CASTLEMAN'S DISEASE IN CHILDHOOD: A CASE REPORT


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Ergül B., Özmen S., Şipal S.

Atatürk Üniversitesi Tıp Fakültesi Cerrahi Tıp Bilimleri Dergisi, cilt.1, sa.3, ss.125-128, 2022 (Hakemli Dergi)

Özet

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.