경부와 종격동에 발생한 캐슬만씨 병(Castleman's Disease)

Castleman's Disease of the Neck and Mediastinum

Objectives: Castleman's disease(CD) is a lymphoproliferative disorder of unknown etiology. To elucidate the clinicopathologic characteristics of CD, we retrospectively reviewed our experience. Methods: Fifteen patients with CD of the neck and mediastinum were identified. Patients were divided into two groups: group I had an unicentric CD and group II had multicentric CD. The histology of CD was divided into 3 subtypes: hyaline-vascular(HV), plasma cell(PC), and mixed. Results: The study included 12 cases of group I, 3 cases of group II in the clinical aspect and 10 cases with HV, 3 cases with PC, 2 cases with mixed type in the histologic aspect. Of group I patients who underwent complete surgical excision, all are currently free of disease. The clinical course of group II patients was variable. Of two patients with multicentric plasma cell CD who were treated, one remain free from disease and the other had a local recurrence in the neck. One patient with multicentric mixed CD died of disease after 30 months of presentation. Conclusion: Surgical resection is recommend for patients with the unicentric CD, regardless of histologic subtype. Patients with multicentric disease do not benefit from surgical resection and should be candidates for multimodality therapy.