Rosanowski F, Tigges M, Gjuric M, Eysholdt U
Abteilung für Phoniatrie und Pädaudiologie, Hals-Nasen-Ohren-Universitätsklinik Erlangen.
Laryngorhinootologie. 1996 Sep;75(9):554-5. doi: 10.1055/s-2007-997633.
Juvenile xanthogranulomas are rare benign tumors of histiocytic origin with cutaneous manifestation predominantly in the head and neck. A case of a 12-month-old girl is presented.
A tumor of the inferior crus of the left auricle had been noticed three weeks after birth. It was of yellowish color, bosselated and firm, and not mobile. During operation the tumor seemed lipomatoid and affected both cutis and subcutis. Histologically cutaneous structures were regular. Subcutaneously, cell-rich connective tissue with foamy histiocytes and Touton giant cells were observed as characteristic features of a juvenile xanthogranuloma.
The pathogenesis of this tumor is unclear. The definite diagnosis is established by histological examination. Histological differentiation should include malignant tumors of histiocytic origin (dermatofibro-sarcoma protuberans, histiocytoma, atypical fibrous histiocytoma, fibro-xanthosarcoma) and malignant melanoma. Surgery is the treatment of choice only in cases in which the clinical diagnosis is doubtful, since juvenile xanthogranulomas usually heal spontaneously.
幼年性黄色肉芽肿是一种罕见的组织细胞起源的良性肿瘤，主要表现为头颈部皮肤病变。本文报告一例12个月大女童的病例。
出生三周后发现左耳下脚有一个肿瘤。肿瘤呈淡黄色，有小凸起且质地坚硬，不可活动。手术中肿瘤看似脂肪瘤样，累及皮肤和皮下组织。组织学检查显示皮肤结构正常。皮下可见富含细胞的结缔组织，伴有泡沫状组织细胞和杜顿巨细胞，这是幼年性黄色肉芽肿的特征性表现。
该肿瘤的发病机制尚不清楚。确诊需依靠组织学检查。组织学鉴别应包括组织细胞起源的恶性肿瘤（隆突性皮肤纤维肉瘤、组织细胞瘤、非典型纤维组织细胞瘤、纤维黄色肉瘤）和恶性黑色素瘤。仅在临床诊断存疑的情况下，手术才是首选治疗方法，因为幼年性黄色肉芽肿通常可自愈。
