Pediatric multicentric Castleman disease; a case report

Abstract

Castleman disease (CD) is a rare B-cell lymphoproliferative disorder of unknown etiology characterized by benign lymphoid follicular hyperplasia and capillary proliferation. CD can be divided histologically into four different variants: hyaline-vascular (HV), plasma-cell (PC), mixed and plasmablastic types. The unicentric CD (UCD) is localized lymphadenopathy while multicentric CD (MCD) is a systemic disorder involving cytokine-induced polyclonal lymphoproliferation and systemic inflammation due to overexpression of interleukine-6, and can be idiopathic or associated with HIV/HHV-8 infection. Surgical removal is the gold standard for patients with unicentric CD, while multicentric CD requires systemic therapy. In our presentation we wanted to show that even multicentric type of CD can be treated surgically only and that this surgical approach can be therapeutic and not mutilating at the same time. As in every rare disease, the recording of cases in rare disease registries is essential. Accumulation of the cases enables the evaluation of treatments and may provide a solid base for guidelines update.