BACKGROUND Numerous studies have indicated that the temozolomide and capecitabine regimen(TEMCAP)exhibits a certain level of efficacy in treating advanced,welldifferentiated gastroenteropancreatic neuroendocrine tumor...BACKGROUND Numerous studies have indicated that the temozolomide and capecitabine regimen(TEMCAP)exhibits a certain level of efficacy in treating advanced,welldifferentiated gastroenteropancreatic neuroendocrine tumors(GEP-NET).However,published data from Peru are limited.We hypothesize that this regimen could be a viable therapeutic option for advanced GEP-NET in the Peruvian population.AIM To evaluate overall survival(OS)in patients diagnosed with advanced GEP-NET treated with TEMCAP at the Instituto Nacional de Enfermedades Neoplásicas(INEN)in Lima-Perú.METHODS A retrospective review was conducted to identify patients with GEP-NEN treated with the TEMCAP regimen between 2011 and 2021 at the INEN.A total of thirtyeight patients were included in the final analysis:Thirty-five received TEMCAP as a first-line treatment,and three as a second-line treatment.The primary objective was to evaluate OS.The efficacy and safety of TEMCAP were assessed until the occurrence of unacceptable toxicity or disease progression.Survival outcomes were estimated using the Kaplan-Meier method.RESULTS The median age of the patients was 52 years(range 24-77 years),and 53.3%were female.The most common symptoms at diagnosis were abdominal pain in 31 patients(81.6%).Primary tumors included 12 in the rectum(31.6%),11 in the pancreas(28.9%),3 in the ileum(7.9%),2 in the mesentery(5.3%),2 in the small intestine(5.3%),1 in the appendix(2.6%),1 in the stomach(2.6%)and 6 cases of liver metastasis of unknown primary(15.8%).Five were neuroendocrine tumors(NET)G1(13.2%),33 were NET G2(86.8%),five had Ki67<3%(13.2%),and 33 had Ki67 between 3%and 20%(86.8%).TEMCAP was administered to 35(92.1%)patients as first-line treatment.OS at 12,36,and 60 months was estimated in 80%,66%,and 42%,respectively,with a median OS of 49 months.CONCLUSION TEMCAP therapy is a viable first-line option regarding efficacy and tolerability in areas where standard therapy is inaccessible.展开更多
Mixed neuroendocrine non-neuroendocrine neoplasms(MiNENs)are rare mixed tumors containing both neuroendocrine and non-neuroendocrine components that occupy at least 30%of the whole tumor.Biologically,both components a...Mixed neuroendocrine non-neuroendocrine neoplasms(MiNENs)are rare mixed tumors containing both neuroendocrine and non-neuroendocrine components that occupy at least 30%of the whole tumor.Biologically,both components appear to derive from an identical cellular precursor undergoing early dual differentiation or late transdifferentiation.While our understanding of MiNENs has improved in recent years,many areas of uncertainty remain.In this context,setting diagnostic criteria capable of capturing the continuum of disease biology while providing clinically meaningful information in terms of prognosis and response to treatments appears vital to advance the field and improve patients’outcomes.Evidence is needed to generate robust classification schemes,and multi-institutional cooperation will likely play a crucial role in building adequately powered cohorts to address some of the most pressing questions discussed in this Editorial.What is the minimum representation for each component needed to define MiNENs?How can the epidemiology of MiNENs change according to different diagnostic definitions?How can we generate the clinical evidence nee-ded to optimize the management of MiNENs?展开更多
Objective： To analyze the angiography appearance of liver metastases from gastroenteropancreatic neuroendocrine tumors （GEP-NETs）, and evaluate the clinical efficacy and prognostic factors of interventional treatme...Objective： To analyze the angiography appearance of liver metastases from gastroenteropancreatic neuroendocrine tumors （GEP-NETs）, and evaluate the clinical efficacy and prognostic factors of interventional treatment for hepatic metastases. Methods： Fifty GEP-NETs patients with hepatic metastases were treated from January 2012 to December 2016, and received transarterial embolization （TAE） in the hepatic tumor or hepatic arterial infusion chemotherapy （HAIC）. All patients received 179 times of the intervention therapy in total. Results： Blood supplies were identified in the 50 eases with angiography, which showed that 35 cases had abundant vessels, while 15 eases had poor blood supply. Twenty-two cases were found either collateral blood supply, or portal vein invasion or arterial-portal vein fistula. The best curative efficacy was complete remission （CR） in 4 cases, partial remission （PR） in 28 cases and stable disease （SD） in 18 eases during the process of treatment. The angiography （P=0.047） and the frequency of intervention （P=0.037） showed significantly statistical difference with Kaplan-Meier analysis. The Cox analysis showed that more than 3 times of interventional therapy was an independent prognostic factor. Conclusions： Interventional treatment is safe and effective for GEP-NETs, and is beneficial to patients with main hepatic metastases after endocrine therapy.展开更多
Neuroendocrine(NE) gastroenteropancreatic tumors are a heterogeneous group of neoplasias arising from neuroendocrine cells of the embryological gut. Their incidence have increased significantly over the past 3 decades...Neuroendocrine(NE) gastroenteropancreatic tumors are a heterogeneous group of neoplasias arising from neuroendocrine cells of the embryological gut. Their incidence have increased significantly over the past 3 decades probably due to the improvements in imaging and diagnosis. The recent advances in molecular biology have translated into an expansion of therapeutic approaches to these patients. Somatostatin analogs, which initially were approved for control of hormonal syndromes, have recently been proven to inhibit tumor growth. Several new drugs such as antiangiogenics and others targeting mammalian target of rapamycin pathways have been approved to treat progressive pancreatic neuroendocrine tumors(NETs) although their role in nonpancreatic is still controversial. The treatment of NETs requires a coordinated multidisciplinary approach. The management of localized NETs primarily involves surgical resection followed by surveillance. However, the treatment of unresectable and/or metastatic disease may involve a combination of surgical resection, systemic therapy, and liver-directed therapies with the goal of alleviating symptoms of peptide release and controlling tumor growth. This article will review the current therapeutic strategies for metastatic gastroenteropancreatic NETs and will take a glimpse into the future approaches.展开更多
Theranostics is the highly targeted molecular imaging and therapy of tumors.Targeted peptide receptor radionuclide therapy has taken the lead in demonstrating the safety and effectiveness of this molecular approach to...Theranostics is the highly targeted molecular imaging and therapy of tumors.Targeted peptide receptor radionuclide therapy has taken the lead in demonstrating the safety and effectiveness of this molecular approach to treating cancers.Metastatic,well-differentiated gastroenteropancreatic neuroendocrine tumors may be most effectively imaged and treated with DOTATATE ligands.We review the current practice,safety,advantages,and limitations of DOTATATE based theranostics.Finally,we briefly describe the exciting new areas of development and future directions of gastroenteropancreatic neuroendocrine tumor theranostics.展开更多
BACKGROUND Long non-coding RNAs(lncRNAs) are a kind of single-stranded RNA of more than 200 nucleotides in length and have no protein-coding function. Amounting studies have indicated that lncRNAs could play a vital r...BACKGROUND Long non-coding RNAs(lncRNAs) are a kind of single-stranded RNA of more than 200 nucleotides in length and have no protein-coding function. Amounting studies have indicated that lncRNAs could play a vital role in the initiation and development of cancers, including gastric cancer(GC). Considering the crucial functions of lncRNAs, the identification and exploration of novel lncRNAs in GC is necessary.AIM To identify independent prognostic markers for the whole gastroenteropancreatic neuroendocrine tumor(GEP-NET) group.METHODS Ninety-three patients diagnosed with GEP-NETs within a specified period were included in this study. Patient data were retrospectively analyzed. The relationships between all independent variables and 5-year survival status calculated during the follow-up period(months) were assessed. In addition, the relationships between the independent variables were investigated.RESULTS When 5-year survival rate was compared, a statistically significant relationship between the age at diagnosis, male gender, tumor size, tumor stage, liver and/or distant metastasis, and tumor grade determined by the Ki-67 level and mitotic count, and the level of C-reactive protein(CRP), was observed. The meansurvival(overall survival) of the study group was 102.5 ± 6.3(SD) mo. The percentages of 1, 3 and 5-year survival were 90%, 72%, and 61%, respectively. In63 of 93 patients, Ki-67 and the mitotic count determined the same grade. The Ki-67 levels in 29 patients and the mitotic count in only 1 patient were in the higher grade. The risk of death increased by 4% for every 1 year increase at the diagnosis age and was 2.0-fold higher for male patients, 3.0-fold higher for G3 according to the mitotic count, 3.7-fold higher for G3 according to the Ki-67 level, 12.7-fold higher for cases with tumor stage 3 or 4 by a 1 cm increase in the ratio of 9% in tumor size, and 6.1-fold higher for patients with liver metastasis for every 1 mg/dL increase in the ratio of 1.5% in CRP level. There was a significant difference between pancreatic and stomach NETs in favor of stomach tumors in terms of survival.CONCLUSION Tumor site, stage, grade and Ki-67 level affected patient survival, and it was observed that CRP affected disease progression(particularly if it was > 20 mg/dL). However, a relationship between surgical resection of the lesion and survival was not shown. Larger scale prospective studies are required to determine whether CRP level may be a poor prognostic factor for the entire GEPNET group.展开更多
Pancreatic neuroendocrine tumors(PNETs)are known to be the second most common epithelial malignancy of the pancreas.PNETs can be listed among the slowest growing as well as the fastest growing human cancers.The preval...Pancreatic neuroendocrine tumors(PNETs)are known to be the second most common epithelial malignancy of the pancreas.PNETs can be listed among the slowest growing as well as the fastest growing human cancers.The prevalence of PNETs is deceptively low;however,its incidence has significantly increased over the past decades.According to the American Cancer Society’s estimate,about 4032(>7%of all pancreatic malignancies)individuals will be diagnosed with PNETs in 2020.PNETs often cause severe morbidity due to excessive secretion of hormones(such as serotonin)and/or overall tumor mass.Patients can live for many years(except for those patients with poorly differentiated G3 neuroendocrine tumors);thus,the prevalence of the tumors that is the number of patients actually dealing with the disease at any given time is fairly high because the survival is much longer than pancreatic ductal adenocarcinoma.Due to significant heterogeneity,the management of PNETs is very complex and remains an unmet clinical challenge.In terms of research studies,modest improvements have been made over the past decades in the identification of potential oncogenic drivers in order to enhance the quality of life and increase survival for this growing population of patients.Unfortunately,the majority of systematic therapies approved for the management of advanced stage PNETs lack objective response or at most result in modest benefits in survival.In this review,we aim to discuss the broad challenges associated with the management and the study of PNETs.展开更多
Gastroenteropancreatic neuroendocrine neoplasms comprise a heterogeneous group of tumors that differ in their pathogenesis,hormonal syndromes produced,biological behavior and consequently,in their requirement for and/...Gastroenteropancreatic neuroendocrine neoplasms comprise a heterogeneous group of tumors that differ in their pathogenesis,hormonal syndromes produced,biological behavior and consequently,in their requirement for and/or response to specific chemotherapeutic agents and molecular targeted therapies.Various imaging techniques are available for functional and morphological evaluation of these neoplasms and the selection of investigations performed in each patient should be customized to the clinical question.Also,with the increased availability of cross sectional imaging,these neoplasms are increasingly being detected incidentally in routine radiology practice.This article is a review of the various imaging modalities currently used in the evaluation of neuroendocrine neoplasms,along with a discussion of the role of advanced imaging techniques and a glimpse into the newer imaging horizons,mostly in the research stage.展开更多
Gastroenteropancreatic neuroendocrine neoplasms are a heterogenous group of rare neoplasms that are increasingly being discovered,often incidentally,throughout the gastrointestinal tract with varying degrees of activi...Gastroenteropancreatic neuroendocrine neoplasms are a heterogenous group of rare neoplasms that are increasingly being discovered,often incidentally,throughout the gastrointestinal tract with varying degrees of activity and malignant potential.Confusing nomenclature has added to the complexity of managing these lesions.The term carcinoid tumor and embryonic classification have been replaced with gastroenteropancreatic neuroendocrine neoplasm,which includes gastrointestinal neuroendocrine and pancreatic neuroendocrine neoplasms.A comprehensive multidisciplinary approach is important for clinicians to diagnose,stage and manage these lesions.While histological diagnosis is the gold standard,recent advancements in endoscopy,conventional imaging,functional imaging,and serum biomarkers complement histology for tailoring specific treatment options.In light of developing technology,our review sets out to characterize diagnostic and therapeutic advancements for managing gastroenteropancreatic neuroendocrine tumors,including innovations in radiolabeled peptide imaging,circulating biomarkers,and endoscopic treatment approaches adapted to different locations throughout the gastrointestinal system.展开更多
Gastric neuroendocrine tumors are gastric neoplasms originating from enterochromaffin type cells and are inserted in a larger group,named gastroenteropancreatic neuroendocrine tumors.They are considered rare and varia...Gastric neuroendocrine tumors are gastric neoplasms originating from enterochromaffin type cells and are inserted in a larger group,named gastroenteropancreatic neuroendocrine tumors.They are considered rare and variable in terms of their clinical,morphological and functional characteristics and may be indolent or aggressive.They are classified into types I,II and III,according to their pathophysiology,behavior and treatment.Their diagnosis occurs,in most cases,incidentally during upper digestive endoscopies,presenting as simple gastric polyps.Most cases(type I and type II)are related to hypergastrinemia,can be multiple and are treated by endoscopic resection,whenever possible.The use of somatostatin analogs for tumor control may be one of the options for therapy,in addition to total or subtotal gastrectomy for selected cases.Adjuvant chemotherapy is only reserved for poorly differentiated neuroendocrine carcinomas.Although rare,gastric neuroendocrine tumors have an increasing incidence over the years,therefore deserving more comprehensive studies on its adequate treatment.The present study reviews and updates management recommendations for gastric neuroendocrine tumors.展开更多
Pancreatic neuroendocrine tumor(P-NET) is rare and slow-growing. Current classifications predict its progno-sis and postoperative recurrence. Curative resection is ideal, although often difficult, because over 80% of ...Pancreatic neuroendocrine tumor(P-NET) is rare and slow-growing. Current classifications predict its progno-sis and postoperative recurrence. Curative resection is ideal, although often difficult, because over 80% of pa-tients have unresectable multiple liver metastases and extrahepatic metastasis. Aggressive surgery for liver metastases is important to improve survival. Aggressive or cytoreductive surgery for liver metastases is indi-cated to reduce hormone levels and improve symptoms and prognosis. Liver transplantation was originally con-ceived as an ideal therapy for unresectable liver metas-tases. Unfortunately, there is no clear consensus on the role and timing of surgery for primary tumor and liver metastases. Surgeons still face questions in deciding the best surgical scenario in patients with P-NET with unresectable liver metastases.展开更多
神经内分泌肿瘤(neuroendocrine neoplasm,NEN)的发病率和患病率近年来迅速增长,其中以原发于胃肠道和胰腺的NEN最常见。2022年,美国国家综合癌症网络(National Comprehensive Cancer Network,NCCN)NEN指南及中国胃肠胰NEN专家共识进行...神经内分泌肿瘤(neuroendocrine neoplasm,NEN)的发病率和患病率近年来迅速增长,其中以原发于胃肠道和胰腺的NEN最常见。2022年,美国国家综合癌症网络(National Comprehensive Cancer Network,NCCN)NEN指南及中国胃肠胰NEN专家共识进行了更新。本文主要参照比对2022年NCCN指南及2022版中国胃肠胰NEN专家共识对NEN的诊疗新进展进行评述。展开更多
目的:分析高级别胃肠胰神经内分泌肿瘤(high-grade G3 gastroenteropancreatic neuroendocrine tumors,GEP NET G3)的临床病理特征及生存预后影响因素。方法:回顾性分析2012年9月至2019年7月间北京大学肿瘤医院收治的86例GEP NET G3,收...目的:分析高级别胃肠胰神经内分泌肿瘤(high-grade G3 gastroenteropancreatic neuroendocrine tumors,GEP NET G3)的临床病理特征及生存预后影响因素。方法:回顾性分析2012年9月至2019年7月间北京大学肿瘤医院收治的86例GEP NET G3,收集临床病理及随访资料,采用单因素Log-rank检验和多因素Cox回归模型进行预后影响因素分析。结果:86例GEP NET G3的原发肿瘤部位包括胰腺(40例)、胃肠(37例)以及原发肿瘤不明(9例)。镜检肿瘤均分化良好,呈器官样、巢状生长,局灶可见假腺样、细条索样或缎带样结构。免疫组织化学分析显示,肿瘤的Ki-67指数范围为21%~60%,中位值为30%,生长抑素受体2(somatostatin receptor type 2,SSTR2)的阳性率为83.9%(26/31),在检测病例中没有发现微卫星不稳定和P53异常表达(分别为0/24、0/11)。生存分析表明,获得随访的76例患者,27例患者死亡,中位生存时间为48.6(26.6~70.6)个月,确诊时有无远处转移和能否进行根治性手术切除对患者的总生存影响差异有统计学意义(P<0.05),但患者的年龄、性别、原发肿瘤部位、确诊时血清神经元特异性烯醇化酶(neuron specific enolase,NSE)水平以及Ki-67指数对患者的总生存影响无显著差异(P>0.05)。多因素分析显示,确诊时有远处转移是影响患者生存的独立危险因素(P=0.01;HR=7.33,95%CI:1.56~34.10)。结论:GEP NET G3的确诊依赖于组织病理学和免疫组织化学检测,易发生远处转移是其临床特点和主要的预后影响因素。展开更多
文摘BACKGROUND Numerous studies have indicated that the temozolomide and capecitabine regimen(TEMCAP)exhibits a certain level of efficacy in treating advanced,welldifferentiated gastroenteropancreatic neuroendocrine tumors(GEP-NET).However,published data from Peru are limited.We hypothesize that this regimen could be a viable therapeutic option for advanced GEP-NET in the Peruvian population.AIM To evaluate overall survival(OS)in patients diagnosed with advanced GEP-NET treated with TEMCAP at the Instituto Nacional de Enfermedades Neoplásicas(INEN)in Lima-Perú.METHODS A retrospective review was conducted to identify patients with GEP-NEN treated with the TEMCAP regimen between 2011 and 2021 at the INEN.A total of thirtyeight patients were included in the final analysis:Thirty-five received TEMCAP as a first-line treatment,and three as a second-line treatment.The primary objective was to evaluate OS.The efficacy and safety of TEMCAP were assessed until the occurrence of unacceptable toxicity or disease progression.Survival outcomes were estimated using the Kaplan-Meier method.RESULTS The median age of the patients was 52 years(range 24-77 years),and 53.3%were female.The most common symptoms at diagnosis were abdominal pain in 31 patients(81.6%).Primary tumors included 12 in the rectum(31.6%),11 in the pancreas(28.9%),3 in the ileum(7.9%),2 in the mesentery(5.3%),2 in the small intestine(5.3%),1 in the appendix(2.6%),1 in the stomach(2.6%)and 6 cases of liver metastasis of unknown primary(15.8%).Five were neuroendocrine tumors(NET)G1(13.2%),33 were NET G2(86.8%),five had Ki67<3%(13.2%),and 33 had Ki67 between 3%and 20%(86.8%).TEMCAP was administered to 35(92.1%)patients as first-line treatment.OS at 12,36,and 60 months was estimated in 80%,66%,and 42%,respectively,with a median OS of 49 months.CONCLUSION TEMCAP therapy is a viable first-line option regarding efficacy and tolerability in areas where standard therapy is inaccessible.
基金Supported by The Associazione Italiana per la Ricerca sul Cancro,No.23583The Apulia Region(“Il Team Oncogenomico:Modello operativo per la riduzione dei costi dell’assistenza sanitaria in Oncologia”Project).
文摘Mixed neuroendocrine non-neuroendocrine neoplasms(MiNENs)are rare mixed tumors containing both neuroendocrine and non-neuroendocrine components that occupy at least 30%of the whole tumor.Biologically,both components appear to derive from an identical cellular precursor undergoing early dual differentiation or late transdifferentiation.While our understanding of MiNENs has improved in recent years,many areas of uncertainty remain.In this context,setting diagnostic criteria capable of capturing the continuum of disease biology while providing clinically meaningful information in terms of prognosis and response to treatments appears vital to advance the field and improve patients’outcomes.Evidence is needed to generate robust classification schemes,and multi-institutional cooperation will likely play a crucial role in building adequately powered cohorts to address some of the most pressing questions discussed in this Editorial.What is the minimum representation for each component needed to define MiNENs?How can the epidemiology of MiNENs change according to different diagnostic definitions?How can we generate the clinical evidence nee-ded to optimize the management of MiNENs?
基金supported by the National Natural Science Foundation of China(No.81571781)
文摘Objective： To analyze the angiography appearance of liver metastases from gastroenteropancreatic neuroendocrine tumors （GEP-NETs）, and evaluate the clinical efficacy and prognostic factors of interventional treatment for hepatic metastases. Methods： Fifty GEP-NETs patients with hepatic metastases were treated from January 2012 to December 2016, and received transarterial embolization （TAE） in the hepatic tumor or hepatic arterial infusion chemotherapy （HAIC）. All patients received 179 times of the intervention therapy in total. Results： Blood supplies were identified in the 50 eases with angiography, which showed that 35 cases had abundant vessels, while 15 eases had poor blood supply. Twenty-two cases were found either collateral blood supply, or portal vein invasion or arterial-portal vein fistula. The best curative efficacy was complete remission （CR） in 4 cases, partial remission （PR） in 28 cases and stable disease （SD） in 18 eases during the process of treatment. The angiography （P=0.047） and the frequency of intervention （P=0.037） showed significantly statistical difference with Kaplan-Meier analysis. The Cox analysis showed that more than 3 times of interventional therapy was an independent prognostic factor. Conclusions： Interventional treatment is safe and effective for GEP-NETs, and is beneficial to patients with main hepatic metastases after endocrine therapy.
文摘Neuroendocrine(NE) gastroenteropancreatic tumors are a heterogeneous group of neoplasias arising from neuroendocrine cells of the embryological gut. Their incidence have increased significantly over the past 3 decades probably due to the improvements in imaging and diagnosis. The recent advances in molecular biology have translated into an expansion of therapeutic approaches to these patients. Somatostatin analogs, which initially were approved for control of hormonal syndromes, have recently been proven to inhibit tumor growth. Several new drugs such as antiangiogenics and others targeting mammalian target of rapamycin pathways have been approved to treat progressive pancreatic neuroendocrine tumors(NETs) although their role in nonpancreatic is still controversial. The treatment of NETs requires a coordinated multidisciplinary approach. The management of localized NETs primarily involves surgical resection followed by surveillance. However, the treatment of unresectable and/or metastatic disease may involve a combination of surgical resection, systemic therapy, and liver-directed therapies with the goal of alleviating symptoms of peptide release and controlling tumor growth. This article will review the current therapeutic strategies for metastatic gastroenteropancreatic NETs and will take a glimpse into the future approaches.
文摘Theranostics is the highly targeted molecular imaging and therapy of tumors.Targeted peptide receptor radionuclide therapy has taken the lead in demonstrating the safety and effectiveness of this molecular approach to treating cancers.Metastatic,well-differentiated gastroenteropancreatic neuroendocrine tumors may be most effectively imaged and treated with DOTATATE ligands.We review the current practice,safety,advantages,and limitations of DOTATATE based theranostics.Finally,we briefly describe the exciting new areas of development and future directions of gastroenteropancreatic neuroendocrine tumor theranostics.
文摘BACKGROUND Long non-coding RNAs(lncRNAs) are a kind of single-stranded RNA of more than 200 nucleotides in length and have no protein-coding function. Amounting studies have indicated that lncRNAs could play a vital role in the initiation and development of cancers, including gastric cancer(GC). Considering the crucial functions of lncRNAs, the identification and exploration of novel lncRNAs in GC is necessary.AIM To identify independent prognostic markers for the whole gastroenteropancreatic neuroendocrine tumor(GEP-NET) group.METHODS Ninety-three patients diagnosed with GEP-NETs within a specified period were included in this study. Patient data were retrospectively analyzed. The relationships between all independent variables and 5-year survival status calculated during the follow-up period(months) were assessed. In addition, the relationships between the independent variables were investigated.RESULTS When 5-year survival rate was compared, a statistically significant relationship between the age at diagnosis, male gender, tumor size, tumor stage, liver and/or distant metastasis, and tumor grade determined by the Ki-67 level and mitotic count, and the level of C-reactive protein(CRP), was observed. The meansurvival(overall survival) of the study group was 102.5 ± 6.3(SD) mo. The percentages of 1, 3 and 5-year survival were 90%, 72%, and 61%, respectively. In63 of 93 patients, Ki-67 and the mitotic count determined the same grade. The Ki-67 levels in 29 patients and the mitotic count in only 1 patient were in the higher grade. The risk of death increased by 4% for every 1 year increase at the diagnosis age and was 2.0-fold higher for male patients, 3.0-fold higher for G3 according to the mitotic count, 3.7-fold higher for G3 according to the Ki-67 level, 12.7-fold higher for cases with tumor stage 3 or 4 by a 1 cm increase in the ratio of 9% in tumor size, and 6.1-fold higher for patients with liver metastasis for every 1 mg/dL increase in the ratio of 1.5% in CRP level. There was a significant difference between pancreatic and stomach NETs in favor of stomach tumors in terms of survival.CONCLUSION Tumor site, stage, grade and Ki-67 level affected patient survival, and it was observed that CRP affected disease progression(particularly if it was > 20 mg/dL). However, a relationship between surgical resection of the lesion and survival was not shown. Larger scale prospective studies are required to determine whether CRP level may be a poor prognostic factor for the entire GEPNET group.
文摘Pancreatic neuroendocrine tumors(PNETs)are known to be the second most common epithelial malignancy of the pancreas.PNETs can be listed among the slowest growing as well as the fastest growing human cancers.The prevalence of PNETs is deceptively low;however,its incidence has significantly increased over the past decades.According to the American Cancer Society’s estimate,about 4032(>7%of all pancreatic malignancies)individuals will be diagnosed with PNETs in 2020.PNETs often cause severe morbidity due to excessive secretion of hormones(such as serotonin)and/or overall tumor mass.Patients can live for many years(except for those patients with poorly differentiated G3 neuroendocrine tumors);thus,the prevalence of the tumors that is the number of patients actually dealing with the disease at any given time is fairly high because the survival is much longer than pancreatic ductal adenocarcinoma.Due to significant heterogeneity,the management of PNETs is very complex and remains an unmet clinical challenge.In terms of research studies,modest improvements have been made over the past decades in the identification of potential oncogenic drivers in order to enhance the quality of life and increase survival for this growing population of patients.Unfortunately,the majority of systematic therapies approved for the management of advanced stage PNETs lack objective response or at most result in modest benefits in survival.In this review,we aim to discuss the broad challenges associated with the management and the study of PNETs.
文摘Gastroenteropancreatic neuroendocrine neoplasms comprise a heterogeneous group of tumors that differ in their pathogenesis,hormonal syndromes produced,biological behavior and consequently,in their requirement for and/or response to specific chemotherapeutic agents and molecular targeted therapies.Various imaging techniques are available for functional and morphological evaluation of these neoplasms and the selection of investigations performed in each patient should be customized to the clinical question.Also,with the increased availability of cross sectional imaging,these neoplasms are increasingly being detected incidentally in routine radiology practice.This article is a review of the various imaging modalities currently used in the evaluation of neuroendocrine neoplasms,along with a discussion of the role of advanced imaging techniques and a glimpse into the newer imaging horizons,mostly in the research stage.
文摘Gastroenteropancreatic neuroendocrine neoplasms are a heterogenous group of rare neoplasms that are increasingly being discovered,often incidentally,throughout the gastrointestinal tract with varying degrees of activity and malignant potential.Confusing nomenclature has added to the complexity of managing these lesions.The term carcinoid tumor and embryonic classification have been replaced with gastroenteropancreatic neuroendocrine neoplasm,which includes gastrointestinal neuroendocrine and pancreatic neuroendocrine neoplasms.A comprehensive multidisciplinary approach is important for clinicians to diagnose,stage and manage these lesions.While histological diagnosis is the gold standard,recent advancements in endoscopy,conventional imaging,functional imaging,and serum biomarkers complement histology for tailoring specific treatment options.In light of developing technology,our review sets out to characterize diagnostic and therapeutic advancements for managing gastroenteropancreatic neuroendocrine tumors,including innovations in radiolabeled peptide imaging,circulating biomarkers,and endoscopic treatment approaches adapted to different locations throughout the gastrointestinal system.
文摘Gastric neuroendocrine tumors are gastric neoplasms originating from enterochromaffin type cells and are inserted in a larger group,named gastroenteropancreatic neuroendocrine tumors.They are considered rare and variable in terms of their clinical,morphological and functional characteristics and may be indolent or aggressive.They are classified into types I,II and III,according to their pathophysiology,behavior and treatment.Their diagnosis occurs,in most cases,incidentally during upper digestive endoscopies,presenting as simple gastric polyps.Most cases(type I and type II)are related to hypergastrinemia,can be multiple and are treated by endoscopic resection,whenever possible.The use of somatostatin analogs for tumor control may be one of the options for therapy,in addition to total or subtotal gastrectomy for selected cases.Adjuvant chemotherapy is only reserved for poorly differentiated neuroendocrine carcinomas.Although rare,gastric neuroendocrine tumors have an increasing incidence over the years,therefore deserving more comprehensive studies on its adequate treatment.The present study reviews and updates management recommendations for gastric neuroendocrine tumors.
文摘Pancreatic neuroendocrine tumor(P-NET) is rare and slow-growing. Current classifications predict its progno-sis and postoperative recurrence. Curative resection is ideal, although often difficult, because over 80% of pa-tients have unresectable multiple liver metastases and extrahepatic metastasis. Aggressive surgery for liver metastases is important to improve survival. Aggressive or cytoreductive surgery for liver metastases is indi-cated to reduce hormone levels and improve symptoms and prognosis. Liver transplantation was originally con-ceived as an ideal therapy for unresectable liver metas-tases. Unfortunately, there is no clear consensus on the role and timing of surgery for primary tumor and liver metastases. Surgeons still face questions in deciding the best surgical scenario in patients with P-NET with unresectable liver metastases.
文摘神经内分泌肿瘤(neuroendocrine neoplasm,NEN)的发病率和患病率近年来迅速增长,其中以原发于胃肠道和胰腺的NEN最常见。2022年,美国国家综合癌症网络(National Comprehensive Cancer Network,NCCN)NEN指南及中国胃肠胰NEN专家共识进行了更新。本文主要参照比对2022年NCCN指南及2022版中国胃肠胰NEN专家共识对NEN的诊疗新进展进行评述。
文摘目的:分析高级别胃肠胰神经内分泌肿瘤(high-grade G3 gastroenteropancreatic neuroendocrine tumors,GEP NET G3)的临床病理特征及生存预后影响因素。方法:回顾性分析2012年9月至2019年7月间北京大学肿瘤医院收治的86例GEP NET G3,收集临床病理及随访资料,采用单因素Log-rank检验和多因素Cox回归模型进行预后影响因素分析。结果:86例GEP NET G3的原发肿瘤部位包括胰腺(40例)、胃肠(37例)以及原发肿瘤不明(9例)。镜检肿瘤均分化良好,呈器官样、巢状生长,局灶可见假腺样、细条索样或缎带样结构。免疫组织化学分析显示,肿瘤的Ki-67指数范围为21%~60%,中位值为30%,生长抑素受体2(somatostatin receptor type 2,SSTR2)的阳性率为83.9%(26/31),在检测病例中没有发现微卫星不稳定和P53异常表达(分别为0/24、0/11)。生存分析表明,获得随访的76例患者,27例患者死亡,中位生存时间为48.6(26.6~70.6)个月,确诊时有无远处转移和能否进行根治性手术切除对患者的总生存影响差异有统计学意义(P<0.05),但患者的年龄、性别、原发肿瘤部位、确诊时血清神经元特异性烯醇化酶(neuron specific enolase,NSE)水平以及Ki-67指数对患者的总生存影响无显著差异(P>0.05)。多因素分析显示,确诊时有远处转移是影响患者生存的独立危险因素(P=0.01;HR=7.33,95%CI:1.56~34.10)。结论:GEP NET G3的确诊依赖于组织病理学和免疫组织化学检测,易发生远处转移是其临床特点和主要的预后影响因素。
