鼻腔鼻窦磷酸盐尿性间叶瘤的诊断与手术治疗  

Diagnosis and surgical treatment of sinonasal phosphaturic mesenchymal tumor

作　　者：唐如 刘世贤 茆松 张维天[1] Tang Ru;Liu Shixian;Mao Song;Zhang Weitian(Department of Otorhinolaryngology Head and Neck Surgery,Shanghai JiaoTong University Affiliated Sixth People′s Hospital,Shanghai 200233,China)

机构地区：[1]上海交通大学附属第六人民医院耳鼻咽喉头颈外科,200233

出　　处：《中华耳鼻咽喉头颈外科杂志》2021年第4期351-355,共5页Chinese Journal of Otorhinolaryngology Head and Neck Surgery

基　　金：国家自然科学基金(81870700,82071014);上海市科技创新行动计划(19411950700)。

摘　　要：目的分析鼻腔鼻窦磷酸盐尿性间叶瘤的临床诊断与手术治疗特点。方法收集2015年1月至2020年5月于上海交通大学附属第六人民医院耳鼻咽喉头颈外科就诊的9例鼻腔鼻窦磷酸盐尿性间叶瘤患者的临床资料,其中男性4例,女性5例,年龄36~59岁。分析患者既往就诊史、临床表现、影像学表现、实验室检查结果、手术资料、病理学结果及术后随访资料。采用描述性统计学方法进行分析。结果患者病程1~19年不等,均出现肿瘤性骨软化症的相关症状。结合影像学检查及术中所见,2例患者出现周围组织浸润,2例合并对侧鼻腔侵犯,1例合并颅内侵犯。5例患者行内镜下单侧肿瘤切除术,2例行内镜下双侧肿瘤切除术,余2例分别行经眶单侧筛前、筛后动脉结扎+内镜下肿瘤切除术和内镜下肿瘤切除术联合经额开颅肿瘤切除术。1例患者半年后复发且出现颅内侵犯,余患者术后恢复可,未见肿瘤复发。结论鼻腔鼻窦磷酸盐尿性间叶瘤的诊断需综合评估其临床、影像学表现及病理学特征。手术应彻底切除肿瘤,术后需长期随访。Objective To investigate the diagnosis and surgical treatment of sinonasal phosphaturic mesenchymal tumor(PMT).Methods The medical records of nine patients who had been diagnosed as sinonasal PMT in Department of Otorhinolaryngology Head and Neck Surgery,Shanghai JiaoTong University Affiliated Sixth People's Hospital between January 2015 and May 2020 were collected,including 4 males and 5 females,ranging from 36 to 59 years.The patient's previous history,clinical manifestations,imaging findings,laboratory results,surgical procedure,pathological results and postoperative follow-up data were analyzed by descriptive statistical analysis.Results All patients presented hypophosphatemia and tumor-induced osteomalacia(TIO)with a disease course of 1 to 19 years.The imaging examination and intraoperative findings identified two cases with peripheral tissue infiltration,two cases with contralateral nasal cavity invasion,and one case with intracranial invasion.Five patients underwent unilateral endoscopic resection while two patients underwent bilateral endoscopic resection,and the remaining two patients underwent unilateral transorbital ethmoid artery ligation plus endoscopic tumor resection and endoscopic combined with transfrontal tumor resection(n=1 each).Expect for one case developed recurrence and intracranial involvement,the other patients achieved clinical remission and no recurrence was observed during the six-month follow-up.Conclusions The diagnosis of sinonasal PMT needs combination of clinical manifestation,imaging,and pathological findings.Complete surgical excision and long-term postoperative follow-up are imperative.

关 键 词：低磷血症 肿瘤性骨软化症 耳鼻喉外科手术 

分 类 号：R765.9[医药卫生—耳鼻咽喉科]