Castleman's disease with diffuse cervical localisation: case report

Castleman's disease is a rare disorder of the lymphoid tissue with a predominantly mediastinic localisation, but possible in any lymph node or extra-lymph node site. The aetiopathogenesis remains to be defined and, in the literature, only just over 500 cases have been reported, with only 57 located in a cervical site. Clinically, a solitary or localised form and a systemic or multicentric form can be distinguished, whilst, from a histological viewpoint, this lesion may be of the hyaline vascular or plasma-cellular type. Even if all the cases localised in a cervical site have been described as a single mass, the case described herein refers to diffuse cervical lymph-node hyperplasia with histological characteristics of the hyaline-vascular type, with the presence of a plasmacellular component. Evolution, over the 7 years following diagnosis, was benign, despite the fact that the patient was not submitted to surgical treatment. The low titre of B and T4 lymphocytes would appear to indicate that Castleman's disease might be an immunological disorder, due to atypical hyperplasia of the lymphoid tissue. It is suggested that Castleman's disease, in the cervical site, be classified into two subtypes: a cervical form, presenting as a single mass, and a multicentric cervical form. Data from a review of the literature are discussed.