纤维瘤病109例临床特征与治疗

目的分析纤维瘤病的临床特点及治疗。方法对我院1986年1月1日至2008年12月31日间确诊的109例纤维瘤病患者的临床特征、治疗情况进行总结分析。结果109例患者中女性70例，男性39例，87．2％的患者是以肿块为首发症状，肿块伴疼痛占33．7％，32．1％（35／109例）肿块发生在腹壁，腹壁肿块患者中女性达94．3％。直径5cm及以上肿块占70．5％。深部肿块直径明显大于浅表肿块，直径分别为（11．1±5．9）cm和（6．8±5．1）cm，P〈0．01。12例患者接受过放射治疗，8例以预防术后复发为目的的患者7例出现放疗后复发，平均复发时间为2．7年。1例患者采用联合化疗达到部分缓解。4例患者由于本病导致死亡。结论手术仍是目前治疗纤维瘤病的主要手段，放疗对部分患者也有效。

Clinical features and treatment of aggressive fibromatosis

Objective To study the clinical features and treatment of aggressive fibromatosis ( AF). Methods One hundred and nine the clinical characters, treatment and efficacy of each patient were reviewed. Results One hundred and nine pathologically diagnosed AF patients presented in Peking Union Medical College Hospital ( PUMCH) was analyzed. 70 females and 39 males were included with an average age of 34.0 and 36.9 yrs respec-tively. 87.2% of patients first presented as mass, 33.7% first presented as mass accompanying pain. 32. 1% of mass located in the abdomen wall among which 94. 3% were female, 51.5% of these females had a history of sur-gery of the abdomen. Diameter of all the masses was(7. 84 ±5.62)cm, diameter of deep mass was statistically lar-ger than that of superior ones, which were(11. 1 ±5.9)cm and(6. 8 ±5. 1 )cm respectively,P <0. 01. Twelve pa-tients got radiotherapy and 8 of them did it for the purpose of preventing disease recurrence. 7 out of them recurrent within an average interval of about 2. 7 years. A patient whose tumor was located in the anterior mediastinum was treated with chemotherapy and had a partial response. 4 patients died of AF. Conclusion Operation is still most widely used therapeutic option. Radiotherapy is also effective in certain percentage of patients and medicine such as chemotherapy or NSAlDs that have not been receipted.