James Benjamin C, Aschebrook-Kilfoy Briseis, Cipriani Nicole, Kaplan Edwin L, Angelos Peter, Grogan Raymon H
Ann Surg Oncol. 2016 Feb;23(2):424-33. doi: 10.1245/s10434-015-4901-9.
With the exception of papillary and follicular thyroid cancer, malignant cancers of the thyroid, parathyroid, adrenal, and endocrine pancreas are uncommon. These rare malignancies present a challenge to both the clinician and patient, because few data exist on their incidence or survival. We analyzed the incidence and survival of these rare endocrine cancers (RECs), as well as the trends in incidence over time.
We used the NCI's SEER 18 database (2000-2012) to investigate incidence and survival of rare cancers of the thyroid, parathyroid, adrenal, and endocrine pancreas. Cancers were categorized using the WHO classification systems. We collected data on incidence, gender, stage, size, and survival. Time trends were evaluated from 2000-2002 to 2010-2012.
We identified 36 types of rare cancers in the endocrine organs captured in the SEER database. RECs of the thyroid had the highest combined incidence rate (IR8.26), followed by pancreas (IR 3.24), adrenal (IR 2.71), and parathyroid (IR 0.41). The incidence rate for all rare endocrine organs combined increased 32.4 % during the study period. The majority of the increase was attributable to rare cancers of thyroid, which increased in not only microcarcinomas, but in all sizes. The mean 5-year survival for RECs is 59.56 % (range 2.49–100 %).
This study is a comprehensive analysis ofthe incidence and survival for rare malignant endocrine cancers. There has been an increase in incidence rate of almost all RECs and their survival is low. We hope that our data will serve as a source of information for clinicians as well as bring awareness regarding these uncommon cancers.
除甲状腺乳头状癌和滤泡状癌外，甲状腺、甲状旁腺、肾上腺及内分泌胰腺的恶性肿瘤并不常见。这些罕见的恶性肿瘤给临床医生和患者都带来了挑战，因为关于它们的发病率和生存率的数据很少。我们分析了这些罕见内分泌癌（REC）的发病率、生存率以及发病率随时间的变化趋势。
我们使用美国国立癌症研究所（NCI）的监测、流行病学和最终结果（SEER）18数据库（2000 - 2012年）来研究甲状腺、甲状旁腺、肾上腺及内分泌胰腺罕见癌症的发病率和生存率。癌症根据世界卫生组织（WHO）分类系统进行分类。我们收集了发病率、性别、分期、大小和生存率的数据。评估了2000 - 2002年至2010 - 2012年的时间趋势。
我们在SEER数据库涵盖的内分泌器官中确定了36种罕见癌症类型。甲状腺REC的综合发病率最高（发病率8.26），其次是胰腺（发病率3.24）、肾上腺（发病率2.71）和甲状旁腺（发病率0.41）。在研究期间，所有罕见内分泌器官的综合发病率上升了32.4%。增加的大部分归因于甲状腺罕见癌症，不仅微癌增加，而且各种大小的癌症都增加。REC的平均5年生存率为59.56%（范围2.49 - 100%）。
本研究是对罕见恶性内分泌癌的发病率和生存率的综合分析。几乎所有REC的发病率都有所上升，且其生存率较低。我们希望我们的数据能为临床医生提供信息来源，并提高对这些不常见癌症的认识。
