Unicentric Castleman disease in the mediastinum

• Main Authors: Samantha J. Wala, Erica M. Fallon, Christopher J. Forlenza, Neerav Shukla, Michael P. LaQuaglia
• Format: Article
• Language: English
• Published: Elsevier 2018-07-01
• Series: Journal of Pediatric Surgery Case Reports
• Subjects: Castleman disease; Pediatrics; Hyaline vascular variant; Middle mediastinum; Mediastinal mass; Thoracotomy
• Online Access: http://www.sciencedirect.com/science/article/pii/S2213576618300976

Castleman disease (CD) is a benign lymphoproliferative disorder that rarely occurs in the pediatric population. This entity arises as either unicentric CD or multicentric CD, and is histopathologically classified as hyaline vascular, plasma cell, or mixed variant. CD is frequently misdiagnosed because it is poorly understood and presents with a variety of symptoms. We present a case of a 15-year-old previously healthy girl with unicentric CD. She presented to an Emergency Department with acute onset of chest pain. Radiographic imaging demonstrated a large right-sided mediastinal mass. Biopsy of the mass demonstrated atypical lymphoid tissue with vascular proliferation and concentric layering of peripheral lymphocytes, consistent with CD, hyaline vascular variant. Following multidisciplinary team discussion, the patient underwent complete resection of the mass. She completely recovered with no evidence of residual or recurrent disease on postoperative imaging.