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Erdheim-Chester Disease Presenting as an Anterior Mediastinal Tumor without Skeletal Involvement
Kanghoon Lee1, Hyeong Ryul Kim2, Jin Roh3
1Department of Thoracic and Cardiovascular Surgery, Korea University Guro Hospital, Korea University College of Medicine.
Abstract:
Erdheim-Chester disease (ECD) is a form of non-Langerhans cell histiocytosis that most commonly involves the skeletal system. We report an unusual case of ECD presenting as an anterior mediastinal tumor without skeletal involvement. A 60-year-old man with no remarkable medical history was referred for evaluation of a mediastinal mass. The patient underwent surgical excision of the tumor via video-assisted thoracoscopic surgery. Histologic examination revealed marked proliferation of atypical histiocytes with sclerosis, and the results of immunohistochemical staining were suggestive of ECD.
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