Castleman disease: Experience from a single institution

Abstract

Castleman disease (CD) describes a group of rare heterogeneous lymphoproliferative disorders characterized by enlarged hyperplastic lymph nodes. It is classified into unicentric CD (UCD) and multicentric CD (MCD). The present retrospective study examined the data of 11 patients with CD diagnosed and treated at a tertiary cancer center from 2017 to 2022. The median age of the study group was 41 years (range, 24 to 68 years). There were 8 males and 3 females. In total, 7 patients were diagnosed with UCD and 4 patients with MCD. The hyaline-vascular variant was the most common histology in both UCD and MCD. Among the 7 patients with UCD, 5 patients underwent excision, 1 patient underwent debulking followed by radiotherapy and 1 patient received single agent rituximab. Of the patients with UCD, 6 had a complete response (CR) and 1 patient had a partial response (PR). All 4 patients with MCD received systemic treatment, which included single agent rituximab (2 patients), rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone (RCHOP) (1 patient) and CHOP (1 patient). Among the patients with MCD, 1 patient attained a CR, 2 patients had a PR and 1 patient succumbed. The 3-year survival rate for the study population was 91%. In summary, CD is a rare disease occurring in immunodeficient patients. UCD is more common and is associated with better outcomes. Surgery is the mainstay of management in UCD whereas MCD requires combination chemotherapy.

Keywords: Castleman disease; human immunodeficiency virus-negative; multicentric; unicentric.

Figure 1

(A) CECT illustrating a mesenteric mass (patient no 4) (arrow). (B) CECT illustrating a posterior mediastinal mass (patient no 6) (arrow).

Figure 2

(A) Hyaline-vascular type: Lymphoid follicles can be observed with germinal centers, penetrated by sclerotic blood vessels (lollipop lesions) (indicated by asterisks) and broad mantle zones with concentric rings of small lymphocytes (onion skin pattern) (indicated by asterisks) (hematoxylin and eosin staining; magnification, x400). (B) Mixed type: Interfollicular area is shown with clusters of plasma cells highlighted by CD138 (indicated by asterisks) (hematoxylin and eosin staining; magnification, x400).