Maziak D E, Todd T R, Keshavjee S H, Winton T L, Van Nostrand P, Pearson F G
University of Toronto, The Toronto Hospital, Department of Thoracic Surgery, Ontario, Canada.
J Thorac Cardiovasc Surg. 1996 Dec;112(6):1522-31; discussion 1531-2. doi: 10.1016/S0022-5223(96)70011-9.
We have reviewed our experience in 38 patients with adenoid cystic carcinoma of the upper airway seen between 1963 and 1995. The mean age was 44.8 years (15 to 80 years) with a male/female ratio of 1:1.1. Thirty-two of the 38 patients were treated by resection and reconstruction (primary anastomosis 28; Marlex mesh prosthesis 4). Twenty-six of the 32 patients undergoing resection received adjuvant radiotherapy. Six patients with unresectable tumors were treated primarily with radiotherapy only.
Pathologic examination revealed local invasion beyond the wall of the trachea in all patients. In a majority, microscopic extension was found in submucosal and perineural lymphatics, well beyond the grossly visible or palpable limits of the tumor. Lymphatic metastases were relatively uncommon, occurring in only five of 32 (19%) patients undergoing resection. Metachronous hematogenous metastases occurred in 17 of 38 patients (44%). Thirteen of these 38 patients (33%) had pulmonary metastases. Sixteen of 32 resections were complete and potentially curative. There were two deaths within 30 days of operation. The mean survival in the 14 patients undergoing complete resection was 9.8 years (12 months to 29 years). Sixteen of 32 resections were incomplete (residual tumor at the airway margin on final pathologic examination), with one operative death occurring in this group. The mean survival in the 15 surviving patients was 7.5 years (4 months to 21 years). Six patients were treated with primary radiation only and had a mean survival of 6.2 years (2 months to 14.3 years). In the patients with pulmonary metastases, mean survival was 37 months (4 months to 7 years) from the time of diagnosis of the pulmonary metastasis until their death.
Adenoid cystic carcinoma of the upper airway is a rare tumor, which is locally invasive and frequently amenable to resection. Although late local recurrence after resection is a feature of this tumor (up to 29 years), excellent long-term palliation is commonly achieved after both complete and incomplete resection. There was a small difference in survival between patients having complete and incomplete resection. Long periods of control can be obtained with radiotherapy alone. The best results, in this series of patients, were obtained by resection. Adjuvant radiotherapy is assumed to favorably influence survival.
我们回顾了1963年至1995年间诊治的38例上呼吸道腺样囊性癌患者的经验。患者平均年龄44.8岁（15至80岁），男女比例为1:1.1。38例患者中有32例接受了切除及重建治疗（一期吻合28例；Marlex网片假体修复4例）。32例接受切除治疗的患者中有26例接受了辅助放疗。6例无法切除的肿瘤患者仅接受了单纯放疗。
病理检查显示所有患者均有气管壁外的局部侵犯。大多数患者在黏膜下和神经周围淋巴管有显微镜下的扩散，远远超出肿瘤肉眼可见或可触及的范围。淋巴结转移相对少见，32例接受切除治疗的患者中只有5例（19%）发生。38例患者中有17例（44%）发生异时性血行转移。这38例患者中有13例（33%）发生肺转移。32例切除中有16例完整切除且可能治愈。术后30天内有2例死亡。14例接受完整切除的患者平均生存期为9.8年（12个月至29年）。32例切除中有16例不完整（最终病理检查气道边缘有残留肿瘤），该组有1例手术死亡。15例存活患者的平均生存期为7.5年（4个月至21年）。6例仅接受单纯放疗的患者平均生存期为6.2年（2个月至14.3年）。肺转移患者从肺转移诊断至死亡的平均生存期为37个月（4个月至7年）。
上呼吸道腺样囊性癌是一种罕见肿瘤，具有局部侵袭性，通常可切除。尽管切除术后局部复发较晚是该肿瘤的一个特点（长达29年），但完整切除和不完整切除后通常都能实现良好的长期缓解。完整切除和不完整切除患者的生存期有微小差异。单纯放疗可获得较长时间的病情控制。在这组患者中，切除治疗效果最佳。辅助放疗被认为对生存期有有利影响。
