儿童头颈部脂肪母细胞瘤临床分析

Clinical analysis of head and neck lipoblastoma in childhood

目的探讨儿童头颈部脂肪母细胞瘤的临床表现、诊断、治疗及预后,以进一步了解该病的特点,指导临床诊断和治疗。方法对首都医科大学附属北京儿童医院耳鼻咽喉头颈外科2012年12月至2015年4月收治的病理诊断为脂肪母细胞瘤患者的临床表现、辅助检查、诊断、治疗和随访数据进行回顾性分析。结果本组病例中男性4例,女性5例,年龄4个月～3岁,中位年龄1岁10个月;临床表现以嗓子呼噜、睡眠打鼾或呼吸困难以及无痛性颈部包块为主;4例患儿肿瘤位于颈部,5例位于咽部;3例颈部肿瘤一期彻底切除,1例颈部肿瘤累及椎管内,分两期手术切除。4例咽部肿瘤彻底切除,1例行肿瘤大部切除;病理检查3例为脂肪母细胞瘤病,6例为脂肪母细胞瘤;随访时间10个月～3年,5例咽部脂肪母细胞瘤患儿中,1例肿瘤未彻底切除患者随诊3年残余病变无明显增大,2例弥漫型病变患儿术后3个月复发,再次手术后随访无复发。4例颈部脂肪母细胞瘤患者均无复发。结论头颈部脂肪母细胞瘤是一种罕见的良性肿瘤,与其他部位病变相比,有其自身特点,需要更加积极地干预;手术是唯一有效的治疗方法,必要时可部分切除或分期手术;咽部脂肪母细胞瘤彻底切除难度高,更易复发。

Objective To investigate the diagnosis,treatment and prognosis of lipoblastoma of head and neck and analyze the clinical manifestations,imaging and pathologic features. Methods Nine cases of histopathologically proven lipoblastoma presented to Beijing Children’s Hospital from December 2012 to April 2015 were reviewed. The clinical characteristics,imaging findings,surgical management and follow-up results were analyzed. Results There were 4 males and 5 females subjects aged from 4 months to 3 years in our series.The chief complaints were snoring,respiratory distress and painless superficial soft tissue masses. Tumors occurred in pharynx（ n = 5）and neck（ n = 4）. One case of cervical tumors involving the spinal canal underwent surgical resections twice and others with once complete surgical resection. Complete surgical resection was performed in 4 cases of pharyngeal tumor and another one underwent conservative surgical resection. The pathological results of 3 cases were lipoblastomatosis and others were lipoblastoma. The follow-up periods ranged from 10 months to 3 years. Recurrence occurred in 2 cases of pharyngeal tumor 3 months after the operation. One case lives with residual tumor and all patients with cervical tumors had no recurrence. Conclusion Pediatric lipoblastoma is a rare benign tumor,especially in the head and neck,which presents some different clinical characteristics from the lipoblastoma of other regions.Surgical resection is the only effective treatment up to now. Although complete surgical resection is more curative,subtotal resection may be an effective alternative for those extensive lesions in the head and neck which are too difficult to get radical treatments. Pharyngeal lipoblastoma may have more potency of recurrence.