Castleman's disease

Abstract

Castleman's disease, a type of lymph node hyperplasia, usually occurs in the mediastinum and rarely presents in the cervical region as an asymptomatic solitary mass. Clinically, they are of two types-solitary and multi-centric. Most of the solitary types are asymptomatic with no associated symptoms, whereas the multi-centric type is associated with systemic symptoms and has a poor prognosis. Histologically, they can be classified as-Hyaline vascular, plasma cell, transitional and stromal rich type. We report a case of Castleman's disease involving the submandibular lymph node in a 75-year-old male patient whose definitive diagnosis was made only on histological examination. Isolated Castleman's disease of the submandibular node is rare and a thorough clinical and histological examination is necessary to rule out the systemic form of the disease and other diseases with manifestations as a cervical lymph node enlargement.

Keywords: Angiolymphoid hyperplasia; Castleman's disease; cervical lymphadenopathy.

Figure 1

Ultrasonography pictures of the sub- mandibular region

Figure 2

Microscopic appearance of the lymph node (H&E, ×40, original magnification)

Figure 3

Vessels with hyalinized walls and proliferating endothelial cells surrounded by concentric layers of lymphocytes (onion skin pattern)

Figure 4

Small capillaries were seen both within the residual lymphoid aggregates and in the fibrous connective tissue

Figure 5

Pathophysiology of interleukin 6 in Castleman disease. Excess interleukin 6 in Castleman disease patients results in increased B-lymphocyte growth, lymph node vascularity and inflammatory response. Autoimmune phenomena may also be present

Figure 6

Schematic representation of the pathogenesis of the disease

Figure 7

Schematic presentation of the treatment plan in Castleman disease. Pred: Prednisolone, Comb. Chem: Combined chemotherapy