Castleman disease: clinical features, pathology, and treatment outcomes in 12 cases

To conduct a retrospective descriptive analysis of the clinical manifestations, pathological characteristics, diagnostic approaches, and treatment outcomes of Castleman disease (CD) in a Chinese regional population, thereby contributing real-world evidence to improve clinical management of this rare disorder.

A retrospective analysis was performed on 12 patients with CD diagnosed via a rigorous three-step differential diagnosis protocol at Linyi People's Hospital between February 2020 and February 2024. The cohort included 10 males and 2 females, age range 20-67 years (median, 45 years). Tumor locations involved retroperitoneum, pelvis, neck, supraclavicular region, axilla, and inguinal region. According to the Frizzera classification, 7 patients were diagnosed with unicentric CD (UCD; 5 hyaline vascular type, 2 mixed type) and 5 with multicentric CD (MCD; 4 plasma cell type, 1 mixed type). All patients tested negative for HHV-8 and HIV. Treatment modalities included surgical resection alone (7 UCD cases), surgical resection followed by systemic therapy (1 MCD mixed-type case), and primary R-CHOP chemotherapy (4 MCD plasma cell type cases).

All 12 patients were alive at follow-up. The 7 UCD patients treated with complete surgical resection alone experienced no recurrence. The MCD mixed-type patient (Case 8) initially received rituximab for 3 months postoperatively, but interleukin-6 (IL-6) levels remained elevated; the regimen was switched to oral thalidomide-cyclophosphamide-dexamethasone (TCD) for 2 months, leading to complete remission. The 4 MCD patients treated with primary R-CHOP achieved favorable outcomes with no recurrence during follow-up (range, 10-45 months).

In this small cohort, CD was associated with a favorable short-term prognosis following individualized treatment. Complete surgical resection is curative for resectable UCD, whereas MCD typically requires systemic therapy. In resource-limited settings where anti-IL-6 monoclonal antibodies are not readily accessible, R-CHOP represents an effective first-line alternative for idiopathic multicentric Castleman disease (iMCD), and the oral TCD regimen is a valuable option for refractory cases. These findings provide practical evidence to guide clinical decision-making for CD in regional medical centers.