1166 Thymoma and Good's Syndrome: Analysing the Role of Surgery in a Case Series

Abstract

Abstract Background Good’s syndrome is a rare paraneoplastic phenomenon with unknown pathogenesis in patients with thymoma. The incidence is 0.15 cases per 100,000 population per year. Patients typically have hypogammaglobulinemia, reduced B cell and CD4+ T cell count, and present with recurrent infections and autoimmune manifestations. Case Summary This case series highlights two patients with Good’s syndrome who underwent thymectomy in a United Kingdom Thoracic Surgery Unit. Patient 1, a 57-year-old female, presented with a history of recurrent infections (including Pneumocystis jiroveci), dysphagia, and dyspnoea. The subsequent discovery of a thymoma led to a diagnosis of Good Syndrome. Patient 2, a 61-year-old male, exhibited recurrent infections and immunological abnormalities, eventually leading to the identification of a thymoma. Both patients were diagnosed in their 5th decade, and shared similar clinical profiles with absent B cells, reduced NK cells, hypogammaglobulinemia, and an anterior thymoma. Preoperatively, they received IVIG transfusions. The surgical intervention involved robotic-assisted thymectomy for both patients. Histopathological assessments classified Patient 1 as T1bNxMxR1 and Patient 2 as T1aNXMXR1 providing insights into the extent of the thymomas. Post-operatively they continue to have prophylactic antibiotics and IVIG and have been reportedly well. Conclusions Patients with Good Syndrome are at significant risk of opportunistic infections and may present repeatedly to medical specialties. Thymoma and its associated symptoms can precede immunodeficiency. Consequently, thorough consideration of this diagnosis, interdisciplinary collaboration, and preoperative planning is vital for the effective management of this extremely rare syndrome. Successful outcomes are achievable through timely IVIG and full surgical resection.