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EP.14.16 Clinical Characteristics, Surgical Risks, ...
EP.14.16 Clinical Characteristics, Surgical Risks, and Long-Term Prognosis of Good's Syndrome: A Retrospective Analysis of 108 Thymoma Cases
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This retrospective study analyzed 108 thymoma cases, including seven with Good’s syndrome (GS), at Hokkaido University Hospital from July 2007 to January 2024. GS is a rare immunodeficiency characterized by thymoma-associated hypogammaglobulinemia leading to increased susceptibility to infections. The study aimed to assess clinical features, surgical risks, and long-term prognosis of GS patients undergoing thymectomy.<br /><br />GS patients had significantly larger tumors and all were WHO histological type AB thymomas, with no invasive characteristics compared to non-GS cases. Surgical approaches included median sternotomy (MS), video-assisted thoracoscopic surgery (VATS), and robotic-assisted thoracoscopic surgery (RATS). Perioperative surgical risks and complication rates did not significantly differ between GS and non-GS groups across all surgical methods, suggesting comparable safety profiles.<br /><br />Despite similar immediate surgical outcomes, GS patients faced elevated long-term risks. Persistent immunodeficiency led to frequent severe infections postoperatively, including cytomegalovirus (CMV) disease and opportunistic fungal infections. Some GS patients developed autoimmune conditions and extrathymic malignancies, consistent with known T-cell dysfunction in GS. The Kaplan-Meier survival analysis showed significantly poorer overall survival in GS patients (hazard ratio 7.23, p=0.002) than non-GS patients.<br /><br />In conclusion, thymectomy in GS patients is feasible with perioperative risks similar to non-GS thymomas. However, due to underlying immune defects, GS patients remain vulnerable to severe infections and new malignancies over the long term, causing poor prognosis. Careful and continuous postoperative monitoring focusing on infection control and malignancy surveillance is essential to optimize outcomes in this population. This study underlines the importance of multidisciplinary management of GS beyond surgical treatment alone.
Asset Subtitle
Shinya Otsuka
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Speaker
Shinya Otsuka
Topic
Mesothelioma, Thymoma, and Other Thoracic Tumors
Keywords
Thymoma
Good's syndrome
Hypogammaglobulinemia
Thymectomy
Immunodeficiency
Surgical risks
Long-term prognosis
Infections
Autoimmune conditions
Multidisciplinary management
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