Unicentric Castleman disease in the mediastinum

• Published in: Journal of pediatric surgery case reports Vol. 34; no. C; pp. 51 - 53
• Main Authors: Wala, Samantha J, Fallon, Erica M, Forlenza, Christopher J, Shukla, Neerav, LaQuaglia, Michael P
• Format: Journal Article
• Language: English
• Published: Elsevier Inc 01-07-2018; Elsevier
• Subjects: Castleman disease; Hyaline vascular variant; Mediastinal mass; Middle mediastinum; Pediatrics; Surgery; Thoracotomy; Pediatrics; Hyaline vascular variant; Mediastinal mass; Thoracotomy; Middle mediastinum; Castleman disease
• ISSN: 2213-5766; 2213-5766
• DOI: 10.1016/j.epsc.2018.05.005

Abstract 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.