原发于眶外的头颈部炎性肌纤维母细胞瘤14例临床分析

Clinical analysis of 14 patients with extraorbital inflammatory myofibroblastic tumor of the head and neck

目的总结原发于眶外的头颈部炎性肌纤维母细胞瘤的临床特征及治疗方法。方法回顾性分析北京协和医院1992年7月至2012年6月收治的14例原发于眶外的头颈部炎性肌纤维母细胞瘤患者的临床资料。结果14例患者中9例病变局限，发生于上颌骨5例，下颌骨2例，颈部2例，均采取肿瘤根治性切除手术，随访1．5—20年无复发，中位随访时间5．0年。4例病变范围广泛，发生于上颌骨并累及眼眶、硬腭或翼腭窝等结构，其中3例采取保守治疗，分别给予单纯糖皮质激素、糖皮质激素＋放疗和糖皮质激素＋化疗，分别随访6年、9年和2年，病变无明显进展；另1例接受2次手术，并证实伴有颈淋巴转移，于发病17个月后因颅内受侵死亡。1例因病变包绕颈总动脉，采取糖皮质激素治疗，肿物明显缩小，随访2年，疾病无进展。结论原发于眶外的头颈部炎性肌纤维母细胞瘤非常少见，临床表现无特异性，确诊有赖于病理检查。病变局限者可采取根治性手术治疗，而病变范围广泛或累及重要结构者，则首选糖皮质激素治疗。

Objective To summarize the clinical features of extraorbital inflammatory myofibroblastic tumor （IMT） of the head and neck. Methods Fourteen cases of extraorbital IMT treated in recent 20 years were analyzed retrospectively. Results Of the 14 patients, 9 cases with limited lesion in maxilla （ n = 5 ）, mandible （ n = 2 ） or neck （ n = 2 ） underwent local resection, and no recurrences were found after 1.5 to 20.0 years; 3 cases diagnosed as maxillary IMT involved in orbit, hard palate or pterygopalatine fossa received conservative therapy （prednisone, prednisone plus radiotherapy or prednisone plus chemotherapy）, and no disease progression was found after 6, 9 or 2 years respectively; and 1 case diagnosed as maxillary IMT involved in orbit and pterygopalatine fossa was confirmed with cervical metastases after two operations and died of brain invasion within 17 months. One patient with localized lesion around the common carotid artery was treated with prednisone and had no disease progression with a 2-year follow-up. Conclusions Extraorbital IMT of the head and neck is a rare clinical entity. Pathology examination is required for final diagnosis. Corticosteroid administration may be a choice of treatments, and radical resection should be taken selectively for limited lesions.