头颈部侵袭性纤维瘤病1例及文献复习

Aggressive fibromatosis of the head and neck:case report and literature review

目的探讨头颈部侵袭性纤维瘤病的临床表现、病理学特点、诊断、治疗方法和预后。方法对1例发生于颈部的侵袭性纤维瘤病患者的病例资料及文献进行回顾分析。结果发生于头颈部软组织的侵袭性纤维瘤病约占全身侵袭性纤维瘤病的5%～9%,是一种较为少见的临界瘤,常表现为质硬、无痛性肿块(偶有疼痛),可快速或隐蔽性生长,活动度差,可导致面部畸形、侵犯颅底或重要的神经血管、压迫呼吸道等严重的后果。磁核共振可作为术前判断病变大小和位置的首选方法,瘤内见特征性的低T1及T2信号的胶原纤维成分对诊断具有一定提示作用。具有较明确的病理学特征,无包膜或偶见包膜,光镜下可见病变由束状平行排列的呈长梭形成纤维细胞和肌纤维母细胞组成,伴有不同程度的胶原化,细胞呈浸润性生长,细胞膜界限不清,细胞质数量不等,无病理性核分裂及异型性;免疫组织化学特征表现为Vim(+)、HHF-35(+)、CD34(-)、S-100(-)、Ki-67(+)。该病具有侵袭性,容易复发,但缺乏远处转移能力。治疗方法主要为手术切除,放化疗、激素治疗、生物治疗等有一定的辅助作用。结论头颈部侵袭性纤维瘤病无特异性的临床特征,其诊断主要依靠病理学检查,主要治疗方法为手术切除,如果无法完整切除或者复发时可辅助放化疗及生物治疗等,术后需定期随访。

Objective To evaluate the clinical manifestations,pathological features,treatment methods and prognosis of aggressive fibromatosis of the head and neck.Methods One patient with aggressive fibromatosis of the neck was analyzed,and the relevant literature was reviewed.Results Head and neck lesions account for approximately 12 to 15%of aggressive fibromatosis,which is a rare type of borderline tumor that is commonly characterized by a hard texture,painlessness(but occasionally with pain),hidden growth and poor mobility.Such tumors can result in facial deformity and invasion of the skull base or main nerves,and blood vessels and can compress the airway.MRI is the preferred method for preoperatively determining the size and location of the lesion.The characteristic low T1 and T2 signals of collagen fiber are helpful for the diagnosis of the tumor.The disease has clear pathological features,with tumors consisting of long spindle fibroblasts and myofibroblasts arranged in parallel fascicles with various levels of collagen formation.The tumor cells exhibit the characteristics of infiltrative growth,ill-defined cell membranes and variable amounts of cytoplasm.Pathologic mitosis and atypia are not seen.Characteristic immunohistochemical features include expression of Vim(+),HHF-35(+),CD34(-),S-100(-)and Ki-67(+).The disease is locally invasive,and patients may relapse easily,but distant metastases are not observed.The primary treatment is surgical resection.Chemotherapy,hormone therapy and biological treatments have auxiliary functions in the treatment of these tumors.Conclusion Aggressive fibromatosis of the head and neck has no specific clinical features;the diagnosis depends primarily on pathological examination.The main treatment is radical surgical resection.Radiotherapy,chemotherapy and biological treatment can be used in combination with surgery in cases of incomplete resection or recurrence.