胸上段食管侵袭性纤维瘤病诊治并文献复习

Aggressive fibromatosis derived from the upper thoracic esophagus: a case report and literature review

目的探讨食管侵袭性纤维瘤病的临床病理特征及诊断治疗方法。方法报告1例胸上段食管侵袭性纤维瘤病患者的临床病理资料,并结合国内外文献报道的4例食管侵袭性纤维瘤病的临床资料进行分析。结果食管侵袭性纤维瘤病以吞咽困难为主要症状,易误诊为食管良性肿瘤,确诊依靠组织病理学,手术无法完整切除病变范围广泛的胸上段食管侵袭性纤维瘤,内镜下食管扩张可缓解吞咽困难症状。结论食管侵袭性纤维瘤病临床少见,病变发展缓慢,其生物学行为属交界性,手术完整切除难度大,定期内镜下食管扩张有助于提高生活质量。

Objective To improve the clinical understanding of aggressive fibromatosis derived from esophagus. Methods A woman with aggressive fibromatosis of the upper thoracic esophagus was treated in our hospital. We reported the clinical pathological data of this patient and analyzed the clinical data of 4 cases with aggressive fibromatosis derived from esophagus which were reported by domestic and foreign literatures. Results The main symptom of aggressive fibromatosis was dysphagia which was easily misdiagnosed as benign tumors of the esophagus,and a definite diagnosis must depend on pathological examination. It was difficult to completely resect the wide range of lesion of esophageal aggressive fibromatosis,but endoscopic esophageal dilatation could alleviate the symptom of dysphagia.Conclusion Aggressive fibromatosis derived from esophagus is rare in clinics,and its growth is slow. The biological behavior of the neoplasm belongs to borderline. It is difficult to resect the lesion located in the upper thoracic esophagus,and regular endoscopic esophageal dilatation is helpful to improve the quality of life for the patients.