Pancreatic neuroendocrine tumors(PNETs) are a rare and diverse group of tumors; nonfunctional(NF) PNETs account for the majority of cases. Most patients with NF-PNETs have metastatic disease at the time of presentatio...Pancreatic neuroendocrine tumors(PNETs) are a rare and diverse group of tumors; nonfunctional(NF) PNETs account for the majority of cases. Most patients with NF-PNETs have metastatic disease at the time of presentation. A variety of treatment modalities exist, including medical, liver directed, and surgical treatments. Aggressive surgical management is associated with prolonged survival, however available data are limited by selection bias and the frequent combination of PNETs with carcinoid tumors. Although few patients with metastatic disease will be cured, application of currently available therapies in a multidisciplinary setting can lead to excellent outcomes with prolonged patient survival.展开更多
AIM: To evaluate the efficacy of endoscopic ultrasonography-guided fine needle aspiration(EUS-FNA) for grading pancreatic neuroendocrine tumors(PNETs).METHODS: A total of 22 patients were diagnosed with PNET by EUS-FN...AIM: To evaluate the efficacy of endoscopic ultrasonography-guided fine needle aspiration(EUS-FNA) for grading pancreatic neuroendocrine tumors(PNETs).METHODS: A total of 22 patients were diagnosed with PNET by EUS-FNA between October 2001 and December 2013 at Fukushima Medical University Hospital.Among these cases,we targeted 10 PNET patients who were evaluated according to the World Health Organization(WHO) 2010 classification.Surgery was performed in eight patients,and chemotherapy was performed in two patients due to multiple liver metastases.Specimens obtained by EUS-FNA were first stained with hematoxylin and eosin and then stained with chromogranin,synaptophysin,CD56,and Ki-67.The specimens were graded by the Ki-67 index according to the WHO 2010 classification.Specimens obtained by surgery were graded by the Ki-67 indexand mitotic count(WHO 2010 classification).For the eight specimens obtained by EUS-FNA,the Ki-67 index results were compared with those obtained by surgery.In the two cases treated with chemotherapy,the effects and prognoses were evaluated.RESULTS: The sampling rate for histological diagnosis by EUS-FNA was 100%.No adverse effects were observed.The concordance rate between specimens obtained by EUS-FNA and surgery was 87.5%(7/8).Fo r t h e t w o c a s e s t re a t e d w i t h c h e m o t h e ra p y,case 1 received somatostatin analog therapy and transcatheter arterial infusion(TAI) targeting multiple liver metastases.Subsequent treatment consisted of everolimus.During chemotherapy,the primary tumor remained unconfirmed,although the multiple liver metastases diminished dramatically.Case 2 was classified as neuroendocrine carcinoma(NEC) according to the Ki-67 index of a specimen obtained by EUS-FNA; therefore,cisplatin and irinotecan therapy was started.However,severe adverse effects,including renal failure and diarrhea,were observed,and the therapy regimen was changed to cisplatin and etoposide.TAI targeting multiple liver metastases was performed.Although the liver metastases diminished,the展开更多
目的了解当前胰腺神经内分泌肿瘤(pNEN)影像研究领域的现状及热点问题。方法检索1998–2018年期间Web of Science核心数据库中有关pNEN研究的相关论文,采用文献计量学方法,通过CiteSpace软件进行定量分析。结果共检索到pNEN影像研究领...目的了解当前胰腺神经内分泌肿瘤(pNEN)影像研究领域的现状及热点问题。方法检索1998–2018年期间Web of Science核心数据库中有关pNEN研究的相关论文,采用文献计量学方法,通过CiteSpace软件进行定量分析。结果共检索到pNEN影像研究领域文献190篇,其中发表文献数量居前3位的国家为美国、德国及意大利。pNEN共被引文献的聚类包括:超声内镜、诊断现状、前瞻性评估、囊性胰腺神经内分泌瘤、富血供神经内分泌瘤、无功能胰腺神经内分泌瘤、体素内不相干运动及转移灶。pNEN研究领域热点词包括:细针穿刺活检、CT、诊断、胰腺、癌症、神经内分泌瘤、肿瘤、癌、管理。研究热点关键词聚类有:神经内分泌瘤、胰腺肿块大小、非高功能神经内分泌瘤、CT表现、转移灶、辅助研究、生长抑素类似物、生长抑素、术中超声及多发性内分泌瘤病Ⅰ型。结论 pNEN的精准影像学诊断仍然是该领域的热点问题。展开更多
BACKGROUND Pancreatic mixed serous-neuroendocrine neoplasms(MSNNs)are mixed tumors containing two components with different pathologies,namely,pancreatic serous cystic neoplasm(PSCN)and pancreatic neuroendocrine tumor...BACKGROUND Pancreatic mixed serous-neuroendocrine neoplasms(MSNNs)are mixed tumors containing two components with different pathologies,namely,pancreatic serous cystic neoplasm(PSCN)and pancreatic neuroendocrine tumor(PanNET).For MSNNs,diffuse PSCN involving the whole pancreas is extremely rare,with only eight previous case reports.CASE SUMMARY A 45-year-old Chinese woman,with a free previous medical history and no obvious symptoms,was found to have a pancreatic neoplasm and admitted to our hospital for further diagnosis in March 2018.Abdominal palpation revealed a painless,mobile mass in the epigastrium,and no abnormalities were observed in an examination of the nervous system and ocular system.A computed tomography scan showed multiple cystic lesions involving the whole pancreas ranging in diameter from 0.4 to 2 cm and also revealed an enhanced mass,2.2 cm in diameter,in the head of the pancreas.Moreover,multiple cysts were found in the kidneys bilaterally,and the right lobe of the liver contained a small cyst.A Whipple operation with total pancreatectomy and splenectomy was performed.A diagnosis of pancreatic MSNN was established,consisting of diffuse serous microcystic cystadenoma with a concomitant grade 2 PanNET.Of note,the patient had no personal or family history of Von Hippel-Lindau syndrome or other disease.CONCLUSION We report the first case of MSNN with a diffuse PSCN component involving the entire pancreas in a Chinese woman.It is important to be aware of its relationship with VHL syndrome,and close clinical follow-up is recommended.展开更多
At present the publishing of case reports or case series involving small numbers of cases is controversial. While in the past they were commonly published by most journals, recently a number of prominent journals have...At present the publishing of case reports or case series involving small numbers of cases is controversial. While in the past they were commonly published by most journals, recently a number of prominent journals have either stopped publishing them or markedly reduced the numbers published. However, recently an increasing case is being made for their value and a number of new journals have been started devoted specifically to their publication. One of the arguments used for their value is their prominent role in rare diseases either in their recognition, full description or development of treatments. However this aspect has not been specifically studied. In this editorial this aspect is specifically examined using their role in neuroendocrine tumors as an example. Furthermore, the background of the controversy is briefly reviewed to better understand the context of this editorial.展开更多
胰腺神经内分泌肿瘤是一种常见的胰腺恶性肿瘤,异质性极强,诊治手段多样,因此,统一、实用的分期系统将有助于临床诊治及研究。目前,胰腺神经内分泌肿瘤包括欧洲神经内分泌肿瘤协会(European Neuroendocrine Tumor Society System,ENETS...胰腺神经内分泌肿瘤是一种常见的胰腺恶性肿瘤,异质性极强,诊治手段多样,因此,统一、实用的分期系统将有助于临床诊治及研究。目前,胰腺神经内分泌肿瘤包括欧洲神经内分泌肿瘤协会(European Neuroendocrine Tumor Society System,ENETS)和美国癌症联合委员会(American Joint Committee on Cancer,AJCC)两种分期,然而两种分期系统对临床指导价值有限,且两种分期系统的并存造成了分期系统混乱的局面。复旦大学附属肿瘤医院胰腺肝胆外科通过结合ENETS中TNM的定义及AJCC中分期的定义,建立了改良的分期系统,该分期能更好地区分出不同预后的人群,更适合于指导临床应用,有利于建立统一的临床分期标准。研究结果发表在《临床肿瘤学杂志》(Journal of Clinical Oncology,JCO)上。研究得到了国际同行的高度重视与认可,被评为"2017 Best of JCO:Gastrointestinal edition"。JCO编辑部进行了专篇述评。该研究就改良分期系统的临床应用进行解读。展开更多
文摘Pancreatic neuroendocrine tumors(PNETs) are a rare and diverse group of tumors; nonfunctional(NF) PNETs account for the majority of cases. Most patients with NF-PNETs have metastatic disease at the time of presentation. A variety of treatment modalities exist, including medical, liver directed, and surgical treatments. Aggressive surgical management is associated with prolonged survival, however available data are limited by selection bias and the frequent combination of PNETs with carcinoid tumors. Although few patients with metastatic disease will be cured, application of currently available therapies in a multidisciplinary setting can lead to excellent outcomes with prolonged patient survival.
文摘AIM: To evaluate the efficacy of endoscopic ultrasonography-guided fine needle aspiration(EUS-FNA) for grading pancreatic neuroendocrine tumors(PNETs).METHODS: A total of 22 patients were diagnosed with PNET by EUS-FNA between October 2001 and December 2013 at Fukushima Medical University Hospital.Among these cases,we targeted 10 PNET patients who were evaluated according to the World Health Organization(WHO) 2010 classification.Surgery was performed in eight patients,and chemotherapy was performed in two patients due to multiple liver metastases.Specimens obtained by EUS-FNA were first stained with hematoxylin and eosin and then stained with chromogranin,synaptophysin,CD56,and Ki-67.The specimens were graded by the Ki-67 index according to the WHO 2010 classification.Specimens obtained by surgery were graded by the Ki-67 indexand mitotic count(WHO 2010 classification).For the eight specimens obtained by EUS-FNA,the Ki-67 index results were compared with those obtained by surgery.In the two cases treated with chemotherapy,the effects and prognoses were evaluated.RESULTS: The sampling rate for histological diagnosis by EUS-FNA was 100%.No adverse effects were observed.The concordance rate between specimens obtained by EUS-FNA and surgery was 87.5%(7/8).Fo r t h e t w o c a s e s t re a t e d w i t h c h e m o t h e ra p y,case 1 received somatostatin analog therapy and transcatheter arterial infusion(TAI) targeting multiple liver metastases.Subsequent treatment consisted of everolimus.During chemotherapy,the primary tumor remained unconfirmed,although the multiple liver metastases diminished dramatically.Case 2 was classified as neuroendocrine carcinoma(NEC) according to the Ki-67 index of a specimen obtained by EUS-FNA; therefore,cisplatin and irinotecan therapy was started.However,severe adverse effects,including renal failure and diarrhea,were observed,and the therapy regimen was changed to cisplatin and etoposide.TAI targeting multiple liver metastases was performed.Although the liver metastases diminished,the
文摘目的了解当前胰腺神经内分泌肿瘤(pNEN)影像研究领域的现状及热点问题。方法检索1998–2018年期间Web of Science核心数据库中有关pNEN研究的相关论文,采用文献计量学方法,通过CiteSpace软件进行定量分析。结果共检索到pNEN影像研究领域文献190篇,其中发表文献数量居前3位的国家为美国、德国及意大利。pNEN共被引文献的聚类包括:超声内镜、诊断现状、前瞻性评估、囊性胰腺神经内分泌瘤、富血供神经内分泌瘤、无功能胰腺神经内分泌瘤、体素内不相干运动及转移灶。pNEN研究领域热点词包括:细针穿刺活检、CT、诊断、胰腺、癌症、神经内分泌瘤、肿瘤、癌、管理。研究热点关键词聚类有:神经内分泌瘤、胰腺肿块大小、非高功能神经内分泌瘤、CT表现、转移灶、辅助研究、生长抑素类似物、生长抑素、术中超声及多发性内分泌瘤病Ⅰ型。结论 pNEN的精准影像学诊断仍然是该领域的热点问题。
基金National Natural Science Foundation of China,No.81702596
文摘BACKGROUND Pancreatic mixed serous-neuroendocrine neoplasms(MSNNs)are mixed tumors containing two components with different pathologies,namely,pancreatic serous cystic neoplasm(PSCN)and pancreatic neuroendocrine tumor(PanNET).For MSNNs,diffuse PSCN involving the whole pancreas is extremely rare,with only eight previous case reports.CASE SUMMARY A 45-year-old Chinese woman,with a free previous medical history and no obvious symptoms,was found to have a pancreatic neoplasm and admitted to our hospital for further diagnosis in March 2018.Abdominal palpation revealed a painless,mobile mass in the epigastrium,and no abnormalities were observed in an examination of the nervous system and ocular system.A computed tomography scan showed multiple cystic lesions involving the whole pancreas ranging in diameter from 0.4 to 2 cm and also revealed an enhanced mass,2.2 cm in diameter,in the head of the pancreas.Moreover,multiple cysts were found in the kidneys bilaterally,and the right lobe of the liver contained a small cyst.A Whipple operation with total pancreatectomy and splenectomy was performed.A diagnosis of pancreatic MSNN was established,consisting of diffuse serous microcystic cystadenoma with a concomitant grade 2 PanNET.Of note,the patient had no personal or family history of Von Hippel-Lindau syndrome or other disease.CONCLUSION We report the first case of MSNN with a diffuse PSCN component involving the entire pancreas in a Chinese woman.It is important to be aware of its relationship with VHL syndrome,and close clinical follow-up is recommended.
基金Supported by Intramural Research Funds of NIDDK,NIH
文摘At present the publishing of case reports or case series involving small numbers of cases is controversial. While in the past they were commonly published by most journals, recently a number of prominent journals have either stopped publishing them or markedly reduced the numbers published. However, recently an increasing case is being made for their value and a number of new journals have been started devoted specifically to their publication. One of the arguments used for their value is their prominent role in rare diseases either in their recognition, full description or development of treatments. However this aspect has not been specifically studied. In this editorial this aspect is specifically examined using their role in neuroendocrine tumors as an example. Furthermore, the background of the controversy is briefly reviewed to better understand the context of this editorial.
文摘胰腺神经内分泌肿瘤是一种常见的胰腺恶性肿瘤,异质性极强,诊治手段多样,因此,统一、实用的分期系统将有助于临床诊治及研究。目前,胰腺神经内分泌肿瘤包括欧洲神经内分泌肿瘤协会(European Neuroendocrine Tumor Society System,ENETS)和美国癌症联合委员会(American Joint Committee on Cancer,AJCC)两种分期,然而两种分期系统对临床指导价值有限,且两种分期系统的并存造成了分期系统混乱的局面。复旦大学附属肿瘤医院胰腺肝胆外科通过结合ENETS中TNM的定义及AJCC中分期的定义,建立了改良的分期系统,该分期能更好地区分出不同预后的人群,更适合于指导临床应用,有利于建立统一的临床分期标准。研究结果发表在《临床肿瘤学杂志》(Journal of Clinical Oncology,JCO)上。研究得到了国际同行的高度重视与认可,被评为"2017 Best of JCO:Gastrointestinal edition"。JCO编辑部进行了专篇述评。该研究就改良分期系统的临床应用进行解读。
