杯状细胞类癌：丹麦83例患者队列的特征
Goblet cell carcinoids: characteristics of a Danish cohort of 83 patients.
作者信息
Olsen Ingrid Holst, Holt Nanna, Langer Seppo W, Hasselby Jane P, Grønbæk Henning, Hillingsø Jens, Mahmoud Masti, Ladekarl Morten, Iversen Lene H, Kjær Andreas, Federspiel Birgitte H, Knigge Ulrich
机构信息
Department of Surgical Gastroenterology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark; Department of Oncology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark; Department of Pathology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark; Department of Endocrinology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark; Department of Clinical Physiology, Nuclear Medicine and PET and Cluster for Molecular Imaging, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark.
Department of Hepatology and Gastroenterology, European NET Centre of Excellence, Aarhus University Hospital, Aarhus, Denmark.
出版信息
PLoS One. 2015 Feb 11;10(2):e0117627. doi: 10.1371/journal.pone.0117627. eCollection 2015.
BACKGROUND
Appendiceal goblet cell carcinoids (GCCs) exhibit neuroendocrine and adenocarcinoma features.
PATIENTS AND METHODS
Analysis of demography, pathology, prognostic markers, treatment and survival in 83 GCC patients (f/m: 56/27) diagnosed 1992-2013.
RESULTS
Median age for f/m was 59/58 years, respectively, and similar for localized and disseminated disease. At diagnosis 54 patients had localized appendiceal disease (f/m: 29/25). According to TNM 24% had Stage I, 70% had Stage II and 6% had Stage III. Twenty-nine patients had disseminated disease (f/m: 27/2). Chromogranin A, synaptophysin and p53 were positive in >90%. Serotonin was positive in 70%. Median Ki67 index was 32% (6-75%) and higher in Tang group C (50%) compared to group A (30%; p<0.0001), and group B (30%; p<0.004). All patients had surgery. Sixty-three (76%) had radical resections including all patients with localized disease. Median OS was 83 months. The 1-, 5- and 10-year survival rates were 90%, 58%, and 38%, respectively. For localized disease OS was 164 months and 1-, 5- and 10-year survival rates were 100%, 80%, and 55%, respectively. For disseminated disease OS was 19 months and 1-, 5- and 10-year survival rates were 73%, 18% and 6%, respectively. The 1-, 5- and 10 year-survival rates for f/m were 87%/96%, 49%/76% and 31%/57%, respectively (p = 0.02). According to the Tang classification group A, B, and C OS was 118, 83 and 20 months, respectively (p = 0.0002).
CONCLUSION
The Tang classification was found to be a significant prognostic factor, while the Ki67 index was not. Localized GCCs occurred equally in males and females, while disseminated GCCs were mostly seen in females. Median age of patients with localized disease and disseminated disease was identical. Cox regression analysis found Stage IV, focally positive synaptophysin and non-radical surgery as strongest negative prognostic factors.
背景
阑尾杯状细胞类癌（GCCs）具有神经内分泌和腺癌特征。
患者与方法
分析1992年至2013年确诊的83例GCC患者（男/女：56/27）的人口统计学、病理学、预后标志物、治疗及生存情况。
结果
女性和男性的中位年龄分别为59岁和58岁，局限性和播散性疾病患者的年龄相似。诊断时，54例患者患有局限性阑尾疾病（男/女：29/25）。根据TNM分期，24%为I期，70%为II期，6%为III期。29例患者患有播散性疾病（男/女：27/2）。嗜铬粒蛋白A、突触素和p53的阳性率>90%。血清素阳性率为70%。Ki67指数中位数为32%（6%-75%），与A组（30%；p<0.0001）和B组（30%；p<0.004）相比，C组（50%）更高。所有患者均接受了手术。63例（76%）接受根治性切除，包括所有局限性疾病患者。中位总生存期为83个月。1年、5年和10年生存率分别为90%、58%和38%。局限性疾病患者的总生存期为164个月，1年、5年和10年生存率分别为100%、80%和55%。播散性疾病患者的总生存期为19个月，1年、5年和10年生存率分别为73%、18%和6%。男性和女性的1年、5年和10年生存率分别为87%/96%、49%/76%和31%/57%（p = 0.02）。根据唐分类，A组、B组和C组的总生存期分别为118个月、83个月和20个月（p = 0.0002）。
结论
发现唐分类是一个重要的预后因素，而Ki67指数不是。局限性GCCs在男性和女性中发病率相同，而播散性GCCs多见于女性。局限性疾病和播散性疾病患者的中位年龄相同。Cox回归分析发现IV期、突触素局灶阳性和非根治性手术是最强的负性预后因素。
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