Oligocentric Castleman disease, a rare presentation of an infrequent disease: case report

Enfermedad de Castleman oligocéntrica, una presentación rara de una enfermedad infrecuente: reporte de caso

Introduction: Castleman disease is a lymphoproliferative disorder characterized by tumor-like lesions predominantly involving B cells. Two main forms of the disease have been described: unicentric Castleman disease (UCD), characterized by the involvement of a single lymph node or lymph node chain, and multicentric Castleman disease (MCD), which affects multiple lymph node chains. Recently, an intermediate form between UCD and MCD has been described, known as oligocentric or regional Castleman disease (OCD), which involves 2 to 3 consecutive lymph node chains but does not meet the clinical or laboratory criteria to be classified as MCD. Clinical case: We present the case of a 39-year-old female patient who presented with a sensation of an abdominal mass in the left hypochondrium, without local or systemic symptoms, and without abnormalities in paraclinical studies. A CT scan revealed a hypodense, oval-shaped lesion with well-defined borders, located beneath the left renal hilum. Laparoscopic surgery was performed, during which a mass adherent to the retroperitoneum was found, involving the gonadal vein and 18 lymph nodes. Anatomopathological and immunohistochemical analyses confirmed the diagnosis of Castleman disease. The patient experienced a satisfactory recovery without the need for additional therapies. Conclusions: OCD is a recently described subtype whose treatment of choice is initially surgical, as in the clinical case presented, and if this is not possible, management with systemic medications is given. Further studies are needed to characterize epidemiology, risk factors, pathophysiology, clinical behavior, and treatment of OCD.