The heterogeneity of thoracic Castleman disease: implications for classification and personalized management

胸部Castleman病的异质性:对分类和个体化治疗的启示

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Abstract

Castleman disease (CD) is a rare lymphoproliferative disorder categorized into unicentric CD (UCD) and multicentric CD (MCD). Surgical intervention is crucial for both diagnosis and treatment. However, comprehensive comparisons and detailed analyses of surgical outcomes remain limited. We retrospectively reviewed thoracic CD patients treated at our hospital from 2010 to 2024. Clinical, pathological, laboratory, and imaging data were compared between UCD and MCD. Treatment outcomes and prognoses were assessed using multivariate Cox regression. We also compared outcomes between video-assisted thoracoscopic surgery (VATS) and open thoracotomy. Among 88 CD patients, 26.1% were diagnosed with MCD and 73.9% with UCD. MCD patients were significantly older (mean age: 50.1 vs. 40.4, p = 0.002), had a higher male predominance (65.2% vs. 35.4%, p = 0.025), exhibited a greater prevalence of plasma cell-type pathology (47.8% vs. 4.6%, p < 0.001), and demonstrated a higher comorbidity index (2.48 vs. 1.14, p < 0.001) compared to UCD patients. MCD patients also had worse 5-year progression-free survival (81.6% vs. 100%, p < 0.001). Surgical UCD patients had better outcomes than non-surgical ones (p < 0.001). Multivariate analysis identified diabetes (HR = 9.9) and clinical subtype (HR = 15.8) as independent prognostic factors. VATS resulted in less bleeding (109 mL vs. 522 mL) and shorter hospital stays (4 vs. 7 days) than thoracotomy, though complication rates and long-term outcomes were similar. UCD and MCD exhibit distinct clinical and pathological profiles, necessitating different treatment approaches and resulting in varying prognoses. Individualized treatment plans should be tailored based on the type, location, and size of the lesion in each patient.

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