Castleman’s Disease

Epidemiology

Castleman’s disease (also known as localized nodular hyperplasia, angiofollicular lymph node hyperplasia, angiomatous lymphoid hamartoma, and giant lymph node hyperplasia) is a rare benign lymphoproliferative disorder that is characterized by lymph node hyperplasia. The mediastinum is most commonly involved, with the extracranial head and neck being the second most common site of involvement. The disease does not appear to have a sex or age predilection; however, isolated involvement of the neck is often reported in adolescents and young adults. The etiology is unknown and may be due to a hamartomatous, infectious, or inflammatory process. There are two distinct types of Castleman’s disease based on histology: hyaline vascular (90%) and plasma cell variant (10%).

Clinical Features

Patients with the hyaline vascular type of Castleman’s disease present with a neck mass but are otherwise asymptomatic. In addition to the palpable neck mass, approximately 50% of patients with the plasma cell variant will have systemic anomalies that include fever, elevated sedimentation rate, and hypergammaglobulinemia.