CASTLEMAN'S DISEASE IN CHILDHOOD: A CASE REPORT

Castleman's Disease (CD) is a rare disease characterized by lymph node hyperplasia described by Benjamin Castleman in 1956.

CD is divided into subtypes based on its etiology, pathology, involved lymph nodes number, and regions. Mediastinal lymph

nodes are often affected, but any lymph node may be involved, including the neck, axilla, and abdomen. CD is initially classified

as Unicentric (UCD) and Multicentric (MCD) Castleman's Disease based on the number of regions of enlarged lymph nodes.

CD is histopathologically divided into three types; hyaline vascular, plasma cell, and mixed type. There are two subtypes of

MCD; HHV-8 (hMCD) associated and idiopathic (iMCD). The etiology of UCD and iMCD has not been clarified. Four

possible causes (virus, acquired or inherited genetic mutation, autoimmunity) are considered. The diagnosis of CD is

problematic because it mimics other neoplastic and autoimmune diseases. Excision of the mass and histopathological

examination is the gold standard in diagnosis. The prognosis changes according to the localization and multicentricity of the

lesion. Although surgery is the first-line treatment option, chemo-radiotherapy and rituximab are also used. Since all Castleman

Disease types, reactive and neoplastic lymph node pathologies are rare in childhood, Castleman Disease in childhood should

be defined in more detail and its differential diagnosis should be made carefully. This article discusses Hyalinized Vascular

Type Castleman Disease in the supraclavicular lymph node in a 14-year-old female patient, which is very rare especially in

children, in light of current literature.