A systematic review of paraneoplastic syndromes associated with thymoma: Treatment modalities, recurrence, and outcomes in resected cases

Zhao, Jasmine; Bhatnagar, Vikrant; Ding, Li; Atay, Scott M.; David, Elizabeth A.; McFadden, P. Michael; Stamnes, Stephanie; Lechtholz-Zey, Elizabeth; Wightman, Sean C.; Detterbeck, Frank C.; Kim, Anthony W.

doi:10.1016/j.jtcvs.2019.11.052

Cited by 51 publications

(53 citation statements)

References 429 publications

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“…Among the autoimmune targets, striated muscle proteins prevail as reflected by the fact that myasthenia gravis (MG) due to autoantibodies to the Acetylcholine Receptor (AChR) and striational autoantigens (e.g., Titin, skeletal and cardiac Ryanodine Receptors (RYRs)) is the leading thymomaassociated autoimmune disease [8]. However, almost any other organ-specific (e.g., thyroid, hepatic, renal) and systemic autoimmune disease (e.g., SLE, RA) can occur either in isolation or combined with MG or other autoimmune diseases [7,9]. The pathogenesis of most thymoma-associated autoimmune diseases is unknown.…”

Section: Autoimmunity and Expression Of Immune Checkpoint Moleculesmentioning

confidence: 99%

Molecular pathology of thymomas: implications for diagnosis and therapy

et al. 2021

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Thymomas exhibit a unique genomic landscape, comprising the lowest on average total mutational burden among adult human cancers; a unique point mutation in the GTF2I gene in WHO type A and AB thymomas (and rarely others); almost unique KMT2A-MAML2 translocations in rare WHO type B2 and B3 thymomas; a unique YAP1-MAML2 translocation in almost all metaplastic thymomas; and unique miRNA profiles in relation to GTF2I mutational status and WHO histotypes. While most thymomas can be diagnosed solely on the basis of morphological features, mutational analyses can solve challenging differential diagnostic problems. No molecular biomarkers have been identified that predict the response of unresectable thymomas to chemotherapy or agents with known molecular targets. Despite the common and strong expression of PDL1 in thymomas, immune checkpoint inhibitors are rarely applicable due to the poor predictability of common, life-threatening autoimmune side effects that are related to the unrivaled propensity of thymomas towards autoimmunity.

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Section: Autoimmunity and Expression Of Immune Checkpoint Moleculesmentioning

confidence: 99%

Molecular pathology of thymomas: implications for diagnosis and therapy

et al. 2021

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“…In a national analysis, Yang and colleagues 36 demonstrated shorter length of stay following minimally invasive thymectomy for stage I through III thymoma, compared with open thymectomy, without increased rates of margin positivity, perioperative morbidity, or decreases in overall survival. Kim and colleagues 37 performed a useful systematic analysis of the paraneoplastic syndromes associated with thymoma and showed that a multimodal treatment approach that includes resection is able to achieve remission of the paraneoplastic syndrome in the majority (76%) of patients, which is associated with improved overall survival.…”

Section: Mediastinummentioning

confidence: 99%

A year in general thoracic surgery published in the Journal of Thoracic and Cardiovascular Surgery: 2020

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et al. 2021

The Journal of Thoracic and Cardiovascular Surgery

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“…While some cases of MG are associated with thymomas and are regarded as paraneoplastic, 7,8 the etiology of MG in most cases is unknown and might influence the incidence and prevalence of the disorder. Thus, the epidemiology of MG has the potential to provide information on the possible causes of the disease and, therefore, has attracted much attention.…”

Section: Introductionmentioning

confidence: 99%

Incidence of AChR Ab‐positive myasthenia gravis in Israel: A population‐based study

et al. 2020

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Background The incidence of myasthenia gravis (MG) has traditionally been low, ranging between 2‐6/106. Several recent epidemiological studies have reported a higher incidence. We, therefore, aimed to assess and characterize the incidence of MG in Israel. Methods We retrospectively reviewed the records of all four laboratories that performed the acetylcholine receptor antibody (AChR Ab) test in Israel between 1994 and 2013 and documented the number of newly diagnosed seropositive MG patients each year. To assure that data indeed reflect only newly diagnosed patients, patient's names and ID numbers were screened at the Hadassah medical center database since 1978, the year when the test was first performed in Israel. In order to calculate the annual incidence of the disease, the population at risk was derived from the annual publication of the Israeli Central Bureau of Statistics. Results The annual incidence of MG for this time period was 13.1/106 inhabitants. The mean incidence of MG between 1994 and 2003 was 7.695/106/y, while the mean incidence between 2004 and 2013 was 18.49/106 (P < .0001). Mean age of diagnosis between 1994 and 2003 was 56.65 ± 0.9351, while between 2004 and 2013, it was 59.89 ± 0.5336 (P = .0012). Male to female (M:F) incidence ratio in the years 1994‐2003 and 2004‐2013 was 2:3.2 and 3:1.8, respectively, reflecting increased incidence among males (P < .0001). Conclusions The incidence of MG in Israel has increased significantly during the last decade, especially among males of older age. These findings may reflect an etiological role of an environmental factor, increased awareness, and increased longevity in general.

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A systematic review of paraneoplastic syndromes associated with thymoma: Treatment modalities, recurrence, and outcomes in resected cases

Cited by 51 publications

References 429 publications

Molecular pathology of thymomas: implications for diagnosis and therapy

Molecular pathology of thymomas: implications for diagnosis and therapy

A year in general thoracic surgery published in the Journal of Thoracic and Cardiovascular Surgery: 2020

Incidence of AChR Ab‐positive myasthenia gravis in Israel: A population‐based study

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