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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 clinical manifestations, diagnosis, treatment and prognosis of head and neck steatohepatoma in children, so as to further understand the characteristics of the disease and guide clinical diagnosis and treatment. Methods Retrospective analysis was performed on clinical manifestations, auxiliary examinations, diagnosis, treatment and follow-up data of patients with pathologically diagnosed lipoblastoma admitted to Department of Otorhinolaryngology Head and Neck Surgery, Beijing Children’s Hospital, Capital Medical University from December 2012 to April 2015. Results The patients in this group were 4 males and 5 females, ranging in age from 4 months to 3 years with a median of 1 year and 10 months. The clinical manifestations were as follows: throat snoring, sleep snoring or dyspnea and painless neck mass were 4 cases of children with tumors located in the neck, 5 cases located in the pharynx; 3 cases of complete removal of the neck of a tumor, 1 case of cervical tumor involving the spinal canal, surgical resection in two phases. 4 cases of pharyngeal tumors were completely resected and 1 case of tumor was mostly resected. Pathological examination included 3 cases of lipoblastoma and 6 cases of lipoblastoma. The follow-up time ranged from 10 months to 3 years. Five cases of pharyngeal lipoma Among the children with cell tumor, one case with incomplete tumor resection had no residual disease after 3 years of follow-up, and 2 cases with diffuse disease had recurrence 3 months after operation. There was no recurrence after operation. None of 4 patients with lipoblastoma recurred. Conclusion Head and neck lipoblastoma is a rare benign tumor, compared with other parts of the lesion, has its own characteristics, the need for more active intervention; surgery is the only effective treatment, if necessary, partial resection or staging Pharyngeal lipoblastoma complete removal of high difficulty, easier to relapse.