- 期刊
顳骨之蘭格罕士氏細胞組織球症－兩例報告
Langerhans' Cell Histiocytosis of the Temporal Bone - Report of Two Cases
摘要
南格罕士氏細胞組織球症(Langerhans’ cell histiocytosis)為一種病因未明之罕見疾病，常出現於1至5歲兒童病患，其特徵為組織球細胞增生和其他發炎細胞聚集。本部於近3年內，經驗2名罹患蘭格罕士氏細胞組織球症之幼童。其中一名患者以右耳耳漏併右耳後乳突部腫脹為表現，另一名病患則出現右耳疼痛與右側外耳道腫塊兩者在顱部X 光攝影和電腦斷層攝影均顯示右顳骨處溶骨性病變以及軟組織佔據右乳突骨處，經切片證實為蘭格罕士氏細胞組織球症。患者接受一系列全身性檢查後，未發現其他系統遭受侵犯，故確定為顳骨局部受犯之蘭格罕士氏組織球症。其中一名患者僅接受低劑量局部放射性治療，藉以保留患者之聽力平衡與顏面神經功能。另一名患者則因侵犯圍較廣，而接受化學治療。此2名患者皆於門診追蹤，恢復情況良好。由於此症之臨床表現常出現於頭頸部，且易與其他耳部疾病，如外耳炎、耳內息肉、急性乳突或惡性耳部腫瘤相混淆，但彼此治療方式卻差異頗大。因此當小而病患出現持續性耳漏，耳痛或乳突處病變，但藥物治療無效時，必須將此症列為鑑別診斷。
並列摘要
Langerhans’ cell histiocytosis is a rare disorder of unknown etiology. It is characterized by proliferation of histiocytes and other inflammatory cells. We have encountered two children suffering from Langerhans’ cell histiocytosis over thelast three years. One presented with persistent otorrhea and post-aurricular swelling, the other presented with right otalgia and mass lesion in the roght extermal ear canal. In both patients, mastoid X-ray films and computed tomography revealed an osteolytic lesion over the right temporal bone and a soft tissue density inside the bony defect in both patients. Incisional biopsy histopathology identified Langerhans’ cell histiocyosis Whole body surveys showed local involvement of the temporal bone. One patient received low-dose radiotherapy, whereas the underwent chemotherapy because of a widespread local lesion. The responses were good and no complications were noted in either case. The clinical presenta-tion of this disease is easily confused with other otologic problems including otitis externa, otitis madia, aural polyps, acute mastoiditis and malignancy of the ear of which requiring different treatments. This disease must be kept in mind by the otolaryn-gologist because of the diagostic and therapeutic problems that can arise if misdiag-nosed.
延伸閱讀
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