Castleman's Disease: Atypical Manifestation in an 11-Year-Old Gir

Capt Vinod K. Gidvani, USAF, MC, Capt Melissa M. Tyree, USAF, MC, Col Samar K. Bhowmick, USAF, MC, Department of Pediatrics, Keesler Air Force Base, Miss

South Med J. 2001;94(2) 

In This Article

Abstract and Introduction


Castleman's disease (angiofollicular lymphoid hyperplasia) is a rare lymphoproliferative disorder that can be found in both nodal and extranodal sites. It is classified histologically as the more common hyaline vascular variant, a plasma cell variant, or a mixed form. The hyaline vascular variant is typically characterized by a benign clinical course with no constitutional symptoms other than localized pressure from the mass. We report an atypical case of the hyaline vascular variant with constitutional symptoms that have been clinically associated with the aggressive plasma cell variant. Diagnosis was confirmed histopathologically after surgical resection, which was curative and resulted in resolution of all symptoms. This case broadens our understanding of Castleman's disease as a part of the spectrum of lymphoproliferative and inflammatory diseases. Furthermore, it supports recent studies suggesting that systemic pathogenicity is related to associated cytokine dysregulation and highlights the need to include Castleman's disease in the differential diagnosis of pediatric lymphoproliferative disorders.


Castleman's disease (also known as angiofollicular lymph node hyperplasia, angiomatous lymphoid hamartoma, and giant lymph node hyperplasia) is an uncommon lymphoproliferative disorder that most frequently is seen as an asymptomatic mass in the mediastinum.[1] Little is known about the cause of this disorder, but the bulk of the evidence points toward faulty immunoregulation, which results in the excessive proliferation of B lymphocytes and plasma cells in lymphoid organs. Three histologic variants (hyaline vascular, plasma cell, and mixed), as well as two clinical variants (localized and multicentric) have been described.[2] We discuss the case of an 11-year-old girl with the hyaline vascular type of Castleman's disease localized in the mediastinum who had significant systemic illness, an unusual manifestation of this histologic form. All symptoms resolved with surgical resection. A brief review of the common histologic and clinical features of Castleman's disease and an analysis of the possible etiologies of the systemic manifestations rarely associated with the hyaline vascular variant are discussed.


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