From the archives of MD Anderson Cancer Center Castleman disease involving the thymus gland: Case report and literature review

Castleman disease (CD) is a term that encompasses a group of uncommon lymphoproliferative disorders that have heterogeneous histopathologic features and variable clinical presentation and prognosis [1]. Clinically, CD can be classified into unicentric CD (UCD) and multicentric CD (MCD) types based on the number of involved lymph node stations. Histologically, Castleman disease has been classified into three variants: hyaline-vascular (HV), plasma cell, and mixed [2].

Hyaline-vascular CD typically presents in unicentric form and commonly involves supradiaphragmatic lymph nodes, particularly mediastinal and cervical lymph nodes, and uncommonly involves extranodal sites [3], [4]. This disease may be asymptomatic and detected incidentally in patients undergoing radiologic studies for other purposes. Other patients may have symptoms secondary to the mass effect of large lymph nodes causing compression of surrounding structures. Others have suggested that HV CD is a benign neoplasm of stromal cells within lymph nodes [5], [6], [7].

We encountered a case of unicentric HV CD involving the thymus gland. The patient presented with a large thymic mass and no other disease sites. Thymectomy was performed and showed a multi-loculated cystic mass with histologic features of hyaline-vascular variant CD. We reviewed the literature showing that CD rarely involves the thymus gland. The case and the relevant literature are presented here in detail.

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