma. However, it should be noted that not all cases of MG are considered to be paraneoplastic. One or more paraneoplastic neurologic disorders are present in up to 9% of patients with SCLC. […] After a paraneoplastic neurological syndrome has been diagnosed, computed tomography (CT) of the chest, abdomen, and pelvis with contrast should be performed as initial screening for associated malignances. Additional imaging studies such as mammogram or pelvic/scrotal US may be ordered as suggested by the clinical syndrome, identification of certain paraneoplastic antibodies, or other risk factors. If initial radiological assessment does not detect any malignancy, fluorodeoxyglucose positron emission tomography (FDG-PET) should be obtained, as it has been shown to have a higher sensitivity for occult malignancy compared with CT. If no tumor is detected at the time of PNS diagnosis, cancer surveillance is advised 3-6 months after initial negative screening, then every 6 months for 4 years in PNS with onconeural antibodies, with the exception of LEMS in which 2 years is sufficient.

• #1 Paraneoplastic Syndromes: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/280744-overview

  The reported frequency of paraneoplastic syndromes ranges from 10-15% to 2-20% of malignancies, However, these could be underestimates. Neurologic paraneoplastic syndromes are estimated to occur in fewer than 1% of patients with cancer. Similarly, the true incidence of deaths and complications related to paraneoplastic syndromes is unknown. […] No race predilection for paraneoplastic syndromes is reported. No sex predilection is known. People of all ages may be affected by cancers and their related paraneoplastic syndromes.

• #1 Paraneoplastic Cerebellar Degeneration: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/1156965-overview

  Paraneoplastic syndromes are a group of rare disorders that are triggered by an abnormal immune system response to an underlying malignant tumor. Patients with paraneoplastic neurological syndrome (PNS) most often present with neurologic symptoms before an underlying tumor is detected or coincide with the cancer diagnosis. […] These syndromes affect 13% of all cancer patients. […] In one study, paraneoplastic cerebellar degeneration (PCD) was observed in 25% of paraneoplastic neurologic syndromes, occurring in 2 of every 1000 patients with cancer. […] In a large-scale UK study of 1500 patients with progressive cerebellar ataxia, 3% were found to have PCD. […] In the study cited above, median survival duration was 100 months for patients with breast cancer and 22 months for those with gynecologic cancer. Although paraneoplastic cerebellar degeneration led to the diagnosis of cancer in 63% of patients, cancer progression was the cause of death in 52%. […] Both sexes are affected, but PCD is far more common in women than in men. […] Prognosis in paraneoplastic cerebellar degeneration (PCD) greatly depends on early detection of the underlying neoplasm and its stage at the time of detection.

• #1 Paraneoplastic neurological syndromes | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-2-22

  Paraneoplastic neurological syndromes (PNS) can be defined as remote effects of cancer that are not caused by the tumor and its metastasis, or by infection, ischemia or metabolic disruptions. PNS are rare, affecting less than 1/10,000 patients with cancer. Only the Lambert-Eaton myasthenic syndrome is relatively frequent, occurring in about 1% of patients with small cell lung cancer. […] PNS are rare diseases occurring in less than about 0.01% of patients with cancer. Only the Lambert-Eaton myasthenic syndrome is relatively frequent, occurring in about 1% of patients with small-cell lung cancer. […] In almost 80% of patients, the PNS antedates the diagnosis of cancer by several months to several years. Most tumors are diagnosed within 46 months. It is accepted that the risk of cancer development decreases significantly two years after diagnosing PNS and becomes very low after four years. All these data explain why it is often difficult to recognize a given neurological disorder as paraneoplastic.

• #1 Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

  https://www.mdpi.com/2227-9059/11/5/1406

  Importantly, nearly 80% of PNS patients show positive diagnostic screening for tumors at the initial assessment. These tumors might be identified with imaging including CT, duplex ultrasound, FDG-PET, and MRI. In selected conditions (for example germ-cell testicular neoplasms), imaging may be negative, and tumors are only revealed by histological examination. […] In PNS it is important to distinguish between the treatment of the underlying tumor, the treatment of the tumor-induced immune response, and the symptomatic treatment of the various PNS-related symptoms. Although not strictly within the aim of this review, the first step of PNS treatment is the oncological treatment (systemic or surgical) of the underlying tumor, when diagnosed, followed by the administration of immune treatments, when required.

• #1 Paraneoplastic Neurological Disorders | PM&R KnowledgeNow

  https://now.aapmr.org/neuromuscular-manifestations-of-neoplasms-and-paraneoplastic-syndromes/

  There is a paucity of evidence in the literature exploring environmental effects on the development and/or the progression of paraneoplastic syndromes. Those who are diagnosed with LEMS and are later found to have cancer will likely have a history of long-term smoking. For myasthenia gravis, medications including aminoglycosides, macrolides, beta-blockers, ACE inhibitors, quinidine, lidocaine, procainamide, phenytoin, prednisone, interferon, and D-penicillamine can cause exacerbation of symptoms. […] As PNS are rare, diagnosis can be difficult, resulting in misdiagnosis or delayed treatment. Neurological symptoms usually develop before the diagnosis of the tumor. Prompt oncologic treatment is beneficial. The failure of the neurologic syndrome to respond to treatment may be due to irreversible neuronal damage that occurred before treatment.

• #1 Paraneoplastic Neurological Syndromes of Central Nervous System | Encyclopedia MDPI

  https://encyclopedia.pub/entry/44943

  The prevalence of neurological irAEs (n-irAEs) is highly variable and may range from 1 to 12% according to different reports; they may involve both the central and peripheral nervous systems, although the latter is more frequently implicated (central: peripheral = 1:3). The challenge for clinicians is that the syndromes associated with ICIs meet diagnostic criteria for PNS, and all alternative etiologies (e.g., carcinomatous meningitis) must be excluded. […] A retrospective study has detected a significant increase of Ma2-associated PNS after the introduction of ICIs in France.

• #1 Paraneoplastic syndromes in small cell lung cancer – Soomro – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/38423/html

  An understanding of the paraneoplastic syndromes found in association with different cancers can provide an opportunity for early diagnosis and intervention. […] The presence and early identification of paraneoplastic syndromes can be beneficial in this context. […] A survival advantage has been demonstrated for patients that are diagnosed early at Stage 1a and received surgical resection. […] Patients with SCLC with paraneoplastic syndromes can present at an early stage of the disease, and offer an opportunity for the early diagnosis and early intervention leading to improved survival outcomes for patients with SCLC. […] Paraneoplastic syndromes in SCLC most frequently involve the endocrine and neurologic systems. […] The presence of these paraneoplastic syndromes in patients with an undiagnosed SCLC offers an opportunity for their early diagnosis and treatment improving their chances of survival.

• #1 Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

  https://www.mdpi.com/2227-9059/11/5/1406

  The traditional classification of PNS and related antibodies has been recently revised by a panel of experts which developed a new set of diagnostic criteria with the aim of improving the clinical management of these conditions. Accordingly, the previously-named “onconeural antibodies” (e.g., intracellular antibodies) are now called “high-risk antibodies” (e.g., associated with cancer in >70% of cases), and the NSA-Ab are now considered “intermediate-risk antibodies” (e.g., associated with cancer in 30–70% of cases) or “low-risk antibodies” (e.g., associated with cancer in <30% of cases). Moreover, PNS have been divided into different risk-related clinical phenotypes: “high-,” “intermediate-,” and “low-risk” for cancer. [...] The clinical diagnosis of PNS requires the exclusion of other, more frequent causes, namely infectious and non-neoplastic-induced autoimmune disorders, cancers (including focal lesions as well as carcinomatous meningitis), rapidly progressive neurodegenerative disorders (e.g., prion disease, dementias, and motor neuron diseases which may present similarly to anti-Ma2-associated syndromes), and toxic/metabolic conditions. In general, a neurological syndrome with a subacute onset should raise the index of suspicion for PNS. The presence of encephalopathic features associated with cognitive fluctuations (even within the same day) is another clue suggesting a possible autoimmune/paraneoplastic process.

• #1 Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

  https://www.mdpi.com/2227-9059/11/5/1406

  Paraneoplastic neurological syndromes pose a unique challenge to the neurologist. Our understanding of the phenomena, which lie at the intersection of neuroscience, immunology, and cancer biology, is constantly evolving. Although rare, with an observed incidence of approximately 0.2 to 1 per 100,000 persons per year, PNS is being diagnosed more frequently. The neurologist plays a critical role in this process: rapid identification of PNS can lead to earlier diagnosis of the underlying malignancy. […] With the present review, we have provided an updated summary of PNS of the CNS according to the most recent clinical and biomarker-based diagnostic criteria. This includes the new category of ICI-induced n-irAEs as well as an overview of the neurologic complications of CAR T-cell therapies. We have described the diagnostic and therapeutic work-up when investigating with probable, possible, and definite PNS based on clinical findings, neuroimaging and antibody testing, and we have discussed the role of specific antibodies in confirming the diagnosis of PNS and guiding the search for a hidden cancer.

• #1 Paraneoplastic Neurological Syndromes | SpringerLink

  https://link.springer.com/10.1007/978-3-031-24297-7_14-1

  Paraneoplastic neurological syndromes (PNS) are immune-mediated disorders triggered by cancer that may affect any level of the nervous system and often associated with autoantibodies targeting either intracellular or extracellular neural antigens. […] Recently, the diagnostic criteria for PNS have been updated in order to highlight the need for a clear causal relationship with cancer to confirm PNS, as well as the relevance of congruent clinical-immunological-oncological associations. […] PNS diagnosis largely relies on neural antibody detection, which must be conducted using the right specimens and techniques. […] Similarly, appropriate tumor screening should be undertaken, since most PNS are diagnosed before the underlying cancer is known. […] PNS physiopathology is so far poorly understood, but novel findings in certain types of PNS support that genetic alterations in the tumors may play an important role, leading to an abnormal expression of neural antigens followed by a crossed immune reaction between the nervous system and cancer.

• #2 Paraneoplastic neurological syndromes: a practical approach to diagnosis and management | Practical Neurology

  https://pn.bmj.com/content/22/1/19

  Paraneoplastic neurological syndromes (PNS) describe the remote neurological immune-mediated consequences of a systemic cancer. They affect ~1:300 patients with tumours, yet population-level epidemiology suggests an incidence rate of only between ~1 and 8/100 000 person-years, indicating ongoing under-recognition. […] A key feature of PNS is that the cancer triggers the immune response, and so it should express the autoantigen to which the immune response (including an autoantibody) is directed: this ensures a direct biological link between the cancer and PNS. […] High-risk clinical presentations are reflected by epidemiological studies which consistently identify autoantibody patterns with subacute cerebellar degeneration, encephalomyelitis, limbic encephalitis and sensory neuronopathy as the leading PNS in European cohorts.

• #2 Paraneoplastic Neurological Disorders | PM&R KnowledgeNow

  https://now.aapmr.org/neuromuscular-manifestations-of-neoplasms-and-paraneoplastic-syndromes/

  Population-based epidemiology studies have indicated that autoimmune disorders affecting the nervous system are not as rare as previously considered. Incidence rates are increasing over time, likely due to increased awareness and improved detection techniques. The incidence of paraneoplastic disorders varies with the neurologic syndrome and type of tumor. The more common syndromes are Lambert-Eaton myasthenic syndrome (LEMS), which affects approximately 3% of patients with small-cell lung cancer (SCLC), and myasthenia gravis (MG), which affects 15% of all patients with thymoma. However, it should be noted that not all cases of MG are considered to be paraneoplastic. One or more paraneoplastic neurologic disorders are present in up to 9% of patients with SCLC. […] After a paraneoplastic neurological syndrome has been diagnosed, computed tomography (CT) of the chest, abdomen, and pelvis with contrast should be performed as initial screening for associated malignances. Additional imaging studies such as mammogram or pelvic/scrotal US may be ordered as suggested by the clinical syndrome, identification of certain paraneoplastic antibodies, or other risk factors. If initial radiological assessment does not detect any malignancy, fluorodeoxyglucose positron emission tomography (FDG-PET) should be obtained, as it has been shown to have a higher sensitivity for occult malignancy compared with CT. If no tumor is detected at the time of PNS diagnosis, cancer surveillance is advised 3-6 months after initial negative screening, then every 6 months for 4 years in PNS with onconeural antibodies, with the exception of LEMS in which 2 years is sufficient.

• #2 Paraneoplastic Syndromes: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/280744-overview

  The reported frequency of paraneoplastic syndromes ranges from 10-15% to 2-20% of malignancies, However, these could be underestimates. Neurologic paraneoplastic syndromes are estimated to occur in fewer than 1% of patients with cancer. Similarly, the true incidence of deaths and complications related to paraneoplastic syndromes is unknown. […] No race predilection for paraneoplastic syndromes is reported. No sex predilection is known. People of all ages may be affected by cancers and their related paraneoplastic syndromes.

• #2 Paraneoplastic syndromes affecting spinal cord, peripheral nerve, and muscle – UpToDate

  https://www.uptodate.com/contents/paraneoplastic-syndromes-affecting-spinal-cord-peripheral-nerve-and-muscle

  Paraneoplastic neurologic syndromes are a heterogeneous group of neurologic disorders associated with systemic cancer and caused by mechanisms other than metastases, metabolic and nutritional deficits, infections, coagulopathy, or side effects of cancer treatment. These syndromes may affect any part of the nervous system from cerebral cortex to neuromuscular junction and muscle, damaging either one area or multiple areas. They can occur with or without detectable autoantibodies in serum and cerebrospinal fluid (CSF). […] Paraneoplastic syndromes affecting the spinal cord, dorsal root ganglia, peripheral nerve, neuromuscular junction, and muscle will be reviewed here. […] In a case series of 31 patients with an isolated progressive paraneoplastic myelopathy, the most common coexisting cancers were lung and breast; kidney cancer, thyroid cancer, gynecologic cancers, and melanomas were also present in a few patients. […] The study suggests that in patients with new-onset progressive myelopathy of unknown cause, the presence of symmetric, longitudinally extensive tract or gray matter-specific changes on spinal MRI should raise suspicion for a paraneoplastic etiology.

• #2 Paraneoplastic syndromes | MedLink Neurology

  https://www.medlink.com/articles/paraneoplastic-syndromes

  The incidence of paraneoplastic neurologic syndromes is estimated at around one case per 100,000 person-years, with a prevalence of four cases per 100,000. […] Many types of tumors have been associated with paraneoplastic disorders, but for most paraneoplastic neurologic syndromes, an overrepresentation of one or a few particular neoplasms is seen. […] Neuroblastoma and small-cell lung carcinoma (a type of neuroendocrine tumor) are the tumors most often associated with paraneoplastic phenomena in children and adults, respectively. […] Approximately 1% to 3% of patients with small cell lung carcinoma develop Lambert-Eaton myasthenic syndrome or another paraneoplastic syndrome. […] Other tumors overrepresented among patients with paraneoplastic syndromes include breast carcinoma, ovarian carcinoma, ovarian teratoma, Hodgkin lymphoma, thymoma, and testicular germ cell tumors.

• #2 Paraneoplastic neurological syndromes: a practical approach to diagnosis and management | Practical Neurology

  https://pn.bmj.com/content/22/1/19

  Overall, the clinical, serological and oncological features in an individual patient help to determine the clinical relevance of the syndrome and hence guide its management. […] Expert guidelines in 2021 have redefined aspects of these disorders in light of the description of novel antibodies and the most robust emergent clinical-serological-oncological associations. […] Tumour identification and appropriate oncological management are key to medical and neurological stabilisation, especially as many PNS associated with cancers display a limited immunotherapy response. […] The risk of death from the paraneoplastic syndrome was highest for patients with dysautonomic features; in this subgroup, nearly 60% died of neurological disease. […] Overall, these authors found PNS coexisted with 1 in 334 cancers, most commonly lung (17%), breast (16%) and lymphoma (12%).

• #2 Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

  https://www.mdpi.com/2227-9059/11/5/1406

  Paraneoplastic neurological syndromes (PNS) include any symptomatic and non-metastatic neurological manifestations associated with a neoplasm. PNS complicates approximately 1–15% of cancers, varying with the associated cancer type. PNS may precede the diagnosis of cancer by 1 to 5 years in up to 70% of patients. PNS of the CNS, despite being relatively rare, have been increasingly recognized and detected over time, with a constantly growing body of newly discovered antibodies and expanded interest among clinicians over the last few years. Hence, PNS of the CNS are often considered in the differential diagnoses in patients presenting with acute or subacute encephalopathy once the more common causes (e.g., infections, toxic and metabolic conditions, and even functional neurologic disorders or psychiatric disorders) are excluded.

• #2 Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

  https://www.mdpi.com/2227-9059/11/5/1406

  Importantly, nearly 80% of PNS patients show positive diagnostic screening for tumors at the initial assessment. These tumors might be identified with imaging including CT, duplex ultrasound, FDG-PET, and MRI. In selected conditions (for example germ-cell testicular neoplasms), imaging may be negative, and tumors are only revealed by histological examination. […] In PNS it is important to distinguish between the treatment of the underlying tumor, the treatment of the tumor-induced immune response, and the symptomatic treatment of the various PNS-related symptoms. Although not strictly within the aim of this review, the first step of PNS treatment is the oncological treatment (systemic or surgical) of the underlying tumor, when diagnosed, followed by the administration of immune treatments, when required.

• #2 Epidemiology of paraneoplastic neurological syndromes: a population-based study – PubMed

  https://pubmed.ncbi.nlm.nih.gov/31552550/

  Background: The epidemiology of paraneoplastic neurological syndromes (PNS) remains to be defined. We present here the first population-based incidence study and report the clinical spectrum and antibody profile of PNS in a large area in Northeastern Italy. […] We performed a 9-year (2009-2017) population-based epidemiological study of PNS in the provinces of Udine, Pordenone and Gorizia, in the Friuli-Venezia Giulia region (983,190 people as of January 1, 2017). PNS diagnosis and subgroups were defined by the 2004 diagnostic criteria. Age- and sex-adjusted incidence rates were calculated. […] We identified 89 patients with a diagnosis of definite PNS. Median age was 68 years (range 26-90), 52% were female. The incidence of PNS was 0.89/100,000 person-years. PNS incidence rates increased over time from 0.62/100,000 person-years (2009-2011), 0.81/100,000 person-years (2012-2014) to 1.22/100,000 person-years (2015-2017). The prevalence of PNS was 4.37 per 100,000. […] This first population-based study found an incidence of PNS that approximates 1/100,000 person-years and a prevalence of 4/100,000. Moreover, the incidence of PNS is increasing over time, probably due to increased awareness and improved detection techniques.

• #2 Paraneoplastic syndromes in small cell lung cancer – Soomro – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/38423/html

  An understanding of the paraneoplastic syndromes found in association with different cancers can provide an opportunity for early diagnosis and intervention. […] The presence and early identification of paraneoplastic syndromes can be beneficial in this context. […] A survival advantage has been demonstrated for patients that are diagnosed early at Stage 1a and received surgical resection. […] Patients with SCLC with paraneoplastic syndromes can present at an early stage of the disease, and offer an opportunity for the early diagnosis and early intervention leading to improved survival outcomes for patients with SCLC. […] Paraneoplastic syndromes in SCLC most frequently involve the endocrine and neurologic systems. […] The presence of these paraneoplastic syndromes in patients with an undiagnosed SCLC offers an opportunity for their early diagnosis and treatment improving their chances of survival.

• #3 Paraneoplastic syndromes | MedLink Neurology

  https://www.medlink.com/articles/paraneoplastic-syndromes

  The incidence of paraneoplastic neurologic syndromes is estimated at around one case per 100,000 person-years, with a prevalence of four cases per 100,000. […] Many types of tumors have been associated with paraneoplastic disorders, but for most paraneoplastic neurologic syndromes, an overrepresentation of one or a few particular neoplasms is seen. […] Neuroblastoma and small-cell lung carcinoma (a type of neuroendocrine tumor) are the tumors most often associated with paraneoplastic phenomena in children and adults, respectively. […] Approximately 1% to 3% of patients with small cell lung carcinoma develop Lambert-Eaton myasthenic syndrome or another paraneoplastic syndrome. […] Other tumors overrepresented among patients with paraneoplastic syndromes include breast carcinoma, ovarian carcinoma, ovarian teratoma, Hodgkin lymphoma, thymoma, and testicular germ cell tumors.

• #3 Paraneoplastic syndromes | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/paraneoplastic-syndromes?lang=us

  Paraneoplastic syndromes occur in up to 15% of patients with cancer. […] Paraneoplastic syndromes arise most commonly with small cell lung cancer as well as gynecological and hematological malignancies. […] Paraneoplastic syndromes can affect multiple systems and have a diverse presentation.

• #3 Paraneoplastic syndromes of the nervous system | PPT

  https://www.slideshare.net/slideshow/paraneoplastic-syndromes-of-the-nervous-system/235904940

  Paraneoplastic syndromes of the nervous system are a heterogeneous group of disorders that accompany benign or malignant tumors but are not directly related to mass effects or invasion. […] The document discusses the pathogenesis of paraneoplastic syndromes and antibodies associated with different syndromes. It provides information on incidence, diagnostic criteria, treatment approaches, prognosis, and testing considerations for paraneoplastic syndromes. […] One or more paraneoplastic neurologic disorder is present in up to 9% of patients with SCLC. Most commonly – LEMS, sensory neuronopathy, limbic encephalitis. […] Paraneoplastic peripheral neuropathy affects 5-15% of patients with plasma cell dyscrasias associated with malignant monoclonal gammopathies. […] Diagnostic criteria include definite and possible categories, with specific conditions outlined for each.

• #3 Paraneoplastic syndromes CNS manifestations | PPT

  https://www.slideshare.net/slideshow/paraneoplastic-syndromes-cns-manifestations-232575140/232575140

  Most PNS are mediated by immune responses triggered by neuronal proteins (onconeural antigens) expressed by tumors. […] Lambert-Eaton myasthenic syndrome (LEMS) Incidence Uncommon, true incidence unknown Occurs much less frequently than myasthenia gravis Middle-aged adults 50% of LEMS associated with a malignancy (small cell lung cancer (SCLC) […] 90% of affected patients are seropositive […] Aggressive search for a primary underlying malignancy (SCLC) is central.

• #3 Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

  https://www.mdpi.com/2227-9059/11/5/1406

  Paraneoplastic neurological syndromes pose a unique challenge to the neurologist. Our understanding of the phenomena, which lie at the intersection of neuroscience, immunology, and cancer biology, is constantly evolving. Although rare, with an observed incidence of approximately 0.2 to 1 per 100,000 persons per year, PNS is being diagnosed more frequently. The neurologist plays a critical role in this process: rapid identification of PNS can lead to earlier diagnosis of the underlying malignancy. […] With the present review, we have provided an updated summary of PNS of the CNS according to the most recent clinical and biomarker-based diagnostic criteria. This includes the new category of ICI-induced n-irAEs as well as an overview of the neurologic complications of CAR T-cell therapies. We have described the diagnostic and therapeutic work-up when investigating with probable, possible, and definite PNS based on clinical findings, neuroimaging and antibody testing, and we have discussed the role of specific antibodies in confirming the diagnosis of PNS and guiding the search for a hidden cancer.

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