Advances in the Diagnosis and Treatment of Appendiceal Mucinous Neoplasms

Primary appendiceal neoplasms represent a relatively low percentage of all gastrointestinal cancers. A subset of these neoplasms, those of epithelial origin, are characterised by the production of a considerable amount of mucus, which is referred to as appendiceal mucinous neoplasms (AMN). Appendiceal mucinous neoplasms (AMN) have a low incidence, are easily misdiagnosed, depend on postoperative examination for confirmation of the diagnosis, are prone to form a “diagnosis”, and have a high incidence of the disease. Furthermore, they are prone to form peritoneal pseudomyxoma peritonei (PMP), are controversial in surgical decision-making, are prone to recurring after surgery alone, and are tricky to manage clinically. In this paper, we review the pathological characteristics, diagnosis and treatment of appendiceal mucinous tumours in the light of recent literature reports, with a view to providing certain references for the clinical diagnosis and treatment of this disease. .

Mixed Neuroendocrine-Non-Neuroendocrine Neoplasm (MiNEN) of the Colon: A Case Report

Here we report a rare case of mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs). A 51-year-old lady with no known family history of malignancies developed this rare type of malignancy without any active gastrointestinal symptoms. However, during a routine health check, physicians noticed a raised Carcinoembryonic Antigen (CEA) level and the patient subsequently was referred to the surgical department for further management. A colonoscopy and CECT abdomen were done and she was electively admitted for a left hemicolectomy operation for a splenic flexure tumour. The histopathological report revealed the tumour is a case of mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) [moderately differentiated adenocarcinoma and neuroendocrine tumour grade 3]. TNM (8th edition, 2016): pT3, pN2 (20/21), pMX. Both resection margins were clear from malignant cells. Colorectal MiNENs, constitute a rare group of gastrointestinal tumours composed of both neuroendocrine and non-neuroendocrine components. Given their non-diagnostic macroscopic features, specific histological features and lack of disease awareness which are responsible for the underestimated incidence and conflicting data. In this case, a multidisciplinary team approach is important in managing patients with this malignancy to achieve the best outcome.

有氧运动改善化疗期间乳腺癌患者体质及生活质量研究:一项随机对照试验

背景蒽环类药物是乳腺癌基础化疗药物之一,但化疗常伴随着体质改变如体脂增加和心肺功能下降,胃肠道反应和骨髓抑制等毒副作用,影响患者的生活质量。目前关于运动改善以上毒副作用的研究结果不一致,有待进一步研究。临床上,应用运动处方改善乳腺癌患者以上化疗毒副作用的效果和安全性需要进一步探究。目的探究有氧运动改善蒽环类药物化疗期间乳腺癌患者体质和生活质量的效果及有氧运动的安全性。方法本研究是一项随机对照试验,纳入2022年3月—2023年1月在北京市朝阳区三环肿瘤医院接受蒽环类药物化疗方案的44例成年女性乳腺癌患者为研究对象,随机分为运动组(23例)与对照组(21例),对照组患者在化疗结束后提供个性化运动指导。运动组患者在化疗住院期间在康复师监督下进行锻炼,在家时通过患者自我监督和试验人员远程监督进行个性化运动干预。在化疗前后收集主要结局指标,包括体质和生活质量,记录胃肠道反应、骨髓抑制的发生次数、严重程度及运动相关不良事件。以化疗前数据为协变量,采用协方差分析比较两组体质情况和生活质量情况。结果本研究干预、随访过程中共流失4例,最终纳入40例患者(运动组21例,对照组19例)。运动干预期间未观察到严重不良事件。运动干预期间,患者平均依从性为81.8%;每次运动时长的平均依从性为91.9%;运动强度平均依从性为92.5%。化疗后,运动组体脂重、体脂百分比、内脏脂肪面积、腰围、腰臀比低于对照组,惯用手握力、相对峰值摄氧量(VO_(2)peak)高于对照组(P<0.05)。化疗后运动组患者功能性障碍发生率(7/20)低于对照组(12/16)(χ^(2)=5.707,P=0.017)。化疗后运动组生理状况、情感状况、附加得分低于对照组,功能状况得分高于对照组(P<0.05)。化疗后对照组生理状况分数(P<0.001)、运动组功能状况分数(P=0.017)高于化疗前。对照组和运动组患者分别共接受了84例次和94例次蒽环类药物化疗,对照组分别发生了84例次胃肠道反应和71次骨髓抑制,运动组分别发生了54例次胃肠道反应和45例次骨髓抑制,两组患者胃肠道反应和骨髓抑制发生情况比较,差异有统计学意义(P<0.05)。结论在蒽环类药物化疗期间进行有氧运动可以改善乳腺癌患者的体质和生活质量,且有监督的有氧运动是安全的。

硒及硒纳米颗粒生物学机制与口腔常见疾病的关系

背景:硒作为人体所需的微量非金属元素具有抗炎、抗氧化、抗菌、免疫调节和抗肿瘤等生物活性,已经在生物医学领域得到广泛应用。目的:总结硒及其纳米颗粒在抗炎、抗氧化、抗菌、免疫调节和抗肿瘤方面的功能和作用机制,以及在口腔医学领域的研究进展。方法:以“selenium,selenium nanoparticles,oral neoplasms,caries,dental pupal disease,periapical disease,dental implant”为英文检索词,以“硒,硒纳米颗粒,口腔癌,龋病,牙髓病,根尖周病,口腔种植”为中文检索词,分别在PubMed、中国知网、万方数据库进行文献检索,检索文献时限为2000年1月至2024年2月。经过对文献的深入分析和仔细阅读,最后选择74篇文献进行全面综述。结果与结论:(1)硒的生物学特性主要是通过合成硒蛋白,以抗氧化机制为核心,调节各种细胞因子和介导与之相关的信号通路,进而产生抗菌、免疫调节、抗肿瘤等作用;(2)硒除了在口腔癌、龋病、牙髓根尖周病等方面可用于早期诊断、治疗和预后判断外,更多的是可作为新型口腔材料在口腔领域应用,例如:用于开发新型牙科封闭剂或根管系统消毒剂,又或在优化口腔种植材料性能等方面有所应用;(3)生物安全性是影响硒在生物医药领域应用的最重要因素之一,选择合适的剂量和形式才能使其发挥出良好作用。硒在口腔材料学方面展现出了巨大的应用潜力和广阔的发展空间。

Current perspectives on pancreatic serous cystic neoplasms:Diagnosis, management and beyond

Pancreatic cystic neoplasms have been increasingly recognized recently. Comprising about 16% of all resected pancreatic cystic neoplasms, serous cystic neoplasms are uncommon benign lesions that are usually asymptomatic and found incidentally. Despite overall low risk of malignancy, these pancreatic cysts still generate anxiety, leading to intensive medical investigations with considerable financial cost to health care systems. This review discusses the general background of serous cystic neoplasms, including epidemiology and clinical characteristics, and provides an updated overview of diagnostic approaches based on clinical features, relevant imaging studies and new findings that are being discovered pertaining to diagnostic evaluation. We also concisely discuss and propose management strategies for better quality of life.

Simultaneous intraductal papillary neoplasms of the bile duct and pancreas treated with chemoradiotherapy

Some authors have suggested that intraductal papillary mucinous neoplasms of the bile duct(IPMN-B) could be the the biliary counterpart of IPMN of the pancreas(IPMN-P) since they share several clinical-pathological features.These include prominent intraductal papil-lary proliferation pattern,a gastrointestinal phenotype,frequent mucin hyper-secretion and progression to mu-cinous carcinoma.To date there are just four reported cases of patients with synchronous IPMN-B and IPMN-P all of which were treated surgically.We hereby report the case of a 76-year-old woman who was incidentally diagnosed with both an asymptomatic 3 cm bulky uid lesion obstructing the bile duct lumen,diagnosed as a malignant IPMN-B,and synchronous multiple pancreatic cystic lesions(10-13 mm) communicating with an irreg-ular Wirsung,diagnosed as branch duct IPMN-P.Since surgery was ruled-out because of the patient's age and preferences,she underwent a conservative manage-ment regimen comprising both chemotherapy and radio-therapy.This was effective in decreasing the mass size and in resolving subsequent jaundice.This is also the f irst reported case of IPMN-B successfully treated with chemoradiotherapy.Clinicians should consider medical treatment as an option in this clinical scenario,in pa-tients who may be unf it for surgery.

Clinicopathologic characteristics and prognosis of gastroenteropancreatic neuroendocrine neoplasms: a multicenter study in South China

Background: Gastroenteropancreatic neuroendocrine neoplasms(GEP-NENs) are a heterogeneous group of rare tumors. Many issues in terms of epidemiologic features, pathogenesis, and treatment of GEP-NENs are still under discussion. Our study aimed to analyze the clinicopathologic characteristics and prognosis of Chinese patients with GEP-NENs.Methods: Complete clinicopathologic data and survival information of 1183 patients with GEP-NENs treated between 2005 and 2015 were collected from five medical centers in Guangdong Province, China. Patient survival was estimated using the Kaplan–Meier method and analyzed using the log-rank test; prognostic factors were analyzed using the Cox proportional hazards model.Results: The most common tumor location was the rectum(37.4%), followed by the pancreas(28.1%), stomach(20.7%), small intestine(7.2%), appendix(3.4%), and colon(3.3%). After initial definitive diagnosis, 1016(85.9%) patients underwent surgery. The 1-, 3-, and 5-year overall survival(OS) rates for the entire cohort were 87.9%, 78.5%, and 72.8%, respectively. The 3-year OS rates of patients with G1, G2, and G3 tumors were 93.1%, 82.7%, and 43.1%, respectively(P < 0.001). The 3-year OS rates of patients with stage I, II, III, and IV tumors were 96.0%, 87.3%, 64.0%, and 46.8%, respectively(P < 0.001). Patients with distant metastasis who underwent palliative surgery had a longer survival than those who did not(P = 0.003). Similar survival benefits of palliative surgery were observed in patients with neuroendocrine tumor(P y, M category, and sur= 0.031) or neuroendocrine carcinoma(P gery were found to be independent prog= 0.046). In multivariate analysis, age, grade, N categornostic factors.Conclusions: Patients with GEP-NENs who are women, younger than 50 years old, have smaller tumor size, have lower tumor grade, have lower T/N/M category, and who undergo surgery can have potentially longer survival time. Our data showed that surgery can improve the prognosis of GEP-NEN patients with distant metastasis. However, randomized controlled trials need to be conducted to establish the optimal criteria for selecting patients to undergo surgery.

Helicobacter pylori-related chronic gastritis as a risk factor for colonic neoplasms

To summarize the current views and insights on associations between Helicobacter pylori(H. pylori)-related chronic gastritis and colorectal neoplasm, we reviewed recent studies to clarify whether H. pylori infection/H. pylori-related chronic gastritis is associated with an elevated risk of colorectal neoplasm. Recent studies based on large databases with careful control for confounding variables have clearly demonstrated an increased risk of colorectal neoplasm associated with H. pylori infection. The correlation between H. pylori-related chronic atrophic gastritis(CAG) and colorectal neoplasm has only been examined in a limited number of studies. A recent large study using a national histopathological database, and our study based on the stage of H. pylori-related chronic gastritis as determined by serum levels of H. pylori antibody titer and pepsinogen, indicatedthat H. pylori-related CAG confers an increased risk of colorectal neoplasm, and more extensive atrophic gastritis will probably be associated with even higher risk of neoplasm. In addition, our study suggested that the activity of H. pylori-related chronic gastritis is correlated with colorectal neoplasm risk. H. pylori-related chronic gastritis could be involved in an increased risk of colorectal neoplasm that appears to be enhanced by the progression of gastric atrophy and the presence of active inflammation.

Intraductal papillary neoplasm of the bile ducts: A case report and literature review

Intraductal papillary neoplasm of the bile duct(IPNB) is a rare bile duct neoplasm mostly found in far eastern nations where hepatolithiasis and clonorchiasis infections are endemic. In western countries,it is very rare and the etiology is unknown. In this article,we report the first IPNB patient we encountered in our clinic and a literature review. The patient is a 38-yearold female with a history of choledocholithiasis who presented with obstructive jaundice. She was found to have a papillary mass at the junction of the right hepatic duct and common hepatic duct with six masses in the liver parenchyma. The immunophenotypic and histologic features of the tumor are consistent with IPNB,gastric subtype. The patient had a partial hepatectomy and has been receiving palliative chemotherapy. In a search of Pub Med database,we collected 354 IPNB patients reported in 22 articles. In these patients,52.8% were from Japan and 27.7% were from western countries including the United States(11.0%). The age of the patients ranged from 35 to 80 years old with an average of 64.6. Male/female ratio was 1.5. Macroscopically,57.5% of the tumors were in the left lobe and 29.5% were in the right lobe. The average size of the tumor were 4.2 cm at the time of diagnosis. Histologically,pancreato-biliary subtype accounted for 41.8%,intestinal 28.0%,gastric 13.5% and oncocytic 16%. An invasive component is most often present in the pancreato-biliary and gastric subtypes. Despite recent advanced technologies,diagnosis of IPNB is still challenging,especially in western countries due to its rarity. Defined clinicopathologic features are in demand for the accurate diagnosis and proper treatment.

Risk factors for bleeding after endoscopic submucosal dissection of colorectal neoplasms

AIM: To investigate the risk factors for delayed bleeding following endoscopic submucosal dissection (ESD) treatment for colorectal neoplasms.

Pancreatic neuroendocrine tumor and solid-pseudopapillary neoplasm: Key immunohistochemical profiles for differential diagnosis

AIM To reveal better diagnostic markers for differentiating neuroendocrine tumor(NET) from solid-pseudopapillary neoplasm(SPN), focusing primarily on immunohistochemical analysis.METHODS We reviewed 30 pancreatic surgical specimens of NET(24 cases) and SPN(6 cases). We carried out comprehensive immunohistochemical profiling using 9 markers: Synaptophysin, chromogranin A, pancytokeratin, E-cadherin, progesterone receptor,vimentin, α-1-antitrypsin, CD10, and β-catenin.RESULTS E-cadherin staining in NETs, and nuclear labeling of β-catenin in SPNs were the most sensitive and specific markers. Dot-like staining of chromogranin A might indicate the possibility of SPNs rather than NETs. The other six markers were not useful because their expression overlapped widely between NETs and SPNs. Moreover, two cases that had been initially diagnosed as NETs on the basis of their morphological features, demonstrated SPN-like immunohistochemical profiles. Careful diagnosis is crucial as we actually found two confusing cases showing disagreement between the tumor morphology and immunohistochemical profiles.CONCLUSION E-cadherin, chromogranin A, and β-catenin were the most useful markers which should be employed for differentiating between NET and SPN.

Intraductal papillary neoplasm of the bile duct

Intraductal papillary neoplasm of the bile duct(IPNB)is a variant of bile duct carcinoma that is characterized by intraductal growth and better outcomes compared with common cholangiocarcinoma.IPNBs are mainly found in patients from Far Eastern areas,where hepatolithiasis and clonorchiasis are endemic.According to the immunohistochemical profiles of the mucin core proteins,IPNBs are classified into four types:pancreaticobiliary,intestinal,gastric,and oncocytic.Approximately 40%-80%of IPNBs contain a component of invasive carcinoma or tubular or mucinous adenocarcinoma,suggesting that IPNB is a disease with high potential for malignancy.It is difficult to make an accurate preoperative diagnosis because of IPNB’s low incidence and the lack of specificity in its clinical manifestation.The most common abnormal preoperative imaging findings of IPNB are intraductal masses and the involvement of bile duct dilation.Simultaneous proximal and distal bile duct dilation can be detected in some cases,which has diagnostic significance.Cholangiography and cholangioscopy are needed to confirm the pathology and demonstrate the extent of the lesions.However,pathologic diagnosis by biopsy cannot reflect the actual stage in many cases because different foci may be of different stages and because mixed pathologic findings may exist in the same lesion.Surgical resection is the major treatment.Systematic cholangioscopy with staged biopsies and frozen sections is recommended during resection to ensure that no minor tumors are left and that curative resection is achieved.Staging,histologic subtype,curative resection and lymph node metastasis are factors affecting long-term survival.

Diagnostic accuracy of endoscopic ultrasonography for rectal neuroendocrine neoplasms

AIM: To investigate the diagnostic accuracy of endoscopic ultrasonography (EUS) for rectal neuroendocrine neoplasms (NENs) and the differential diagnosis of rectal NENs from other subepithelial lesions (SELs).

Imaging pitfalls of pancreatic serous cystic neoplasm and its potential mimickers

The aim of this article is to clarify diagnostic pitfalls of pancreatic serous cystic neoplasm(SCN) that may result in erroneous characterization. Usual and unusual imaging findings of SCN as well as potential SCN mimickers are presented. The diagnostic key of SCN is to look for a cluster of microcysts(honeycomb pattern), which may not be always found in the center. Fibrosis in SCN may be mistaken for a mural nodule of intraductal papillary mucinous neoplasm(IPMN). The absence of cyst wall enhancement may be helpful to distinguish SCN from mucinous cystic neoplasm. However, oligocystic SCN and branch duct type IPMN may morphologically overlap. In addition, solid serous adenoma, an extremely rare variant of SCN, is difficult to distinguish from neuroendocrine tumor.

Efficacy and safety of 0.4 percent sodium hyaluronate for endoscopic submucosal dissection of gastric neoplasms

AIM:To evaluate the efficacy and safety of sodium hyaluronate solution(SH) in endoscopic submucosal dissection(ESD) of gastric neoplasms.METHODS:A prospective multicenter randomized,double blind,controlled trial was designed and utilized in this study.A total of 76 patients with 5-20 mm sized gastric neoplasms were enrolled at three academic hospitals in South Korea from June 2011 to October 2011.Patients were randomly assigned to the 0.4% sodium hyaluronate or control groups.All lesions underwent endoscopic ESD.ESD was performed with 0.4%SH and normal saline(NS) solution for submucosal injection.Efficacy was assessed using en bloc resection and the number of additional injections.Secondary evaluation variables were the volume of injection material,steepness of mucosal elevation,bleeding rate,procedural time and operator satisfaction.Finally,the safety was assessed by analyzing adverse events during the study.RESULTS:The usefulness rate in the 0.4%SH group and the controlled group had statistically significant difference under intention to treat(ITT) analysis(90.91% vs 61.11% P = 0.0041).Under per protocol(PP),the usefulness rate is statistically significant different(93.10% vs 61.76%,P = 0.0036).The difference in volume of the solution injected between 0.4%SH group and the controlled group and NS group was also statistically significant under intention to treat and per protocol analysis(ITT:0.03 ± 0.02 mL vs 0.06 ± 0.03 mL,P = 0.0003,PP:0.03 ± 0.02 mL vs 0.06 ± 0.03 mL,P = 0.0004).Satisfaction above the grade good was significantly higher in the SH group under intention to treat and per protocol analysis(ITT:90.91% vs 61.11%,P = 0.0041,PP = 93.11% vs 61.77%,P = 0.0022).Adverse events above grade 3 were not noticed in either group.All adverse events were treated and were judged as not associated with the submucosal injection solutions.CONCLUSION:0.4%SH solution is a safe and effective agent that doesn't cause any significant adverse events and is useful for submucosal injection during ESD.

Synchronous pancreatic solid pseudopapillary neoplasm and intraductal papillary mucinous neoplasm

Solid pseudopapillary neoplasm (SPN) is a rare and low-grade malignant pancreatic neoplasm composed of poorly cohesive monomorphic neoplastic cells forming solid and pseudopapillary structures with frequent hemorrhagic-cystic degeneration. Intraductal papillary mucinous neoplasm (IPMN) is a pancreatic exocrine tumor composed of intraductal papillary growth of mucin containing neoplastic cells in the main pancreatic duct or its major branches. In the case presented here, a 53-year-old, Japanese man was found to have multiple cystic lesions and dilatation of the main pancreatic duct in the neck of the pancreas. Histological examination revealed a main-duct and branch-duct type IPMN, of the gastric-type, involving the neck of the pancreas, associated with a 0.5 cm SPN in the caudal side of the IPMN. We diagnosed this case as synchronous SPN and IPMN. As far as we know, only one other case of synchronous SPN and IPMN has been reported. Both the present case and the previously reported case showed abnormal nuclear expression of β-catenin in SPN, whereas IPMN showed no abnormal nuclear expression. These results suggest that β-catenin abnormality is not a common pathogenetic factor of synchronous SPN and IPMN.

International guidelines for the management of pancreatic intraductal papillary mucinous neoplasms

The management of intraductal papillary mucinous neoplasms(IPMN) is presently evolving as a result of the improved understanding of the natural history and biological behavior of the different pancreatic cystic neoplasms; and better preoperative diagnosis of these neoplasms due to advancement in preoperative diagnostic tools. International consensus guidelines for the management of IPMN were first formulated in 2006 and subsequently revised in 2012. Both these guidelines were constructed based on expert opinion and not on robust clinical data. The main limitation of the original Sendai guidelines was that it had a low positive predictive value resulting in many benign neoplasms being resected. Hence,these guidelines were revised in 2012. However,although the updated guidelines resulted in an improvement in the positive predictive value over the Sendai Guidelines,the results of several studies validating these guidelines demonstrated that its positive predictive value remained low. Furthermore,although both guidelines were associated with high negative predictive values,several investigators have demonstrated that some malignant IPMNs may be missed. Finally,it is imperative to emphasize that major considerations when managing a patient with IPMN including the patient's surgical risk,life-expectancy and even cost of investigations are not taken into account in current guidelines. The management of a patient with IPMN should be individualized and tailored according to a patient's risk benefit profile for resection vs surveillance.

Endoscopic ultrasound guided radiofrequency ablation,for pancreatic cystic neoplasms and neuroendocrine tumors

AIM: To outline the feasibility, safety, adverse events and early results of endoscopic ultrasound(EUS)-radiofrequency ablation(RFA) in pancreatic neoplasms using a novel probe. METHODS: This is a multi-center, pilot safety feasibility study. The intervention described was radiofrequency ablation(RF) which was applied with an innovative monopolar RF probe(1.2 mm Habib EUS-RFA catheter) placed through a 19 or 22 gauge fine needle aspiration(FNA) needle once FNA was performed in patients with a tumor in the head of the pancreas. The HabibTM EUSRFA is a 1 Fr wire(0.33 mm, 0.013") with a working length of 190 cm, which can be inserted through the biopsy channel of an echoendoscope. RF power is applied to the electrode at the end of the wire to coagulate tissue in the liver and pancreas.RESULTS: Eight patients [median age of 65(range 27-82) years; 7 female and 1 male] were recruited in a prospective multicenter trial. Six had a pancreatic cysticneoplasm(four a mucinous cyst, one had intraductal papillary mucinous neoplasm and one a microcystic adenoma) and two had a neuroendocrine tumors(NET) in the head of pancreas. The mean size of the cystic neoplasm and NET were 36.5 mm(SD ± 17.9 mm) and 27.5 mm(SD ± 17.7 mm) respectively. The EUSRFA was successfully completed in all cases. Among the 6 patients with a cystic neoplasm, post procedure imaging in 3-6 mo showed complete resolution of the cysts in 2 cases, whilst in three more there was a 48.4% reduction [mean pre RF 38.8 mm(SD ± 21.7 mm) vs mean post RF 20 mm(SD ± 17.1 mm)] in size. In regards to the NET patients, there was a change in vascularity and central necrosis after EUS-RFA. No major complications were observed within 48 h of the procedure. Two patients had mild abdominal pain that resolved within 3 d. CONCLUSION: EUS-RFA of pancreatic neoplasms with a novel monopolar RF probe was well tolerated in all cases. Our preliminary data suggest that the procedure is straightforward and safe. The response ranged from complete resolution to a 50% reduction in size.

Retrospective study on mixed neuroendocrine non-neuroendocrine neoplasms from five European centres

BACKGROUND Mixed neuroendocrine non-neuroendocrine neoplasm(MiNEN)is a rare diagnosis,mainly encountered in the gastro-entero-pancreatic tract.There is limited knowledge of its epidemiology,prognosis and biology,and the best management for affected patients is still to be defined.AIM To investigate clinical-pathological characteristics,treatment modalities and survival outcomes of a retrospective cohort of patients with a diagnosis of MiNEN.METHODS Consecutive patients with a histologically proven diagnosis of MiNEN were identified at 5 European centres.Patient data were retrospectively collected from medical records.Pathological samples were reviewed to ascertain compliance with the 2017 World Health Organisation definition of MiNEN.Tumour responses to systemic treatment were assessed according to the Response Evaluation Criteria in Solid Tumours 1.1.Kaplan-Meier analysis was applied to estimate survival outcomes.Associations between clinical-pathological characteristics and survival outcomes were explored using Log-rank test for equality of survivors functions(univariate)and Cox-regression analysis(multivariable).RESULTS Sixty-nine consecutive patients identified;Median age at diagnosis:64 years.Males:63.8%.Localised disease(curable):53.6%.Commonest sites of origin:colon-rectum(43.5%)and oesophagus/oesophagogastric junction(15.9%).The neuroendocrine component was;predominant in 58.6%,poorly differentiated in 86.3%,and large cell in 81.25%,of cases analysed.Most distant metastases analysed(73.4%)were occupied only by a poorly differentiated neuroendocrine component.Ninety-four percent of patients with localised disease underwent curative surgery;53%also received perioperative treatment,most often in line with protocols for adenocarcinomas from the same sites of origin.Chemotherapy was offered to most patients(68.1%)with advanced disease,and followed protocols for pure neuroendocrine carcinomas or adenocarcinomas in equal proportion.In localised cases,median recurrence free survival(RFS);14.0 months(95%CI:9.2-24.4),and median overall survival(OS):28.6 months(95%CI:18.3-41.1).On univariate analysis,receipt of perioperative treatment(vs surgery alone)did not improve RFS(P=0.375),or OS(P=0.240).In advanced cases,median progression free survival(PFS);5.6 months(95%CI:4.4-7.4),and median OS;9.0 months(95%CI:5.2-13.4).On univariate analysis,receipt of palliative active treatment(vs best supportive care)prolonged PFS and OS(both,P<0.001).CONCLUSION MiNEN is most commonly driven by a poorly differentiated neuroendocrine component,and has poor prognosis.Advances in its biological understanding are needed to identify effective treatments and improve patient outcomes.