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. .

Synchronous multiple primary malignant neoplasms in breast,kidney,and bilateral thyroid:A case report

BACKGROUND Multiple primary malignant neoplasms(MPMNs)are rare,while synchronous MPMNs(SMPMNs)are even less common.Owing to the progression of medical technology and the extension of life expectancy,its incidence is gradually increasing.CASE SUMMARY Although reports of breast and thyroid dual cancers are common,cases of an additional diagnosis of kidney primary cancer within the same individual are rare.CONCLUSION We present a case of simultaneous MPMN of three endocrine organs,reviewing the relevant literature to enhance our understanding of SMPMNs while emphasizing the increasingly important need for accurate diagnosis and multidisciplinary management whenever this challenging situation arises.

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.

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.

Differences between main-duct and branch-duct intraductal papillary mucinous neoplasms of the pancreas

In the last decade,intraductal papillary mucinous neoplasms(IPMNs) have become commonly diagnosed.From a morphological standpoint,they are classified in main-duct IPMNs(MD-IPMNs) and branch-duct IPMNs(BD-IPMNs),depending on the type of involvement of the pancreatic ductal system by the neoplasm.Despite the fact that our understanding of their natural history is still incomplete,recent data indicate that MD-IPMNs and BD-IPMNs show significant differences in terms of biological behaviour with MD-IPMNs at higher risk of malignant degeneration.In the present paper,clinical and epidemiological characteristics,rates of malignancy and the natural history of MD-IPMNs and BD-IPMNs are analyzed.The profile of IPMNs involving both the main pancreatic duct and its side branches(combined-IPMNs) are also discussed.Finally,general recommendations for management based on these differences are given.

Gastric neuroendocrine neoplasms type 1: A systematic review and meta-analysis

BACKGROUND To date, the histopathological parameters predicting the risk of lymph node (LN) metastases and local recurrence, associated mortality and appropriateness of endoscopic or surgical resection in patients with gastric neuroendocrine neoplasms type 1 (GNENs1) have not been fully elucidated. AIM To determine the rate of LN metastases and its impact in survival in patients with GNEN1 in relation to certain clinico-pathological parameters. METHODS The PubMed, EMBASE, Cochrane Library, Web of Science and Scopus databases were searched through January 2019. The quality of the included studies and risk of bias were assessed using the Newcastle-Ottawa Scale (NOS) in accordance with the Cochrane guidelines. A random effects model and pooled odds ratios (OR) with 95%CI were applied for the quantitative meta-analysis. RESULTS We screened 2933 articles. Thirteen studies with 769 unique patients with GNEN1 were included. Overall, the rate of metastasis to locoregional LNs was 3.3%(25/769). The rate of LN metastases with a cut-off size of 10 mm was 15.3% for lesions > 10 mm (vs 0.8% for lesions < 10 mm) with a random-effects OR of 10.5 (95%CI: 1.4 -80.8;heterogeneity: P = 0.126;I2 = 47.5%). Invasion of the muscularis propria was identified as a predictor for LN metastases (OR: 17.2;95%CI: 1.8-161.1;heterogeneity: P = 0.165;I2 = 44.5%), whereas grade was not clearly associated with LN metastases (OR: 2;95%CI: 0.3-11.6;heterogeneity: P = 0.304;I2 = 17.4%). With regard to GNEN1 local recurrence, scarce data were available. The 5-year disease-specific survival for patients with and without LN metastases was 100% in most available studies irrespective of the type of intervention. Surgical resection was linked to a lower risk of recurrence (OR: 0.3;95%CI: 0.1-1.1;heterogeneity: P = 0.173;I2 = 31.9%). The reported complication rates of endoscopic and surgical intervention were 0.6 and 3.8%, respectively. CONCLUSION This meta-analysis confirms that tumor size ≥ 10 mm and invasion of the muscularis propria are linked to a higher risk of LN metastases in patients with GNEN1. Overall, the metastatic propensity of GNEN1 is low with favorable 5- year disease-specific survival rates reported;hence, no clear evidence of the prognostic value of LN positivity is available. Additionally, there is a lack of evidence supporting the prediction of local recurrence in GNEN1, even if surgery was more often a definitive treatment.

Update on surgical treatment of pancreatic neuroendocrine neoplasms

Pancreatic neuroendocrine neoplasms(PNENs) are rare and account for only 2%-4% of all pancreatic neoplasms. All PNENs are potential(neurendocrine tumors PNETs) or overt(neuroendocrine carcinomas PNECs) malignant,but a subset of PNETs is low-risk. Even in case of low-risk PNETs surgical resection is frequently required to treat hormone-related symptoms and to obtain an appropriate pathological diagnosis. Low-risk PNETs in the body and the tail are ideal for minimallyinvasive approaches which should be tailored to the individual patient. Generally,surgeons must aim for parenchyma sparing in these cases. In high-risk and malignant PNENs,indications for tumor resection are much wider than for pancreatic adenocarcinoma,in many cases due to the relatively benign tumor biology. Thus,patients with locally advanced and metastatic PNETs may benefit from extensive resection. In experienced hands,even multi-organ resections are accomplished with acceptable perioperative morbidity and mortality rates and are associated with excellent long term survival. However,poorly differentiated neoplasms with high proliferation rates are associated with a dismal prognosis and may frequently only be treated with chemotherapy. The evidence on surgical treatment of PNENs stems from reviews of mostly singlecenter series and some analyses of nation-wide tumor registries. No randomized trial has been performed to compare surgical and non-surgical therapies in potentially resectable PNEN. Though such a trial would principally be desirable,ethical considerations and the heterogeneity of PNENs preclude realization of such a study. In the current review,we summarize recent advances in the surgical treatment of PNENs.

Electrophysiological neuromonitoring of the laryngeal nerves in thyroid and parathyroid surgery: A review

Recurrent laryngeal nerve(RLN) injury is one of the most common complications of thyroid surgery. Injury to the external branch of the superior laryngeal nerve is less obvious and affects the voice variably; however, it can be of great significance to professional voice users. Recent literature has led to an increase in the use of neuromonitoring as an adjunct to visual nerve identification during thyroid surgery. In our review of the literature, we discuss the application, efficacy and safety of neuromonitoring in thyroid surgery. Although intraoperative neuromonitoring(IONM) contributes to the prevention of laryngeal nerves injury, there was no significant difference in the incidence of RLN injury in thyroid surgery when IONM was used compared with visual identification alone. IONM use is recommended in high risk patients; however, there are no clear identification criteria for what constitutes "high risk". There is no clear evidence that IONM decreases the risk of laryngeal nerve injury in thyroid surgery. However, continuous IONM provides a promising tool that can prevent imminent nerve traction injury by detecting decreased amplitude combined with increased latency.

Extrathyroidal Implantation of Thyroid Hyperplastic/neoplastic Cells after Endoscopic Thyroid Surgery

Objective To report a case of the implantation of thyroid hyperplastic or neoplastic tissue after endoscopic thyroidectomy and discuss this complication in aspects of prevalence, pathogenesis, protection, and therapies. Methods A systematic search of literature from the PubMed database was conducted for identifying eligible studies on implantation of thyroid hyperplastic or neoplastic cells after endoscopic thyroid surgery. Results Overall, 5 reported cases on patients suffering from endoscopic thyroid surgery with implantation of thyroid hyperplastic or neoplastic cells were included in the systematic review. Conclusions Unskilled surgeons, rough intraoperative surgical treatment, scarification or rupture of tumor, contamination of instruments, chimney effect, aerosolization of tumor cells may be associated with the implantation after endoscopic thyroidectomy. To minimize the risk of such complication, we should be more meticulous and strict the endoscopic surgery indications.

Endoscopic submucosal dissection for colorectal neoplasms

Although endoscopic submucosal dissection(ESD) gains acceptance as one of the standard treatments for esophageal and stomach neoplasms in Japan,it is still in the developing stage for colorectal neoplasms.In terms of indications,little likelihood of nodal metastasis and technical resectability are principally considered.Some of intramucosal neoplasms,carcinomas with minute submucosal invasion,and carcinoid tumors,which are technically unresectable by conventional endoscopic treatments,may become good candidates for ESD,considering substantial risks and obtained benefits.ESD as a staging measure to obtain histological information of the invasion depth and lymphovascular infiltration is acceptable because preoperative prediction is difficult in some cases.In terms of techniques,advantages of ESD in comparison with other endoscopic treatments are to be controllable in size and shape,and to be resectable even in large and fibrotic neoplasms.The disadvantages may be longer procedure time,heavier bleeding,and higher possibility of perforation.However,owing to refinement of the techniques,invention of devices,and the learning curve,acceptable technical safety has been achieved.Colorectal ESD is very promising and become one of the standard treatments for colorectal neoplasms in the near future.

Correlation analysis of serum thyroglobulin,thyroid-stimulating hormone levels,and thyroid-cancer risk in thyroid nodule surgery

BACKGROUND Thyroglobulin(Tg)is one of the markers of thyroid cancer,and its concentration may be elevated in patients with malignant thyroid tumors.Thyroid-stimulating hormone(TSH)is secreted by the pituitary gland,which has a significant impact on thyroid gland function.Excessively high or low TSH levels may be associated with an increased risk of thyroid cancer.Thus,in-depth studies on the association of serum Tg and TSH levels with thyroid cancer risk in patients with thyroid nodules are warranted.This can help determine whether Tg and TSH levels can predict the degree of malignancy of thyroid nodules,which can in turn guide doctors in making accurate diagnoses and treatment decisions.Furthermore,such studies can provide more accurate diagnostic methods for thyroid nodules and help patients become aware of the presence of thyroid cancer as early as possible,thereby improving the success rate of treatment and prognosis.AIM To investigate the association of serum Tg and TSH levels with the risk of thyroid cancer in patients undergoing thyroid nodule surgery.METHODS The clinical data and laboratory examination results of 130 patients who underwent thyroid nodule surgery were retrospectively analyzed.Furthermore,their preoperative serum Tg and TSH levels were recorded.Histopathological examination conducted during follow-up revealed the presence of thyroid cancer.Correlation analysis were used to analyze the association of Tg and TSH levels with the risk of thyroid cancer.RESULTS Of the 130 patients,60 were diagnosed with thyroid cancer.Statistical analysis revealed a significant positive correlation between serum Tg levels and the risk of thyroid cancer(P<0.05).This suggests that high serum Tg levels are associated with an increased risk of thyroid cancer.However,no significant correlation was observed between serum TSH levels and the risk of thyroid cancer(P>0.05).CONCLUSION In patients who underwent thyroid nodule surgery,serum Tg levels exhibited a significant correlation with the risk of thyroid cancer but serum TSH levels did not.These findings suggest that serum Tg can serve as an important biomarker for assessing the risk of thyroid cancer in these patients.

The effect of intravenous high-dose glucocorticoids and orbital decompression surgery on sight-threatening thyroid-associated ophthalmopathy

AIM: To report the effects of intravenous high-dose glucocorticoids(iv GC) and orbital decompression(OD) surgery for treatment of sight-threatening thyroid-associated ophthalmopathy(TAO).METHODS: A retrospective review of medical records from patients with sight-threatening TAO [definite or highly suspected dysthyroid optic neuropathy(DON)] treated with iv GC(60 cases) and OD(25 cases) was conducted at the Zhongshan Ophthalmic Center between January 2001 and January 2009. Patients were initially treated with iv GC(iv GC group). If no significant improvement in visual function was obtained, they then received OD surgery(OD group). The pre-versus post-treatment efficacies of either iv GC or OD in these patients were assessed using several indices, including visual acuity, intraocular pressure, ocular alignment, ocular motility, and exophthalmos. RESULTS: Nighty-one eyes had definite DON while 79 were considered to have highly suspected DON. In the iv GC group, 51 individuals(85.0%) eventually demonstrated normal vision, while 10 patients(16.7%) demonstrated a reduction in deviation(P<0.01), and 35 cases(58.3%) showed slight improvements in ocular motility(P<0.01). In OD group, visual acuity improved in 24 cases(96.0%, P<0.01) and all patients showed varying reductions of exophthalmos(mean: 4.35±1.13 mm, P<0.01). Eight cases(32.0%) experienced an 8-15 PD reduction of deviation and ocular motility improved in 12 cases(48.0%), while 3 patients(12.0%) developed new-onset strabismus with diplopia post-surgically(P<0.01). Patients were followed up at an average of 1.55±1.07 y. CONCLUSION: Both iv GC and OD show good therapeutic efficacy in the treatment of sight-threatening TAO. Thepresence of extremely poor eyesight(≥0.5 log MAR) was corrected in some patients with iv GC alone, thus sparing these patients from subsequent OD surgery. In patients who were refractory to steroids, subsequent OD surgery often provided satisfactory outcomes, however, new-onset strabismus with diplopia was observed in 12.0% of these cases.

Therapeutic strategies for gastroenteropancreatic neuroendocrine neoplasms: State-of-the-art and future perspectives

Although gastroenteropancreatic neuroendocrine neoplasms(GEP-NENs)have always been considered rare tumors,their incidence has risen over the past few decades.They represent a highly heterogeneous group of neoplasms with several prognostic factors,including disease stage,proliferative index(Ki67),and tumor differentiation.Most of these neoplasms express somatostatin receptors on the cell surface,a feature that has important implications in terms of prognosis,diagnosis,and therapy.Although International Guidelines propose algorithms aimed at guiding therapeutic strategies,GEP-NEN patients are still very different from one another,and the need for personalized treatment continues to increase.Radical surgery is always the best option when feasible;however,up to 80%of cases are metastatic upon diagnosis.Regarding medical treatments,as GEP-NENs are characterized by relatively long overall survival,multiple therapy lines are adopted during the lifetime of these patients,but the optimum sequence to be followed has never been clearly defined.Furthermore,although new molecular markers aimed at predicting the response to therapy,as well as prognostic scores,are currently being studied,their application is still far from being part of daily clinical practice.As they represent a complex disease,with therapeutic protocols that are not completely standardized,GEP-NENs require a multidisciplinary approach.This review will provide an overview of the available therapeutic options for GEP-NENs and attempts to clarify the possible approaches for the management of these patients and to discuss future perspectives in this field.

CONGENITAL EXPRESSION OF mdr-1 GENE IN FRESH CANCER TISSUES FROM SEVERAL HIGH-INCIDENCE NEOPLASMS WITHOUT PREOPERATIVE CHEMOTHERAPY

Objective: The purpose of the present study is to detect characteristics of primary expression of mdr 1 gene in several neoplasms which has high morbidity in clinic. Methods: 151 resected samples, which are pathologically malignant and clinically untreated before operation, were obtained from Anyang Cancer Hospital. All of them were investigated with RT PCR for the expression of mdr 1 gene and correlated each other. Besides, we evaluated the advantages of RT PCR in this study. Results: The mdr 1 gene expression rate of these 151 samples, including cancers of stomach and gastric cardia (n=51), esophagus (n=46), colorectum (n=16), breast (n=15), thyroid (n=10), lung (n=9), uterine cervix (n=4), was 33.3%, 37%, 31.3%, 13.2%, 40%, 55%, 0%, respectively. Conclusion: Compared with other methods, RT PCR for studying mdr 1 gene expression had certain advantages in simplicity, reliability, and accuracy. Overexpression of mdr 1 gene in these neoplasms suggested that cases should be distinguished before treatment according to MDR of tumor and to choose effective drugs for individual cancer patient.

Chronic infectious unilateral giant thyroid cyst related to diabetes mellitus:A case report

BACKGROUND Patients rarely develop complicated infections in thyroid cysts.Here,we describe a patient with chronic infected unilateral giant thyroid cyst related to diabetes mellitus(DM).CASE SUMMARY A 66-year-old male was admitted due to an evident neck lump for 5 d after approximately 40 years of gradually progressive neck mass and 7 years of DM.Doppler ultrasound and computed tomography scan showed a giant lump in the left thyroid gland lobe.He was diagnosed with a large thyroid nodule complicated by tracheal dislocation and had surgical indications.Surgical exploration revealed evident inflammatory edema and exudation between the left anterior neck muscles,the nodule and glandular tissue.Fortunately,inflammatory lesions did not affect major neck vessels.Finally,a left partial thyroidectomy was performed.Macroscopic observation showed that the cystic thyroid mass consisted of extensive cystic wall calcification and was rich in massive rough sand-like calculi content and purulent matter.Postoperative pathology confirmed benign thyroid cyst with chronic infection.CONCLUSION The progression of this chronic infectious unilateral giant thyroid cyst may have been related to DM,and identifying blood vessels involvement can prevent serious complications during operation.

Surgical Reports of the Inferior Laryngeal Nerve and the Inferior Thyroid Artery in General Surgery and in ENT

Aim: The purpose of this study was to determine the frequency of the hurts of the inferior laryngeal nerve, according to its anatomical reports with the inferior thyroid artery during the thyroid surgery. Methodology: We realized a forward-looking and retrospective study from January, 1979 till December, 2017 in the service of surgery “B” to the University hospital of the Point G of Bamako and in the service of ENT and cervico-facial surgery of the Teaching Hospital “Mother-Child”, the Luxembourg of Bamako (Mali). All the patients operated in both services for mild goiters were retained. Cancers and other thyroid pathologies were not included. The diagnosis of mild goiter was paused by the histological examination realized on all the surgical specimens. Results: On 2109 dissections of the inferior laryngeal nerve realized during the surgical operations on the thyroid, the frequency of lesion of the inferior laryngeal nerve was 1.09% (20 cases) when it passed dorsally with regard to the inferior thyroid artery (1837 cases) and when 4.04%, it was transvascular or prevascular (272 cases). Conclusion: The prevascular route or transvascular of the inferior laryngeal nerve favors its lesion per operating.

Pancreas sparing duodenectomy in the treatment of primary duodenal neoplasms and other situations with duodenal involvement

Background:There are no clearly defined indications for pancreas-preserving duodenectomy.The present study aimed to analyze postoperative morbidity and the outcomes of patients undergoing pancreaspreserving duodenectomy.Methods:Patients undergoing pancreas-preserving duodenectomy from April 2008 to May 2020 were included.We divided the series according to indication:scenario 1,primary duodenal tumors;scenario 2,tumors of another origin with duodenal involvement;and scenario 3,emergency duodenectomy.Results:We included 35 patients.Total duodenectomy was performed in 1 patient of adenomatous duodenal polyposis,limited duodenectomy in 7,and third+fourth duodenal portion resection in 27.The indications for scenario 1 were gastrointestinal stromal tumor(n=13),adenocarcinoma(n=4),neuroendocrine tumor(n=3),duodenal adenoma(n=1),and adenomatous duodenal polyposis(n=1);scenario 2:retroperitoneal desmoid tumor(n=2),recurrence of liposarcoma(n=2),retroperitoneal paraganglioma(n=1),neuroendocrine tumor in pancreatic uncinate process(n=1),and duodenal infiltration due to metastatic adenopathies of a germinal tumor with digestive hemorrhage(n=1);and scenario 3:aortoenteric fistula(n=3),duodenal trauma(n=1),erosive duodenitis(n=1),and biliopancreatic limb ischemia(n=1).Severe complications(Clavien-Dindo≥IIIb)developed in 14%(5/35),and postoperative mortality was 3%(1/35).Conclusions:Pancreas-preserving duodenectomy is useful in the management of primary duodenal tumors,and is a technical option for some tumors with duodenal infiltration or in emergency interventions.

Metastasis of liver cancer to the thyroid after surgery:A case report

BACKGROUND Secondary thyroid malignancies are rarely reported,especially thyroid metastasis after resection of hepatocellular carcinoma(HCC).We report a patient with thyroid metastasis after resection of HCC.CASE SUMMARY A 42-year-old female underwent partial hepatectomy for HCC three years ago.She attended hospital because of neck discomfort.After various examinations,she was diagnosed with metastatic HCC.She survived after surgical resection of the affected side of the thyroid.CONCLUSION Although secondary malignant tumor of the thyroid is classified as distant metastasis,surgical resection is still necessary according to the patient's condition.

Collision Neoplasms of the Vermiform Appendix

Neoplasms of the vermiform appendix represent 1% of all gastrointestinal tumors, with epithelial and neuroendocrine histological subtypes being the most frequent. When two or more neoplasms with different components originate synchronically in the appendix with clear margin between them, it is called a collision tumor, with exceptional cases being reported in literature. The purpose is to present two new cases of collision tumors of the appendix and perform a review of the published literature. Two cases of an 82-year-old female and a 41-year-old male with collision tumors of the vermiform appendix are presented, with clinical presentation, radiological evaluation and surgical treatment exposed. Our results are compared with other published reports. In accordance with the other reported cases, both patients presented with low-grade appendiceal mucinous neoplasm (LAMN). Female patient with synchronous goblet cell carcinoma managed with a completion right colectomy and Hyperthermic intraperitoneal chemotherapy (HIPEC) and the other patient with well-differentiated neuroendocrine (NET) tumor managed with laparoscopic appendectomy without adjuvant treatment. Since very few cases have been reported, no diagnostic and therapeutic consensus and guidelines exist up to date. We agree that local, regional, and systemic therapies should be chosen according to the tumor that represents the worst prognosis.

Current status of surgical management of patients with gastroenteropancreatic neuroendocrine neoplasms

Neuroendocrine neoplasms(NENs)of the gastroenteropancreatic system are rare and heterogeneous tumours,yet with increasing prevalence.The most frequent primary sites are the small intestine,rectum,pancreas,and stomach.For a localized disease,surgical resection with local lymph nodes is usually curative with good overall and disease free survival.More complex situation is the treatment of locally advanced lesions,liver metastases,and,surprisingly,small asymptomatic tumours of the rectum and pancreas.In this review,we focus on the current role of surgical management of gastroenteropancreatic NENs.We present surgical approach for the most frequent primary sites.We highlight the role of endoscopic surgery and the watch-and-wait strategy for selected cases.As liver metastases pose an important clinical challenge,we present current indications and contraindications for liver resection and a role of liver transplantation for metastatic NENs.