Medini E, Medini A M, Lee C K, Gapany M, Levitt S H
Department of Radiation Oncology and Otolaryngology, University of Minnesota, and Veterans Administration Medical Center, Minneapolis 55417, USA.
Am J Clin Oncol. 1998 Apr;21(2):121-5. doi: 10.1097/00000421-199804000-00004.
A patient is diagnosed with an unknown primary of the head and neck when metastatic disease is present in the cervical lymph node or nodes and no primary lesion is detected by thorough physical examination, directed biopsies of suspicious or most likely primary sites, and imaging studies. The optimal management of patients who have this syndrome is still unclear and controversial. We report our results and analysis of the management of 24 patients with this syndrome. From 1976 through 1992, 24 patients who had metastatic squamous cell carcinoma in the cervical lymph nodes were seen in our medical center. A thorough search did not detect a primary lesion in any of them. Patients underwent radical neck dissection of the involved neck; 23 had unilateral and I had bilateral neck disease. Postoperative radiotherapy was delivered to both sides of the neck and to the potential primary mucosal and submucosal sites. The relation between clinical N stage, histologic findings of numerous involved lymph nodes, presence of extracapsular tumor extension, and survival were statistically analyzed. The Kaplan-Meier method was used for the survival analysis. The p values of log-rank test for the comparison of the two groups 1) N1 and N2 versus N3, and 2) presence of extracapsular tumor extension versus its absence are less than 0.005, with extracapsular tumor extension versus nonextracapsular tumor extension slightly smaller. The 5- and 10-year disease-free survival rate for the entire group was 54.2% (70.5% for N1 and N2, and 14.2% for N3). Three patients had locoregional failure, two in the primary sites, one in the nasopharynx, and the other in the oropharynx (the latter also had recurrent disease in the undissected neck). In 8 patients, distant metastases developed 7 to 38 months after radiotherapy. All 11 patients (45.8%) who had recurrent disease had advanced clinical N stage, microscopic findings of numerous involved lymph nodes, and prominent extracapsular tumor extension to the surrounding soft tissue and blood vessels. The high incidence of distant metastases shortly after treatment suggests a hematogenous spread before treatment in patients who had extensive nodal and extranodal disease. Our long-term disease-free survival beyond ten years seems to indicate combined treatment modalities, including radical neck dissection with postoperative radiotherapy of the neck, and the potential primary site in patients with N2 and N3 disease (our N1 group is too small for analysis). Further improvement of cure rate can be expected in the future with early detection and treatment.
当颈部淋巴结出现转移性疾病，而经过全面体格检查、对可疑或最可能的原发部位进行定向活检以及影像学检查均未发现原发病变时，患者被诊断为头颈部不明原发癌。对于患有这种综合征的患者，最佳治疗方案仍不明确且存在争议。我们报告了对24例患有这种综合征患者的治疗结果及分析。从1976年至1992年，我们医疗中心共诊治了24例颈部淋巴结转移性鳞状细胞癌患者。全面检查未在任何患者中发现原发病变。患者接受了患侧颈部根治性颈清扫术；23例为单侧颈部病变，1例为双侧颈部病变。术后对双侧颈部以及潜在的原发黏膜和黏膜下部位进行了放疗。对临床N分期、多个受累淋巴结的组织学表现、包膜外肿瘤扩展情况与生存率之间的关系进行了统计学分析。采用Kaplan-Meier方法进行生存分析。两组比较的log-rank检验p值：1）N1和N2与N3比较，以及2）有包膜外肿瘤扩展与无包膜外肿瘤扩展比较均小于0.005，有包膜外肿瘤扩展与无包膜外肿瘤扩展的p值略小。整个组的5年和10年无病生存率为54.2%（N1和N2为70.5%，N3为14.2%）。3例患者出现局部区域复发，2例在原发部位，1例在鼻咽部，另1例在口咽部（后者未清扫的颈部也有复发疾病）。8例患者在放疗后7至38个月出现远处转移。所有复发的11例患者（45.8%）临床N分期均较晚，有多个受累淋巴结的微观表现，且包膜外肿瘤明显扩展至周围软组织和血管。治疗后不久远处转移的高发生率表明，对于有广泛淋巴结和结外病变的患者，在治疗前存在血行转移。我们超过十年的长期无病生存似乎表明联合治疗模式是有效的，包括根治性颈清扫术及术后颈部放疗，对于N2和N3期疾病患者还包括对潜在原发部位的放疗（我们的N1组样本量太小无法进行分析）。随着早期检测和治疗，未来有望进一步提高治愈率。
