Atypical lymphoproliferative disorders. Castelmann’s disease. Case report and review of the literature.

Castleman's disease (CD) is a rare atypical lymphoproliferative disorder characterized by enlarged hyperplastic lymph nodes with striking vascular proliferations. CD is categorized as being either localized or disseminated and further subdivided into hyaline-vascular, plasma cell, or mixed histopathological patterns. Here we report a case of CD in a 15-year-old girl who presented with a solitary asymptomatic hyaline-vascular mass in the right supraclavicular space. In addition, we discuss the pathogenesis, clinical features and reported co-morbidities of unicentric and multicentric CD and evaluate effective treatment strategies based on the results of lymph node biopsy and careful staging. Surgical excision is curative for the localized variants of CD, either hyaline-vascular or plasma cell type. If complete resection is not possible, partial resection or radiotherapy may be useful to control possible systemic manifestations. Multicentric CD, regardless of the histological subtype, is a more aggressive clinical entity, commonly with a chronic or rapidly fatal course. Patients with multicentric CD do not benefit from surgical treatment and should be candidates for systemic therapy (steroids, combination chemotherapy, novel therapies), although this is still in a fairly experimental phase.