Spiro R H
Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York, USA.
Am J Surg. 1997 Nov;174(5):495-8. doi: 10.1016/s0002-9610(97)00153-0.
Adenoid cystic carcinoma (ACC) is an aggressive, often indolent tumor, with a high incidence of distant metastasis (DM). Relatively little has been written about the factors that influence distant spread and subsequent survival because it is uncommon and more than a decade of observation may be required to appreciate the prolonged clinical course in some patients.
We have retrospectively studied 196 determinate patients who received definitive treatment in our hospital between 1939 and 1986 for ACC in all salivary sites. Inclusion criteria were no prior treatment elsewhere other than excisional biopsy and eligibility for follow-up of at least 10 years. Variables assessed for their impact on distant metastasis included age, gender, site, size, node status, stage, grade, and locoregional treatment failure.
Treatment failure occurred in a total of 122 of 196 determinate patients (62%), 74 of whom had DM (38%). This was usually associated with locoregional recurrence (51 patients), but DM was the only indication of failure in 23 whose primary tumor was controlled. Of the 74 patients with known DM, the lung was recorded as the only involved site in 50 patients, lung was involved in addition to other sites in 17, bone metastases alone occured in 5, and the remaining 2 developed disseminated disease. Disease-free intervals varied from 1 month to 19 years (median 36 months) and exceeded 10 years in 9 of 113 patients (8%) with adequate information about treatment failure. Survival with DM was less than 3 years in 54%, but more than 10 yrs in 10% (maximum 16 years). The only significant factors influencing survival were the size of the primary tumor (P <0.0000), local or neck recurrence (P = 0.0006), and the presence of nodal involvement (P = 0.02).
The high incidence of DM with locoregional failure confirms the importance of aggressive initial surgery, combined with irradiation, for high-stage tumors or involved surgical margins. Large tumor size and lymph node involvement, rather than microscopic appearance, were predictive of DM. Considering that lung metastases are usually multiple, and prolonged survival without treatment is not unusual, resection of pulmonary metastases may be hard to justify in ACC patients based on the limited experience thus far reported. Chemotherapy for metastatic ACC is probably best withheld until symptoms appear.
腺样囊性癌（ACC）是一种侵袭性、通常生长缓慢的肿瘤，远处转移（DM）发生率较高。关于影响远处转移及后续生存的因素，相关文献相对较少，因为该肿瘤并不常见，可能需要十多年的观察才能了解部分患者的漫长临床病程。
我们回顾性研究了1939年至1986年间在我院接受确定性治疗的196例确诊为ACC的所有涎腺部位患者。纳入标准为除切除活检外未在其他地方接受过治疗且有资格进行至少10年的随访。评估其对远处转移影响的变量包括年龄、性别、部位、大小、淋巴结状态、分期、分级和局部区域治疗失败情况。
196例确诊患者中共有122例（62%）出现治疗失败，其中74例发生远处转移（38%）。这通常与局部区域复发相关（51例），但在23例原发肿瘤得到控制的患者中，远处转移是唯一的失败指征。在74例已知发生远处转移的患者中，50例记录为肺部是唯一受累部位，17例肺部除其他部位外也受累，5例仅发生骨转移，其余2例发生播散性疾病。无病间期从1个月到19年不等（中位值36个月），在113例有足够治疗失败信息的患者中，9例（8%）超过10年。发生远处转移后的生存率在54%的患者中不到3年，但在10%的患者中超过10年（最长16年）。影响生存的唯一显著因素是原发肿瘤大小（P<0.0000）、局部或颈部复发（P = 0.0006）以及存在淋巴结受累（P = 0.02）。
远处转移合并局部区域失败的高发生率证实了对于高分期肿瘤或手术切缘受累情况，积极的初始手术联合放疗的重要性。肿瘤体积大及淋巴结受累而非微观表现可预测远处转移。鉴于肺转移通常为多发，且未经治疗而长期生存并不罕见，基于目前报道的有限经验，ACC患者肺转移切除术可能难以证明其合理性。转移性ACC的化疗可能最好在症状出现前推迟使用。
