Lorenzen Jacob C, Godballe Christian, Kerndrup Gitte B
Department of ENT, Head and Neck Surgery, Odense University Hospital, DK-5000 Odense C, Denmark.
Auris Nasus Larynx. 2005 Jun;32(2):169-73. doi: 10.1016/j.anl.2005.01.016. Epub 2005 Apr 21.
Lipoblastomatous tumours are rare, and they occur primarily in children younger than 3 years of age. They are benign and may be divided in lipoblastomas and lipoblastomatosis. A case with cervical lipoblastoma causing respiratory difficulty is reported, and a clinical characterisation of patients with lipoblastomatous tumours in the neck is presented. A 6-year-old boy with complains of stridorous respiration and significant reduction in physical capacity was referred to the ENT Department, Odense University Hospital, Denmark. He was treated with total surgical resection of a soft and slowly growing tumour in the left side of the neck, extending from the base of the skull to the upper part of the mediastinum. The histological examination showed a lipoblastoma. After surgery all symptoms disappeared, and the patient was without any operative sequelae. Including the actual case, a review of English literature resulted in the identification of 37 patients with cervical lipoblastoma or lipoblastomatosis. However, in most cases the information was sparse, and only 13 patients were eligible for an analysis of basic clinical and demographic data. The median age was 25 months (range: 7-75 months), and the median tumour size 9 cm (range: 3-18 cm). No difference in sex distribution was found. Lipoblastomas (85%) seemed more frequent than lipoblastomatosis (15%). Stridorous respiration was present in 31%. It is concluded that a considerable part of lipoblastomatous tumours in the neck are combined with respiratory difficulties (31%), and that complete but gentle surgical treatment in most cases will restore normal physiological conditions.
脂肪母细胞瘤性肿瘤较为罕见,主要发生于3岁以下儿童。它们是良性的,可分为脂肪母细胞瘤和脂肪母细胞瘤病。本文报告了1例导致呼吸困难的颈部脂肪母细胞瘤病例,并对颈部脂肪母细胞瘤性肿瘤患者进行了临床特征分析。一名6岁男孩因呼吸急促和体力明显下降被转诊至丹麦欧登塞大学医院耳鼻喉科。他接受了颈部左侧一个柔软且生长缓慢的肿瘤的全手术切除,该肿瘤从颅底延伸至纵隔上部。组织学检查显示为脂肪母细胞瘤。手术后所有症状消失,患者无任何手术后遗症。包括本病例在内,对英文文献的回顾共识别出37例颈部脂肪母细胞瘤或脂肪母细胞瘤病患者。然而,大多数病例信息稀少,仅有13例患者符合基本临床和人口统计学数据分析条件。中位年龄为25个月(范围:7 - 75个月),中位肿瘤大小为9 cm(范围:3 - 18 cm)。未发现性别分布差异。脂肪母细胞瘤(85%)似乎比脂肪母细胞瘤病(15%)更常见。31%的患者存在呼吸急促。结论是,颈部脂肪母细胞瘤性肿瘤有相当一部分(31%)伴有呼吸困难,且大多数情况下完整而轻柔的手术治疗将恢复正常生理状态。