Juvenile Xanthogranuloma of the Nasal Cavity-A Case Report and Literature Review

鼻腔內幼年性黃色肉芽腫－案例分享及文獻回顧

賴宇荷(Yeu-Her Lai) ； 陳琮琳(Chung-Lin Chen)

《臺灣整形外科醫學會雜誌》 30卷2期 (2021/06) Pp. 118-123

摘要

Background: Juvenile xanthogranuloma (JXG) is a benign and self-limited neoplasm of histiocytic proliferation with an incidence rate of 0.52% that is most commonly seen in infancy and childhood. It most commonly occurs in the head and neck area, followed by the upper trunk, the upper extremities and the lower extremities. However, intranasal JXG is even more uncommon as only twelve cases are reported in the English literature till now. Aim and Objectives: The aim of this study is to report an extremely rare case of intranasal juvenile xanthogranuloma. Materials and Methods: Here, we present the case of an 8 year-old boy with a non-tender, beige, and dome-shaped nodule in the roof of his right nasal vestibule with gradual enlargement for 1 year. Results: Histology showed foamy histiocytes and multinucleated Touton giant cells. The immunohistochemical staining was positive for CD68 and factor XIIIa but negative for S100. JXG was diagnosed based on these findings. Conclusion: In addition to presenting the case and discussing its clinical, morphological, and specific histopathological characteristics, we also provide a comprehensive review on JXG of the nasal cavity known to date from online searches in PubMed.

關鍵字

xanthogranuloma ； juvenile ； histiocytes

並列摘要

背景：幼年性黃色肉芽腫為一良性的組織細胞增生，最常見於幼兒時期，其發生率約為0.52%。好發的位置為頭頸部、軀幹、上肢及下肢。而位於鼻腔內幼年性黃色肉芽腫十分罕見，在英文文獻回顧中至今只有十二件案例被報導過。目的及目標：本篇案例報導一個罕見的鼻腔內幼年性黃色肉芽腫。材料及方法：文中敘述一個八歲男童於右側鼻腔內發現一顆膚色、無壓痛、圓頂狀的結節，手術切除成功後診斷為黃色肉芽腫。我們接著針對曾經診斷幼年性鼻腔內黃色肉芽腫的案例報導做文獻回顧以及探討。結果：在我們的案例中，組織學發現multinucleated Touton giant cells，免疫染色上，CD68及factor XIIIa為陽性，S100為陰性。根據臨床特徵及染色結果診斷幼年性黃色肉芽腫。結論：我們分析這案例的臨床特徵、病理特色，並且回顧所有至今英文文獻中報導鼻腔內幼年性黃色肉芽腫的案例。