The localized variant of Castleman disease presents as a mass
lesion. The mediastinum, abdomen, and neck are the sites most commonly
affected. Lesions up to 16 cm in diameter have been reported. Constitutional
symptoms are most commonly seen in the plasma cell and multicentric forms.
Symptoms include asthenia, weight loss, fever, skin rash, hemolytic anemia,
and/or hypergammaglobulinemia. Hepatomegaly and/or splenomegaly are present
in approximately 75% of cases. In approximately 25% of
patients, the disease may also present with features of PEP (plasma cell
dyscrasia, endocrinopathy, polyneuropathy) syndrome. Amyloidosis has been
described in a number of cases. Patients with multicentric disease are prone to infections and
malignancies. Treatment is surgical excision where possible (localized
variant). Surgery, steroids, and chemotherapy are mainly used for the
multicentric variant, which has a poor prognosis. Paraneoplastic pemphigus vulgaris has been described in some patients suffering from Castleman disease. The disease in the
pediatric population appears to have a more favorable course than in adults.