Retroperitoneal Unicentric Castleman Disease: Surgery is Curative

Castleman disease (CD) comprises a group of rare nonclonal lymphoproliferative disorders. Unicentric Castleman disease (UCD) typically presents as isolated lymphadenopathy with progressive enlargement and an indolent course. Most patients are asymptomatic or may present symptoms related to a localized mass effect on organ function. Multicentric Castleman disease (MCD) affects various lymph node stations and leads to systemic constitutional symptoms and systemic cytokine dysregulation, resulting in laboratory abnormalities, hepatosplenomegaly, and complex organ dysfunction. We report a case of retroperitoneal UCD. Imaging studies showed a solitary, solid, right pararenal lesion causing anterior deviation of the inferior vena cava and renal pedicle. Complete surgical resection was performed, and histopathologic analyses confirmed the diagnosis of the hyaline-vascular subtype of UCD. Patients with UCD generally have an excellent prognosis following curative surgical resection. However, recurrence may occur, and long-term follow-up is advised.