Work

Oligocentric Castleman disease: clinical characteristics and surgical outcomes from a single-centre retrospective study

Year: 2025

Type: article

Abstract: Introduction: Oligocentric Castleman Disease (OCD), a distinct subtype of Castleman Disease (CD) intermediate between Unicentric (UCD) and idiopathic Multicentric (iMCD) forms, remains poorly characterised. Methods: This study retrospectively analysed the clinical characteristics, treatment, and prognosis of 100 CD patients (63 UCD, 37 OligoCD). Results: Compared with UCD, OCD patients had a higher proportion of mixed type (Mixed-CD) histology and elevated CRP/ESR levels, along with significantly poorer Progression-Free Survival (PFS) (P = 0.0067). Within the OCD cohort, debulking surgery alone or combined chemotherapy achieved an 80.0% Complete Response (CR) rate; plasmacytic type(PC-CD), non-contiguous lesions, involvement of ≥3 regions, failure to achieve CR after initial treatment, and elevated baseline inflammatory markers were significant predictors of inferior PFS. Exploratory subgroup analysis divided the OCD cohort into asymptomatic and high-inflammatory groups with significantly different PFS (P = 0.042). The rate of progression to iMCD among surgically managed asymptomatic OCD patients in our study was similar to that in a large aMCD cohort predominantly managed with 'watch-and-wait', suggesting 'active surveillance' might be a more appropriate initial strategy for asymptomatic OCD. For the high-inflammatory subgroup, characterised by higher PC-CD rates and more widespread disease distribution despite effective symptom control with surgery, the post-operative relapse risk was higher. Discussion: In conclusion, debulking surgery is effective for alleviating symptoms in OCD but may be unnecessary for asymptomatic patients; factors associated with a hyper-inflammatory state predict relapse, underscoring the need for careful treatment planning and exploration of novel therapeutic strategies for this high-risk subgroup.

Source: Frontiers in Immunology

DOI: 10.3389/fimmu.2025.1637792