背景:原发性食管恶性黑色素瘤(PMME)是一种罕见的恶性疾病,在临床病理和生存率方面尚未得到很好的表征。
目的:探讨中国人PME患者的临床特征和生存因素。
方法:总结河南省人民医院10例PMME患者的临床病理资料。此外,回顾并分析了1980年至2021年9月以中国患有PMME的患者为重点的英语和中文文献.采用单变量和多变量分析来研究可能与生存相关的临床病理因素。
结果:共有290名中国患者患有PMME,本研究纳入了我院的10例和文献中的280例。只有大约一半的患者(55.8%)在手术前得到了准确诊断。此外,91.1%的患者接受了食管切除术,88例(36.5%)患者在手术后接受辅助治疗。淋巴结转移率(LNM)为51.2%(107/209),即使肿瘤局限于粘膜下层,LNM的阳性率也为45.3%。随着pT分期[P<0.001,比值比(OR):2.47,95%置信区间(CI):1.72-3.56]和更大的肿瘤大小(P=0.006,OR:1.21,95CI:1.05-1.38),LNM的风险显着增加。中位总生存期(OS)为11.0mo(范围:1-204mo)。多因素Cox分析显示,pT分期[P=0.005,风险比(HR):1.70,95CI:1.17-2.47]和LNM(P=0.009,HR:1.78,95CI:1.15-2.74)是OS的独立预后因素。中位无病生存期(DFS)为5.3mo(范围:0.8-114.1mo)。多因素分析表明,仅pT分期(P=0.02,HR:1.93,95CI:1.09-3.42)是PMME患者RFS不良的重要独立指标。
结论:手术前对PMME的正确诊断较低,医生应更加注意避免误诊或漏诊。即使肿瘤局限于粘膜下层,在PMME的手术中应强调扩大淋巴结清扫。LNM和pT分期是OS的独立预后因素,pT分期是影响PMME患者DFS的预后因素。
BACKGROUND: Primary malignant melanoma of the esophagus (PMME) is a rare malignant disease and has not been well characterized in terms of clinicopathology and survival.
OBJECTIVE: To investigate the clinical features and survival factors in Chinese patients with PMME.
METHODS: The clinicopathological findings of ten cases with PMME treated at Henan Provincial People\'s Hospital were summarized. Moreover, the English- and Chinese-language literature that focused on Chinese patients with PMME from 1980 to September 2021 was reviewed and analyzed. Univariate and multivariate analyses were employed to investigate the clinicopathologic factors that might be associated with survival.
RESULTS: A total of 290 Chinese patients with PMME, including ten from our hospital and 280 from the literature were enrolled in the present study. Only about half of the patients (55.8%) were accurately diagnosed before surgery. Additionally, 91.1% of the patients received esophagectomy, and 88 patients (36.5%) received adjuvant therapy after surgery. The frequency of lymph node metastasis (LNM) was 51.2% (107/209), and LNM had a positive rate of 45.3% even when the tumor was confined to the submucosal layer. The risk of LNM increased significantly with the pT stage [P < 0.001, odds ratio (OR): 2.47, 95% confidence interval (CI): 1.72-3.56] and larger tumor size (P = 0.006, OR: 1.21, 95%CI: 1.05-1.38). The median overall survival (OS) was 11.0 mo (range: 1-204 mo). The multivariate Cox analysis showed both the pT stage [P = 0.005, hazard ratio (HR): 1.70, 95%CI: 1.17-2.47] and LNM (P = 0.009, HR: 1.78, 95%CI: 1.15-2.74) were independent prognostic factors for OS. The median disease-free survival (DFS) was 5.3 mo (range: 0.8-114.1 mo). The multivariate analysis indicated that only the advanced pT stage (P = 0.02, HR: 1.93, 95%CI: 1.09-3.42) was a significant independent indicator of poor RFS in patients with PMME.
CONCLUSIONS: The correct diagnosis of PMME before surgery is low, and physicians should pay more attention to avoid a misdiagnosis or missed diagnosis. Extended lymph node dissection should be emphasized in surgery for PMME even though the tumor is confined to the submucosal layer. Both the LNM and pT stage are independent prognosis factors for OS, and the pT stage is the prognosis factor for DFS in patients with PMME.