Influence of ectopic thymic tissue on clinical outcome following extended thymectomy in generalized seropositive nonthymomatous myasthenia gravis

Ponseti, José M.; Gamez, Josep; Vilallonga, Ramón; Ruíz, Carmen Anarte; Azem, Jamal; López‐Cano, Manuel; Armengol, Manel

doi:10.1016/j.ejcts.2008.07.049

Cited by 49 publications

(24 citation statements)

References 23 publications

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“…24 In some studies, ectopic thymus has had a negative effect on outcomes at a follow-up of more than 7 years. 30 Furthermore, numerous reports describe patients with myasthenia gravis as not having clinical improvement after incomplete thymic resections. 31–33 Randomized trials to compare resectional techniques are needed.…”

Section: Discussionmentioning

confidence: 99%

Randomized Trial of Thymectomy in Myasthenia Gravis

et al. 2016

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BACKGROUND Thymectomy has been a mainstay in the treatment of myasthenia gravis, but there is no conclusive evidence of its benefit. We conducted a multicenter, randomized trial comparing thymectomy plus prednisone with prednisone alone. METHODS We compared extended transsternal thymectomy plus alternate-day prednisone with alternate-day prednisone alone. Patients 18 to 65 years of age who had generalized nonthymomatous myasthenia gravis with a disease duration of less than 5 years were included if they had Myasthenia Gravis Foundation of America clinical class II to IV disease (on a scale from I to V, with higher classes indicating more severe disease) and elevated circulating concentrations of acetylcholine-receptor antibody. The primary outcomes were the time-weighted average Quantitative Myasthenia Gravis score (on a scale from 0 to 39, with higher scores indicating more severe disease) over a 3-year period, as assessed by means of blinded rating, and the time-weighted average required dose of prednisone over a 3-year period. RESULTS A total of 126 patients underwent randomization between 2006 and 2012 at 36 sites. Patients who underwent thymectomy had a lower time-weighted average Quantitative Myasthenia Gravis score over a 3-year period than those who received prednisone alone (6.15 vs. 8.99, P<0.001); patients in the thymectomy group also had a lower average requirement for alternate-day prednisone (44 mg vs. 60 mg, P<0.001). Fewer patients in the thymectomy group than in the prednisone-only group required immunosuppression with azathioprine (17% vs. 48%, P<0.001) or were hospitalized for exacerbations (9% vs. 37%, P<0.001). The number of patients with treatment-associated complications did not differ significantly between groups (P=0.73), but patients in the thymectomy group had fewer treatment-associated symptoms related to immunosuppressive medications (P<0.001) and lower distress levels related to symptoms (P=0.003). CONCLUSIONS Thymectomy improved clinical outcomes over a 3-year period in patients with nonthymomatous myasthenia gravis. (Funded by the National Institute of Neurological Disorders and Stroke and others; MGTX ClinicalTrials.gov number, NCT00294658.)

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Section: Discussionmentioning

confidence: 99%

Randomized Trial of Thymectomy in Myasthenia Gravis

et al. 2016

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“…The difference between both groups is close to significance ( p = 0.06) that might indicate that the transcervicalsubxiphoid-VATS maximal thymectomy is more complete than extended transsternal thymectomy. An incidence of ectopic foci of thymic tissue in the adipose tissue of the neck and the mediastinum reported by Jaretzki et al was 98.0%, by Masaoka et al 72.2%, by Ponseti et al 42.2% and by Ashour 39.5% [16,[23][24][25].…”

Section: Commentmentioning

confidence: 98%

Comparison of complete remission rates after 5 year follow-up of three different techniques of thymectomy for myasthenia gravis☆

Zieliński¹,

Hauer²,

Hauer³

et al. 2010

European Journal of Cardio-Thoracic Surgery

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(1) The results of complete remission rates after 5-year follow-up were statistically better in patients with myasthenia gravis (MG), who were operated on with extended transsternal thymectomy and transcervical-subxiphoid-videothoracoscopic 'maximal' thymectomy than the patients who underwent basic transsternal thymectomy. (2) The difference can be explained by the removal of ectopic foci of the thymic tissue from the neck and the mediastinum in these patients.

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“…Considering this biological background of EOMG and the known wide‐spread distribution of inflamed thymic tissue across the cervical and mediastinal region in many EOMG patients, researchers agreed that early 39 and total (“maximal”) transcervical–transsternal thymectomy 28 would be desirable 3,40,41 . However, practical considerations that are mainly centered on the prevention of potential severe side effects of total thymectomy (like phrenic nerve injury) have made the slightly less invasive “extended thymectomy” the most feasible surgical technique in EOMG, 3 though with an ongoing debate whether the standard transsternal approach or recently introduced minimally invasive procedures yield better results for MG outcome 3,42–44 …”

Section: Historic Overview On Thymectomy In Myasthenia Gravis and Thementioning

confidence: 99%

Thymus pathology observed in the MGTX trial

Marx

Pfister

Schalke

et al. 2012

Annals of the New York Academy of Sciences

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The MGTX trial is the first prospective, randomized clinical trial that aims to evaluate the impact of extended transsternal thymectomy on myasthenic symptoms, prednisone requirements, and quality of life in patients with nonthymomatous, anti-acetylcholine receptor autoantibody-positive myasthenia gravis (MG). Here, we give an overview of the rationale of thymectomy and the standardized macroscopic and histopathological work-up of thymectomy specimens as fixed in MGTX standard operating procedures, including the grading of thymic lymphofollicular hyperplasia and the morphometric strategy to assess thymic involution.

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Influence of ectopic thymic tissue on clinical outcome following extended thymectomy in generalized seropositive nonthymomatous myasthenia gravis

Abstract: The clinical outcome of patients with nonthymomatous seropositive myasthenia gravis is significantly affected by the presence of ectopic thymic tissue in the mediastinal fat.

Cited by 49 publications

References 23 publications

Randomized Trial of Thymectomy in Myasthenia Gravis

Randomized Trial of Thymectomy in Myasthenia Gravis

Comparison of complete remission rates after 5 year follow-up of three different techniques of thymectomy for myasthenia gravis☆

Thymus pathology observed in the MGTX trial

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