Combination of modified FOLFIRINOX with stereotactic body radiotherapy as an induction therapy for locally advanced pancreatic adenocarcinoma - a prospective single-arm study.

Introduction: Radical resection is the only potentially curative treatment for pancreatic adenocarcinoma; however, only a minor fraction of patients are eligible for resection. Induction therapy may be offered to patients, but the response rate in cases with significant vascular involvement is limited. This study aimed to evaluate the efficacy and safety of modified of FOLFIRINOX chemotherapy (mFFX) + stereotactic body radiotherapy (SBRT) in combination as induction therapy for locally advanced pancreatic carcinoma. The primary endpoints were the resection rate and one-year overall survival (OS). The secondary endpoints were progression-free survival (PFS), toxicity, and quality of live (QoL). Material and methods: Thirty patients with locally advanced pancreatic adenocarcinoma were treated with 6 cycles of mFFX, followed by SBRT and additional 3 cycles of mFFX. The response was measured prior to SBRT and after regimen completion. In the absence of disease progression, the patients were referred for surgery. The patients were requested to complete quality of life questionnaires (QLQ)-C30 and QLQ-PAN26 questionnaires biweekly. Results: On the first evaluation, disease control was noted in 26 (86.7%) patients. Stereotactic body radiotherapy was performed in 20 patients. Twelve patients underwent laparotomy, with radical resection possible in 3 cases. The one-year OS rate was 63.3%. Overall, 11 grade ≥ 3 adverse events were noted. No deterioration in the overall QoL was observed. The median PFS was 7.53 months. Conclusions: The expected resection rate of ≥ 30% was not achieved. However, the combination was associated with good local control, low adverse event rate, and good QoL, which advocate its further investigation in this clinical situation.

Prospective Evaluation of the NETest as a Liquid Biopsy for Gastroenteropancreatic and Bronchopulmonary Neuroendocrine Tumors: An ENETS Center of Excellence Experience.

BACKGROUND: There is a substantial unmet clinical need for an accurate and effective blood biomarker for neuroendocrine neoplasms (NEN). We therefore evaluated, under real-world conditions in an ENETS Center of Excellence (CoE), the clinical utility of the NETest as a liquid biopsy and compared its utility with chromogranin A (CgA) measurement. METHODS: The cohorts were: gastroenteropancreatic NEN (GEP-NEN; n = 253), bronchopulmonary NEN (BPNEN; n = 64), thymic NEN (n = 1), colon cancer (n = 37), non-small-cell lung cancer (NSCLC; n = 63), benign lung disease (n = 59), and controls (n = 86). In the GEPNEN group, 164 (65%) had image-positive disease (IPD, n = 135) or were image-negative but resection-margin/biopsy-positive (n = 29), and were graded as G1 (n = 106), G2 (n = 49), G3 (n = 7), or no data (n = 2). The remainder (n = 71) had no evidence of disease (NED). In the BPNEN group, 43/64 (67%) had IPD. Histology revealed typical carcinoids (TC, n = 14), atypical carcinoids (AC, n = 14), small-cell lung cancer (SCLC, n = 11), and large-cell neuroendocrine carcinoma (LCNEC, n = 4). Disease status (stable or progressive) was evaluated according to RECIST v1.1. Blood sampling involved NETest (n = 563) and NETest/CgA analysis matched samples (n = 178). NETest was performed by PCR (on a scale of 0-100), with a score ≥20 reflecting a disease-positive status and >40 reflecting progressive disease. CgA positivity was determined by ELISA. Samples were deidentified and measurements blinded. The Kruskal-Wallis, Mann-Whitney U, and McNemar tests, and the area under the curve (AUC) of the receiver-operating characteristics (ROC) were used in the statistical analysis. RESULTS: In the GEPNEN group, NETest was significantly higher (34.4 ± 1.8, p < 0.0001) in disease-positive patients than in patients with NED (10.5 ± 1, p < 0.0001), colon cancer patients (18 ± 4, p < 0.0004), and controls (7 ± 0.5, p < 0.0001). Sensitivity for detecting disease compared to controls was 89% and specificity was 94%. NETest levels were increased in G2 vs. G1 (39 ± 3 vs. 32 ± 2, p = 0.02) and correlated with stage (localized: 26 ± 2 vs. regional/distant: 40 ± 3, p = 0.0002) and progression (55 ± 5 vs. 34 ± 2 in stable disease, p = 0.0005). In the BPNEN group, diagnostic sensitivity was 100% and levels were significantly higher in patients with bronchopulmonary carcinoids (BPC; 30 ± 1.3) who had IPD than in controls (7 ± 0.5, p < 0.0001), patients with NED (24.1 ± 1.3, p < 0.005), and NSCLC patients (17 ± 3, p = 0.0001). NETest levels were higher in patients with poorly differentiated BPNEN (LCNEC + SCLC; 59 ± 7) than in those with BPC (30 ± 1.3, p = 0.0005) or progressive disease (57.8 ± 7), compared to those with stable disease (29.4 ± 1, p < 0.0001). The AUC for differentiating disease from controls was 0.87 in the GEPNEN group and 0.99 in BPC patients (p < 0.0001). Matched CgA analysis was performed in 178 patients. In the GEPNEN group (n = 135), NETest was significantly more accurate for detecting disease (99%) than CgA positivity (53%; McNemar test χ2 = 87, p < 0.0001). In the BPNEN group (n = 43), NETest was significantly more accurate for disease detection (100%) than CgA positivity (26%; McNemar's test χ2 = 30, p < 0.0001). CONCLUSIONS: The NETest is an accurate diagnostic for GEPNEN and BPNEN. It exhibits tumor biology correlation with grading, staging, and progression. CgA as a biomarker is significantly less accurate than NETest. The NETest has substantial clinical utility that can facilitate patient management.

Small Intestinal Intussusception Due to Complicated Giant Jejunal Diverticulosis.

Background: Jejunal diverticulosis and jejunal lipomatosis are uncommon conditions. Usually asymptomatic, they may cause severe complications in some cases. Intussusception is unusual in adults, but when diagnosed swiftly it can be treated surgically, usually with good outcome. Case presentation: We present a 60-year-old female patient with a history of chronic malnutrition and anemia, complaining of acute abdominal pain, vomiting and diarrhea. Contrast-enhanced abdominal computed tomography (CT) showed intussusception, multiple giant jejunal diverticula and multiple lipomas. The patient underwent urgent surgery, but radical treatment was not possible due to the extent of the diseases. One month later, another surgery was needed due to ileostomy obstruction caused by lipomas. The patient's condition deteriorated due to malnutrition and concomitant metabolic disorders, which eventually led to her demise. Conclusions: Radical treatment is not always possible in an extensive jejunal disease. Prolonged malnutrition impairs postoperative healing, and therefore surgical or nutritional treatment should be considered in jejunal diverticulosis before the onset of severe complications requiring urgent surgical intervention.

The new method, the old problem - role of contrast-enhanced spectral mammography in the diagnosis of breast cancer among Polish women.

PURPOSE: The aim of the study was to evaluate spectral mammography (CESM) in diagnosing breast cancer, which is based on sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). MATERIAL AND METHODS: The study included a group of 547 women who underwent spectral mammography and histopathological verification of the lesion, previously seen in mammography and/or ultrasound. In the group of 547 women, 593 focal lesions were diagnosed. All CESM examinations were carried-out with a digital mammography device dedicated to performing dual-energy CESM acquisitions. An intravenous injection of 1.5 ml/kg of body mass of non-ionic contrast agent was performed. RESULTS: The analysis includes 593 breast lesions, in this group cancer was detected in 327 (55.14%) lesions, and in 256 (43.17%) cases benign lesions were confirmed by histopathological examination and at least 12 months of observation. The method shows differentiation of benign and malignant lesions in the breast: sensitivity of 97.86%, specificity of 59.4%, PPV - 74.76%, NPV - 95.76%. CONCLUSIONS: Spectral mammography could be an ideal method to detect breast cancer. Thanks to the high NPV (95.76%), it facilitates the exclusion of cancer in situations where pathological contrast enhancement is not observed. The unsatisfactory specificity of the study (59.4%) would not make it safe to avoid a core needle biopsy of lesions that undergo contrast enhancement.

Traumatic basal subarachnoid haemorrhage or ruptured brain aneurysm in 16-year-old boy? - case report

Traumatic basal subarachnoid haemorrhage (TBSAH) represents only 1.8% of all subarachnoid haemorrhage cases diagnosed during autopsy. This report presents such a case from the current practice of the authors. Sixteen-year-old boy was beaten by the aggressors. Suddenly he lost his consciousness and fall after he received a single blow in the neck. He was resuscitated immediately, but died at the scene. During the external examination we did not find any significant external injuries. Autopsy revealed large contusion of right sternocleidomastoid muscle. In the cranial cavity we found extensive subarachnoid haemorrhage, located mainly on brain basis, in the posterior cranial fossa and covering the subtentorial structures. During the preparation of blood vessels we noticed a slight change of morphology suggesting damaged vessel or aneurysm, or vascular malformation located in the basilar artery bifurcation, which was taken to detailed microscopic evaluation using the special stainings. Histological examination showed vital interruption of the basilar artery wall with massive haemorrhage, without the presence of general microscopic pathology. From the medico-legal viewpoint, to determine traumatic background of haemorrhage it is necessary to find the coexistence of the following circumstances: a sustained trauma, post-mortem findings consistent with a time of injury, the presence of temporal relationship between injury and death, and morphological vital injury of the brain vessel, as well as the absence of prior vascular malformations. For this purpose Verhoeff-van Gieson's, Masson's, Turnbull's and Gomori' histological stainings may be successfully used.

Detection of cerebral artery fenestrations by computed tomography angiography.

BACKGROUND AND PURPOSE: Cerebral artery fenestrations (CAF) are rare congenital variations usually diagnosed by digital subtraction angiography (DSA). The aim of this study was to examine the frequency of occurrence of fenestrations in cerebral arteries and their coexistence with cerebral aneurysms in computed tomography angiography (CTA). MATERIAL AND METHODS: All reports of cerebral CTA (1140) performed in one institution from March 2005 to December 2007 were analysed. We found 40 patients with single fenestrations of the intracranial arteries. All 40 examinations were retrospectively reviewed for location of vascular malformations and presence of aneurysms or subarachnoid haemorrhage (SAH). Medical histories of those patients were then analysed for evidence of SAH and referral reasons for CTA. RESULTS: Forty fenestrated arteries were found in CTA: 18 basilar arteries (45%), 16 anterior cerebral arteries (40%), 4 anterior communicating arteries (10%) and one middle cerebral artery (2.5%). Only one vertebral artery fenestration was found due to the technique of the examination. Six patients (15%) with fenestrated arteries had a total of 8 aneurysms, although only one aneurysm was ipsilateral to the fenestration. In 8 cases of SAH, two were with no evidence of vascular malformation. The coexistence of CAF and aneurysms in CTA amounted to 15% (6/40), but the incidence of ipsilateral aneurysm was only 2.5% (1/40) and it affected the anterior cerebral artery. CONCLUSIONS: Basilar artery fenestration is the most frequent observed fenestration in CTA, followed by anterior cerebral artery and anterior communicating artery fenestrations. Coexistence of fenestration and aneurysm is uncommon in CTA examination.

Update of the diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours) [Aktualizacja zalecen ogólnych dotyczacych postepowania diagnostyczno-terapeutycznego w nowotworach neuroendokrynnych ukladu pokarmowego (rekomendowane przez Polska Siec Guzów Neuroendokrynnych)].

Continuous progress in the diagnostics and treatment of neuroendocrine neoplasms (NENs), the emerging results of new clinical trials, and the new guidelines issued by medical societies have prompted experts from the Polish Network of Neuroendocrine Tumours to update the 2017 recommendations regarding the management of neuroendocrine neoplasms. This article presents the general recommendations for the management of NENs, resulting from the findings of the experts participating in the Fourth Round Table Conference, entitled "Polish Guidelines for the Diagnostics and Treatment of Neuroendocrine Neoplasms of the gastrointestinal tract, Zelechów, June 2021". Drawing from the extensive experience of centres treating these cancers, we hope that we have managed to formulate the optimal method of treating patients with NENs, applying the latest reports and achievements in the field of medicine, which can be effectively implemented in our country. The respective parts of this work present the approach to the management of: NENs of the stomach and duodenum (including gastrinoma), pancreas, small intestine, and appendix, as well as large intestine.

Pancreatic neuroendocrine neoplasms - update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne trzustki - uaktualnione zasady diagnostyki i leczenia (rekomendowane przez Polska Siec Guzów Neuroendokrynych)].

In this paper, we present the current guidelines for the diagnostics and management of pancreatic neuroendocrine neoplasms (PanNENs) developed by Polish experts providing care for these patients in everyday clinical practice. In oncological diagnostics, in addition to biochemical tests, molecular identification with the use of NETest liquid biopsy and circulating microRNAs is gaining importance. Both anatomical and functional examinations (including new radiopharmaceuticals) are used in imaging diagnostics. Histopathological diagnosis along with immunohistochemical examination still constitute the basis for therapeutic decisions. Whenever possible, surgical procedure is the treatment of choice. Pharmacological management including biotherapy, radioisotope therapy, targeted molecular therapy and chemotherapy are important methods of systemic therapy. Treatment of PanNENs requires a multidisciplinary team of specialists in the field of neuroendocrine neoplasms.

Gastroduodenal neuroendocrine neoplasms including gastrinoma - update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne zoladka i dwunastnicy z uwzglednieniem gastrinoma - uaktualnione zasady postepowania (rekomendowane przez Polska Siec Guzów Neuroendokrynnych)].

After another meeting of experts of the Polish Network of Neuroendocrine Tumours, updated recommendations for the management of patients with gastric and duodenal neuroendocrine neoplasms, including gastrinoma, have been issued. As before, the epidemiology, pathogenesis and clinical symptoms of these neoplasms have been discussed, as well as the principles of diagnostic procedures, including biochemical and histopathological diagnostics and tumour localisation, highlighting the changes introduced in the recommendations. Updated principles of therapeutic management have also been presented, including endoscopic and surgical treatment, and the options of pharmacological and radioisotope treatment. The importance of monitoring patients with gastric and duodenal NENs, including gastrinoma, has also been emphasised.

Colorectal neuroendocrine neoplasms - update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne jelita grubego - uaktualnione zasady diagnostyki i leczenia (rekomendowane przez Polska Siec Guzów Neuroendokrynych)].

Colorectal neuroendocrine neoplasm (CRNEN), especially rectal tumours, are diagnosed with increased frequency due to the widespread use of colonoscopy, including screening examinations. It is important to constantly update and promote the principles of optimal diagnostics and treatment of these neoplasms. Based on the latest literature and arrangements made at the working meeting of the Polish Network of Neuroendocrine Tumours (June 2021), this paper includes updated and supplemented data and guidelines for the management of CRNEN originally published in Endokrynologia Polska 2017; 68: 250-260.

Neuroendocrine neoplasms of the small intestine and the appendix - update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne jelita cienkiego i wyrostka robaczkowego - uaktualnione zasady diagnostyki i leczenia (rekomendowane przez Polska Siec Guzów Neuroendokrynnych)].

Updated Polish recommendations for the management of patients with neuroendocrine neoplasms (NENs) of the small intestine (SINENs) and of the appendix (ANENs) are presented here. The small intestine, and especially the ileum, is one of the most common locations for these neoplasms. Most of them are well-differentiated and slow-growing tumours; uncommonly - neuroendocrine carcinomas. Their symptoms may be untypical and their diagnosis may be delayed or accidental. Najczesciej pierwsza manifestacja ANEN jest jego ostre zapalenie. Typical symptoms of carcinoid syndrome occur in approximately 20-30% of SINENs patients with distant metastases. In laboratory diagnostics the assessment of 5-hydroxyindoleacetic acid concentration is helpful in the diagnosis of carcinoid syndrome. The most commonly used imaging methods are ultrasound examination, computed tomography, magnetic resonance imaging, colonoscopy and somatostatin receptor imaging. Histopathological examination is crucial for the proper diagnosis and treatment of patients with SINENs and ANENs. The treatment of choice is a surgical procedure, either radical or palliative. Long-acting somatostatin analogues (SSAs) are essential in the medical treatment of functional and non-functional SINENs. In patients with SINENs, at the stage dissemination with progression during SSAs treatment, with high expression of somatostatin receptors, radioisotope therapy should be considered first followed by targeted therapies - everolimus. After the exhaustion of the above available therapies, chemotherapy may be considered in selected cases. Recommendations for patient monitoring are also presented.

Pancreatic neuroendocrine carcinoma metastasis to the skin.

Not required for Clinical Vignette.

Are We Overdoing It? Changes in Diagnostic Imaging Workload during the Years 2010-2020 including the Impact of the SARS-CoV-2 Pandemic.

Since the 1990s, there has been a significant increase in the number of imaging examinations as well as a related increase in the healthcare expenditure and the exposure of the population to X-rays. This study aimed to analyze the workload trends in radiology during the last decade, including the impact of COVID-19 in a single university hospital in Poland and to identify possible solutions to the challenges that radiology could face in the future. We compared the annual amount of computed tomography (CT), radiography (X-ray), and ultrasound (US) examinations performed between the years 2010 and 2020 and analyzed the changes in the number of practicing radiologists in Poland. The mean number of patients treated in our hospital was 60,727 per year. During the last decade, the number of CT and US examinations nearly doubled (from 87.4 to 155.7 and from 52.1 to 86.5 per 1000 patients in 2010 and 2020 respectively), while X-ray examinations decreased from 115.1 to 96.9 per 1000 patients. The SARS-CoV-2 pandemic did not change the workload trends as more chest examinations were performed. AI, which contributed to the COVID-19 diagnosis, could aid radiologists in the future with the growing workload by increasing the efficiency of radiology departments as well as by potentially minimizing the related costs.

Pancreaticopleural and pancreaticomediastinal fistula extending to the cervical region, with dysphagia as initial symptom: A case report.

RATIONALE: Pancreaticopleural and pancreaticomediastinal fistulas are rare complications of pancreatitis. They are often misdiagnosed and there are no strict guidelines of treatment. In this study, we present a brief report of a combined pancreaticopleural and pancreaticomediastinal fistula extending to the cervical region, causing dysphagia and cervical swelling as initial symptoms. PATIENT CONCERNS: A 36-year-old female with history of alcohol abuse and pancreatitis presented progressing dysphagia and mild dyspnea on admission. DIAGNOSIS: Chest X-ray and chest and abdominal computed tomography scan (CT) indicated pancreaticopleural fistula combined with pancreaticomediastinal fistula, a diagnosis confirmed by high amylase levels in pleural fluid. INTERVENTIONS: Conservative treatment was administered and ERCP was performed but pancreatic duct stenting was impossible. The patient presented rapid anterior cervical swelling with progressing dysphagia and dyspnea. CT showed fistula penetration to the cervical region. The patient underwent urgent surgery and pancreaticojejunal anastomosis was performed. OUTCOMES: The surgery led to recovery. Six months later, the patient reported good health and weight gain. LESSONS: Coexistence of pancreaticopleural and pancreaticomediastinal fistula with cervical penetration is an extremely rare pancreatitis complication. It presents with dysphagia and anterior cervical swelling as initial symptoms. It is important to consider this complication in all patients with history of pancreatitis, presenting with dysphagia.

NETest liquid biopsy is diagnostic of small intestine and pancreatic neuroendocrine tumors and correlates with imaging.

INTRODUCTION: Current monoanalyte biomarkers are ineffective in gastroenteropancreatic neuroendocrine tumors (GEP-NETs). NETest, a novel multianalyte signature, provides molecular information relevant to disease biology. AIM(S): Independently validate NETest to diagnose GEP-NETs and identify progression in a tertiary referral center. MATERIALS AND METHODS: Cohorts: 67 pancreatic NET (PNETs), 44 small intestine NETs (SINETs), 63 controls. Well-differentiated (WD): PNETs, n=62, SINETs, all (n=44). Disease extent assessment at blood draw: anatomical (n=110)- CT(n=106), MRI(n=7) and/or functional- 68Ga-SSA-PET/CT(n=69) or 18F-FDG-PET/CT (n=8). Image positive disease (IPD) was defined as either CT/MRI or 68Ga-SSA-PET/CT/18F-FDG-PET/CT-positive. Both CT/MRI and 68Ga-SSA-PET/CT-negative in WD-NETs was considered image negative disease (IND). NETest (normal: 20): PCR (spotted plates). DATA: mean±SD. RESULTS: Diagnosis: NETest was significantly increased in NETs (n=111; 26±21) vs. controls (8±4, p<0.0001). 75 (42 PNET, 33 SINET) were image-positive. Eleven (8 PNET, 3 SINET; all WD) were IND. In IPD, NETest was significantly higher (36±22) vs. IND (8±7, p<0.0001). NETest accuracy, sensitivity, specificity: 97%, 99%, 95%. Concordance with imaging: NETest was 92% (101/110) concordant with anatomical imaging, 94% (65/69) with 68Ga-SSA-PET/CT, 96% (65/68) dual modality (CT/MRI and 68Ga-SSA-PET/CT). In 70 CT/MRI-positive, NETest was elevated in all (37±22). In 40 CT/MRI-negative, NETest was normal (11±10) in 31. In 56 68Ga-SSA-PET/CT-positive, NETest was elevated (36±22) in 55. In 13 68Ga-SSA-PET/CT-negative, NETest was normal (9±8) in 10. Disease status: NETest was significantly higher in progressive (61±26; n=11) vs. stable disease (29±14; n=64; p<0.0001) (RECIST 1.1). CONCLUSION: NETest is an effective diagnostic for PNETs and SINETs. Elevated NETest is as effective as imaging in diagnosis and accurately identifies progression.

Cystic pancreatic neuroendocrine tumours - a gastroenterologist's point of view.

Cystic pancreatic tumors are detected with increasing frequency and remain a clinical problem. Since they have different potential of malignancy the management and decision making process is a hard task. Guidelines, concerning pancreatic cystic tumors indicate the management with mucinous, serous cystic pancreatic neoplasms and solid pseudopappilary tumor, while the management with pancreatic cystic neuroendocrine tumors is not included into these standards. This review tries to answer the question are the cystic pancreatic neuroendocrine tumors different entity from solid tumors of neuroendocrine origin.The management and differential diagnosis of these neoplasms with special focus on features on imaging studies allowing preoperative diagnosis are discussed.

Primary neuroendocrine carcinoma of the breast - a report of four cases.

Breast neuroendocrine tumours are rare, accounting for up to 5% of all breasts tumours and approximately 1% of all neuroendocrine tumours. In most cases, breast neuroendocrine tumours are histologically and moderately well differentiated. Neuroendocrine breast tumours lack characteristic imaging patterns. The histopathological assessment of these tumours is difficult, and in most cases the correct diagnosis is made after proper examination of the postsurgical specimen.

Diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours).

Progress in the diagnostics and therapy of gastro-entero-pancreatic (GEP) neuroendocrine neoplasms (NEN), the published results of new randomised clinical trials, and the new guidelines issued by the European Neuroendocrine Tumour Society (ENETS) have led the Polish Network of Neuroendocrine Tumours to update the 2013 guidelines regarding management of these neoplasms. We present the general recommendations for the management of NENs, developed by experts during the Third Round Table Conference - Diagnostics and therapy of gastro-entero-pancreatic neuroendocrine neoplasms: Polish recommendations in view of current European recommenda-tions, which took place in December 2016 in Zelechów near Warsaw. Drawing from the extensive experience of centres dealing with this type of neoplasms, we hope that we have managed to develop the optimal management system, applying the most recent achievements in the field of medicine, for these patients, and that it can be implemented effectively in Poland. These management guidelines have been arranged in the following order: gastric and duodenal NENs (including gastrinoma); pancreatic NENs; NENs of the small intestine and appendix, and colorectal NENs.

Gastroduodenal neuroendocrine neoplasms, including gastrinoma - management guidelines (recommended by the Polish Network of Neuroendocrine Tumours).

This paper presents the updated Polish Neuroendocrine Tumour Network expert panel recommendations on the management of neuroendocrine neoplasms (NENs) of the stomach and duodenum, including gastrinoma. The recommendations discuss the epidemiology, pathogenesis, and clinical presentation of these tumours as well as their diagnosis, including biochemical, histopathological, and localisation diagnoses. The principles of treatment are discussed, including endoscopic, surgical, pharmacological, and radionuclide treatments. Finally, there are also recommendations on patient monitoring.