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The heterogeneity of thoracic Castleman disease: implications for classification and personalized management
Yuansheng Zheng1, Junkan Zhu1, Jiahao Jiang1
1Department of Thoracic Surgery, Zhongshan Hospital, Fudan University, No. 180, Fenglin Road, Shanghai, 200032, China.
Insights
Castleman disease (CD) subtypes, unicentric (UCD) and multicentric (MCD), show distinct clinical and pathological features. MCD patients have poorer prognoses, highlighting the need for individualized treatment strategies for rare lymphoproliferative disorders.
Area of Science:
- Thoracic surgery
- Oncology
- Hematology
Background:
- Castleman disease (CD) is a rare lymphoproliferative disorder with unicentric (UCD) and multicentric (MCD) subtypes.
- Surgical intervention is vital for CD diagnosis and treatment, yet comparative outcome data are limited.
Purpose of the Study:
- To retrospectively compare clinical, pathological, and surgical outcomes between UCD and MCD patients.
- To identify prognostic factors and compare surgical approaches (VATS vs. thoracotomy).
Main Methods:
- Retrospective review of 88 thoracic CD patients (2010-2024).
- Comparison of clinical, pathological, laboratory, and imaging data between UCD and MCD.
- Multivariate Cox regression for prognostic factor analysis and comparison of VATS versus open thoracotomy outcomes.
Main Results:
- MCD patients were older, more frequently male, had more plasma cell pathology, and higher comorbidity index than UCD patients.
- MCD showed worse 5-year progression-free survival (81.6% vs. 100%).
- Diabetes and clinical subtype were independent prognostic factors. VATS offered reduced bleeding and shorter hospital stays compared to thoracotomy.
Conclusions:
- UCD and MCD exhibit distinct profiles, requiring tailored treatment approaches.
- Prognoses vary significantly between UCD and MCD, emphasizing the need for individualized care.
- Surgical intervention improves outcomes for UCD, and VATS presents advantages over thoracotomy.
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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