上顎に発生したランゲルハンス細胞組織球症の1症例

A case of Langerhans cell histiocytosis involved in the maxilla

Ⅰ．はじめに

　ランゲルハンス細胞組織球症（Langerhans cell histiocytosis：LCH）は，ランゲルハンス細胞が増殖する原因不明の腫瘍性疾患と定義されている。今回は，当科で経験した上顎に発生したLCHの1症例を報告する。

　A 3-year-old boy presented with a left progressive maxillary swelling. Images of CT and MRI delineated an osteolytic soft tissue mass extending into the left maxillary sinus and the orbital floor. Biopsy under general anesthesia confirmed Langerhans cell histiocytosis（LCH）. Clinical type was determined as multi-system multi-site（MM）. Treatments with chemotherapeutic protocols of Japan LCH Study Group（JLSG）-02,which consisted of induction therapy for 6 weeks and subsequent maintenance therapy for 12 months,lead the patient cured. Low grade hematologic side effects were observed. Any recurrent lesions have not been observed during the follow-up period.