74例原发性气管腺样囊性癌的临床分析

目的探讨原发性气管腺样囊性癌的临床特点。方法74例患者均经病理或细胞学证实。T分期中T18例（10.8%），T219例（25.7%），T323例（31.1%），T424例（32.4%）。纵隔淋巴结阳性8例（10.8%）。远处转移2例（2.7%）。62例（83.8%）接受手术治疗，27例手术完全切除，R1或R2切除35例。单纯手术24例，单纯放疗12例，综合治疗38例。结果复发或转移的患者共43例（58.1%），局部复发20例，远处转移17例，局部复发＋远处转移6例。除2例外，所有远处转移患者均有肺转移。第一次复发或转移的中位时间为32月，第二次、第三次复发或转移的中位时间分别为12月和6月。所有患者中位生存时间55月，5年、10年总生存率分别为69.7%和35.9%。结论原发性气管腺样囊性癌治疗效果较好。病变长度、T分期以及包含手术的治疗可能是影响预后的因素。患者治疗后出现多次复发或转移，积极治疗仍能获得长期生存的机会。远处转移中肺转移最多见。

Clinical analysis of 74 cases of primary tracheal adnoid cystic carcinoma

Objective To analyze the primary tracheal adnoid cystic carcinoma. Methods Seventy-four patients with primary tracheal adnoid cystic carcinoma were confirmed by pathology or cytology with a median age of 43. Cough was the most common symptom. T stage classification showed T1 in 8 cases （10.8%）,T2 in 19 （25.7%）,T3 in 23 （31.1%） and T4 in 24 （32.4%）. Mediastinal lymph node metastasis was found in 8 cases,distance metastasis in 2. Sixty-two patients （83.8%）underwent surgery,with R0 resection in 27 and R1 or R2 resection in 35,among whom 24 received surgery only, 12 received radiotherapy only and 38 received combined therapy. Results Recurrence or metastasis occurred in 43 patients （58. 1%）, including 20 recurrence, 17 distance metastasis and 6 with both recurrence and distance metastasis. All distance metastases were in lung except 2. The median time of first recurrence or distance metastasis was 32 months and that for the second and third were 12 and 6 months. The survival rates of 5-year and 10-year were 69.7% and 35.9% ,with the median survival time of 55 months. Conclusions The primary tracheal adnoid cystic carcinoma has a generally good prognosis. The tumor length, T stage and treatment including surgery may be the prognostic factors. The patients with recurrence or distance metastasis may survive longer if they receive active treatment. Lung metastasis is the most common distance metastasis.