The Clinicopathologic Features and Treatment of 607 Hindgut Neuroendocrine Tumor (NET) Patients at a Single Institution

The Clinicopathologic Features and Treatment of 607 Hindgut Neuroendocrine Tumor (NET) Patients at a Single Institution
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DOI:
10.1097/md.0000000000003534
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发表时间:
2016-05-01
期刊:
影响因子:
1.6
通讯作者:
Park, Young Suk
Park, Young Suk
中科院分区:
医学4区
文献类型:
--
作者:
Kim, Seung Tae;Ha, Sang Yun;Park, Young Suk

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后肠神经内分泌肿瘤(NET)的临床病理特征以及治疗结果并不清楚。目前尚无关于转移性后肠NET患者治疗结局的已发表数据。本研究的目的是对后肠NET患者的临床病理特征、治疗和生存率进行综合分析。在2001年3月至2015年2月期间在三星医疗中心病理诊断为后肠NET的患者中,本研究分析了607例患者。后肠NET被定义为起源于横结肠和远端结肠、直肠和肛门的NET。原发部位包括结肠81例(13.3%)和直肠526例(86.7%)。根据WHO分类,578例患者(95.2%)为1级NETs,17例(2.8%)为2级NETs,12例(2.0%)为神经内分泌癌(NEC)。42例患者(6.9%)有广泛的疾病,而大多数(93.1%,565例患者)仅表现出局限性疾病。565例局限性NET患者的5年和10年生存率分别为98.1%和95.3%。42例广泛性疾病患者的中位OS为24.8个月(95% CI,10.7-38.8)。在565例局限性疾病患者中,大多数(484例,85.7%)由胃肠病学家进行内镜手术治疗。对于42例广泛疾病患者,17例患者接受支持治疗,3例接受同步放化疗(CCRT),22例接受全身治疗。在这22例患者中,12例患者仅接受一线治疗,8例患者接受二线治疗,仅2例患者接受三线治疗。最常用的一线化疗方案为依托泊苷联合顺铂(N=7)和长效奥曲肽(N=7)。在治疗过程中,最常用的方案是长效奥曲肽。接受全身治疗的22例转移性NET患者的中位OS为19.3个月(95% CI,3.2-35.3)。对607例后肠NETs患者的多因素分析表明,病变范围和原发部位是影响长期生存的重要独立预后因素。该分析提供了有关后肠NET患者的临床病理特征、治疗和生存结局的有用信息。
The clinicopathologic features of hindgut neuroendocrine tumor (NET) as well as the treatment outcomes are not well known. There are currently no published data on treatment outcomes for patients with metastatic hindgut NET. The aim of this study was to conduct a comprehensive analysis of clinicopathologic features, treatments and survival in hindgut NET patients. Among patients who were pathologically diagnosed with hindgut NET at Samsung Medical Center between March 2001 and February 2015, 607 were analyzed in this study. Hindgut NETs were defined as NETs that originated from the transverse and distal colon, rectum, and anus. Primary sites included 81 colon (13.3%) and 526 rectum (86.7%). According to the WHO classification, 578 patients (95.2%) had grade 1 NETs, 17 (2.8%) grade 2 NETs, and 12 (2.0%) had neuroendocrine carcinoma (NEC). Forty-two patients (6.9%) had extensive disease, while the majority (93.1%, 565 patients) only exhibited localized disease. The 5- and 10-year survival rates of 565 localized NET patients were 98.1% and 95.3%, respectively. The median OS in 42 patients with extensive disease was 24.8 months (95% CI, 10.7-38.8). Among 565 patients with localized disease, the majority (484 patients, 85.7%) were treated with endoscopic procedure by gastroenterologists. For 42 patients with extensive disease, 17 patients were managed by supportive care, 3 by concurrent chemoradiotherapy (CCRT), and 22 by systemic therapy. Among these 22 patients, 12 patients received only first-line therapy, 8 had second-line, and only 2 patients had third-line therapy. As first-line chemotherapy, the most commonly used regimens were etoposide plus cisplatin (N=7) and long acting octreotide (N=7). During treatment courses, the most commonly used regimen was long-acting octreotide. The median OS in 22 metastatic NET patients receiving systemic therapy was 19.3 months (95% CI, 3.2-35.3). Multivariate analysis in all 607 hindgut NETs patients suggested that the extent and the primary site of disease were significant independent prognostic factors for long term survival. This analysis provides useful information about the clinicopathologic features, treatments and survival outcomes for hindgut NET patients.