Castleman's disease

a variety of lymph node hyperplasias of unknown aetiology. There are many synonyms for this entity including angiofollicular lymph node hyperplasia, angiomatous lymphoid hamartoma and giant lymph node hyperplasia. Two histological forms of the disease have been described. The hyaline vascular type that is the most frequently observed is characterized by hypervascular hyaline germinal centres and extensive capillary proliferation in the nodes. Patients are young, and usually asymptomatic. Most of the patients have a single mass localized in any compartment of the mediastinum. The disease is benign with cure following complete surgical resection. On chest radiography and CT, calcifications may be present. On contrast-enhanced CT, the mediastinum mass shows contrast enhancement (Fig.1).

The plasma-cell variety of Castleman's disease presents as a multicentric process associated with generalized lymphadenopathy and hepatosplenomegaly. Multicentric Castleman's disease occurs in older patients than the localized disease, most being between 50 and 70 years old. It is often associated with systemic symptoms: fever, fatigue, anaemia, hypergammaglobulinaemia, infections, lymphoma or Kaposis sarcoma. On CT scans, the multicentric form is characterized by early, dense and uniform enhancement of enlarged mediastinal lymph nodes (lymphadenopathy mediastinal). Pericardial and pleural effusion, ascites, axillary, inguinal and abdominal lymphadenopathy, and splenomegaly may also be present. The multicentric form of the disease is usually progressive despite treatment.

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