Langerhans' Cell Histiocytosis Presenting as Large Anterior Mediastinal Tumor.
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概要
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We report a 2-year-old boy who was admitted to our hospital because of large anterior mediastinal mass. Initial Symptoms were swelling of right maxilla and oral tumor. Biopsy showed infiltration of his-tiocytes. Thoracotomy revealed white colored tumor extending over the heart and lungs to such an extent that only partial excision was performed. Histological examination showed destruction of thymic architecture with infiltrating large histiocytes which were strongly positive for S-100 protein, and Birbeck's granules were detected by electron microscopic examination. Flow cytometric analysis revealed a marked decrease of CD1<SUP>+</SUP> and CD4<SUP>+</SUP> 8<SUP>+</SUP> cells. Diagnosis of Langerhans' cell histiocytosis was made and chemotherapy with VP16 and AraC had been performed for one year. One year later, he showed relapse at the left jaw joint. The same chemotherapy was effective and was continued for an additional year. He has been free from disease for three years since the last therapy.
- 特定非営利活動法人 日本小児血液・がん学会の論文
特定非営利活動法人 日本小児血液・がん学会 | 論文
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