Clinical features and prognosis of malignant small bowel tumors: Experience from a university hospital in Chile.

BACKGROUND: Small bowel tumors (SBT) are infrequent and represent a small proportion of digestive neoplasms. There is scarce information about SBT in Latin America. AIM: To describe the epidemiology, clinical characteristics, diagnostic methods, and survival of malignant SBTs. METHODS: Retrospective observational study of adult patients with histopathological diagnosis of SBT between 2007 and 2021 in a university hospital in Chile. RESULTS: A total of 104 patients [51.9% men; mean age 57 years] with SBT. Histological type: neuroendocrine tumor (NET) (43.7%, n=38), gastrointestinal stromal tumors (GIST) (21.8%, n=19), lymphoma (17.2%, n=15) and adenocarcinoma (AC) (11.5%, n=10). GIST was more frequent in duodenum (50%; n=12) and NET in the ileum (65.8%; n=25). Metastasis was observed in 17 cases, most commonly from colon and melanoma. Nausea and vomiting were significantly more often observed in AC (p=0.035), as well as gastrointestinal bleeding in GIST (p=0.007). The most common diagnostic tools were CT and CT enteroclysis with an elevated diagnostic yield (86% and 94% respectively). The 5-year survival of GIST, NET, lymphoma and AC were 94.7% (95%CI: 68.1-99.2), 82.2% (95%CI: 57.6-93.3), 40.0% (95%CI: 16.5-82.8) and 25.9% (95%CI: 4.5-55.7%), respectively. NET (HR 6.1; 95%CI: 2.1-17.2) and GIST (HR 24.4; 95%CI: 3.0-19.8) were independently associated with higher survival compared to AC, adjusted for age and sex. CONCLUSIONS: Malignant SBT are rare conditions and NETs are the most common histological subtype. Clinical presentation at diagnosis, location or complications may suggest a more probable diagnosis. GIST and NET are associated with better survival compared to other malignant subtypes.

[Pancreatic neuroendocrine tumours. What do we know of their history?]. / Tumores neuroendocrinos pancreáticos. ¿Qué conocemos de su historia?

Starting with Paul Langerhans, who first described pancreatic islets in 1869, this article reviews the various protagonists who, in the last century and a half, have contributed to the discovery of the main hormones originating in the pancreas, the analytical methods for their measurement, the imaging techniques for identifying tumoural location, and the various pancreatic neoplasms.

Prognostic factors in resected pancreatic neuroendocrine tumours: Experience in 95 patients. / Factores pronósticos de los tumores neuroendocrinos de páncreas resecados. Experiencia en 95 pacientes.

INTRODUCTION: The aim of this study was to analyze prognostic factors for survival and recurrence in patients with resected pancreatic neuroendocrine tumors (PNT). METHODS: Medical records of 95 patients with resected PNT were retrospectively reviewed. The variables studied were: age, sex, form of presentation (sporadic/familial tumors), functionality, type of tumor, localization, type of surgery, tumor size, multifocal tumors and recurrent rate. The new WHO classification (2010) was used. RESULTS: There were 45 men and 50 women. Mean age was 46.8 years. Regarding the mode of presentation, it was sporadic in 66 patients (69.8%) and 29 cases were familial neuroendocrine tumors (30.2%) in association with MEN 1 syndrome. The 59% of patients suffered from non-functional tumors and 41% were functional: 20 insulinoma, 16 gastrinoma, and 3 glucagonoma. Distal pancreatectomy was the most common surgical procedure, followed by tumor enucleation in 19 patients. According to the WHO classification, 59 patients had a PNT G1, 24 PNT G2 and 12 with a poorly-differentiated carcinoma, respectively. The 5-year survival in well-differentiated tumors was 100%, regardless of the functionality. Sporadic PNT are more commonly unifocal (P<0.001), associating liver metastasis. Survival and recurrence rates after a mean follow-up of 85.3 months were 65.8 and 24%, respectively. CONCLUSIONS: In our experience, WHO classification was an independent prognostic factor in PNT survival.

Rare tumors of the rectum. Narrative review.

Most rectal neoplasms are adenocarcinomas, but there is a small percentage of tumors which are of other histological cell lines such as neuroendocrine tumors, sarcomas, lymphomas and squamous cell carcinomas, which have special characteristics and different treatments. We have reviewed these rare tumors of the rectum from a clinical and surgical point of view.

Carcinoid crisis: The challenge is still there.

Carcinoid crisis (CC) has classically been considered the extreme end of the spectrum of carcinoid syndrome (CS). However, this presumption and other aspects of CC remain poorly understood. Consequently, current clinical guidelines are based on a low quality of evidence. There is no standard definition of CC and its incidence is unknown. Patients with florid CS and elevated serotonin (or its derivatives) which develop CC have been reported during decades. Nevertheless, the hypothesis that CC is due to the sudden massive release of serotonin or other vasoactive substances is unproven. Many triggers of CC (surgery, anaesthesia, peptide receptor radionuclide therapy, tumour biopsy or liver-directed treatments) have been proposed. However, data from studies are heterogeneous and even contradictory. Finally, the role of octreotide in the prevention of CC has been questioned. Herein, we report a clinical case and perform a critical review of the evidence available today on this topic.

Portuguese Pancreatic Club Perspective on the Surveillance Strategy for Pancreatic Neuroendocrine Tumours: When and How to Do It?

Background: Pancreatic neuroendocrine tumours (pNETs) are a highly heterogeneous group of tumours with widely variable biological behaviour. The incidence of pNETs has risen exponentially over the last three decades, particularly for asymptomatic small pNETs (≤2 cm), due to the widespread use of cross-sectional imaging in clinical practice. Summary: Current consensus guidelines suggest that incidentally discovered pNETs ≤2 cm can be selectively followed due to the overall low risk of malignancy. Nevertheless, the "watch-and-wait" management strategy for small asymptomatic pNETs is still not widely accepted due to the lack of long-term data on the natural history of these small lesions. Additionally, it is clear that a subset of small pNETs may show malignant behaviour. Key Message: Given the non-negligible risk of malignancy even in small pNETs, it is of the utmost importance to identify other preoperative factors, other than size, that may help to stratify the risk of malignant behaviour and guide clinical management. In this article, the Portuguese Pancreatic Club reviews the importance of risk stratification of pNETs and presents an updated perspective on the surveillance strategy for sporadic well-differentiated pNETs.

Contexto: Os tumores neuroendócrinos do pâncreas (pNETs) correspondem a um grupo heterogéneo de tumores com comportamento biológico variável. A sua incidência aumentou exponencialmente nas últimas três décadas, particularmente à custa do diagnóstico incidental de pNETs de reduzidas dimensões (≤2 cm) devido à utilização crescente de exames de imagem seccional na prática clínica. Sumário: As normas de consenso internacionais sugerem que os pNETs ≤2 cm poderão ser seletivamente vigiados, dado o seu baixo risco global de comportamento maligno. No entanto, a estratégia proposta de "watch and wait" na abordagem dos pNETs assintomáticos ≤2cm não tem sido amplamente aceite devido à ausência de dados a longo-prazo relativos à sua história natural. Adicionalmente, é hoje evidente que um subgrupo destes pequenos tumores poderá apresentar comportamento maligno. Mensagens Chave: Dado o risco não desprezível de agressividade biológica mesmo nos pNETs incidentais de reduzidas dimensões, torna-se essencial identificar fatores pré-operatórios, para além da dimensão do tumor, que permitam estratificar o seu risco de malignidade e guiar a abordagem clínica. No presente artigo o Clube Português de Pâncreas apresenta uma perspectiva atual sobre a estratificação do risco e a estratégia a adoptar na vigilância dos pNETs esporádicos bem-diferenciados.

Role of systemic inflammatory factors in gastroenteropancreatic neuroendocrine tumors (GEP-NETs) treated with peptide receptor radionuclide therapy (PRRT): From biology to theragnosis.

AIM: Systemic inflammatory factors have been validated as indicators of ongoing systemic inflammation that could be predictive markers of poor prognosis for oncological outcomes. However, the prognostic impact of systemic inflammation markers is unknown in patients with gastroenteropancreatic neuroendocrine tumors (GEP-NETs) treated with peptide receptor radionuclide therapy (PRRT). METHODS: We conducted an observational, retrospective, multicentric study of 40 patients with GEP or unknown origin NETs treated with PRRT between 2016 and 2020. The systemic inflammatory markers were calculated as follows: neutrophil to lymphocyte ratio (NLR)=neutrophil count/lymphocyte count, monocyte to lymphocyte ratio (MLR)=monocyte count/lymphocyte count, platelet to lymphocyte ratio (PLR)=platelet count/lymphocyte count, albumin to lymphocyte ratio (ALR)=albumin levels/lymphocyte count and derived Neutrophil to Lymphocyte ratio (dNLR)=neutrophil count/(leucocytes count - neutrophils count). Baseline analysis and after the second dose were used for the calculation of different ratios. RESULTS: The median age was 63 years (range 41-85), 55% were male. The baseline cut-off values for NLR were 2.61, for MLR 0.31, for PLR 110.14, for ALR 2.39 and for dNLR 1.71. The cut-off values after the 2° dose were, for NLR 2.3, for MLR 0.3, for PLR 131.61, ALR 4.16, and dNLR 1.48. Median progression-free survival (PFS) was 21.7 months (95% CI 10.7-32.8 months) and overall survival (OS) was 32.1 months (95% CI 19.6-44.7 months), PFS was shorter in patients with elevated NLR (p=0.001), ALR (0.03), and dNLR (p=0.001) in baseline analysis. DCR was 81% and ORR 18%. CONCLUSIONS: In GEP or unknown origin NETs treated with PRRT, we have identified the predictive and prognostic impact of baseline systemic inflammatory factors.

Indications for genetic study in gastro-entero-pancreatic and thoracic neuroendocrine tumors.

Gastro-entero-pancreatic (GEP-NET) and thoracic neuroendocrine tumours (NETs) are one of the most heritable groups of neoplasms in the body, being multiple endocrine neoplasia syndrome type 1 (MEN1), the genetic syndrome most frequently associated with this type of tumours. Moreover, Von Hippel Lindau syndrome, tuberous sclerosis, type 4 multiple neoplasia syndrome, and type 1 neurofibromatosis are associated with an increased risk of developing GEP-NETs. Another important aspect in GEP-NETs and thoracic NETs is the knowledge of the molecular background since the molecular profile of these tumours may have implications in the prognosis and in the response to specific treatments. This review summarizes the main indications for performing a genetic study in patients with GEP-NETs and thoracic NETs, and the methods used to carry it out. Moreover, it offers a description of the main hereditary syndromes associated with these NETs and their molecular background, as well as the clinical implications of the molecular profile.

Morfo-functional nutritional status in patients with gastroenteropancreatic neuroendocrine tumors (GEPNET).

INTRODUCTION: Patients with gastroenteropancreatic neuroendocrine tumors (GEPNET) can suffer from malnutrition. This is the first descriptive study of morfo-functional evaluation in GEPNET. OBJETIVES: To describe a morfo-functional evaluation, and to compare with reference population available, and to correlate different nutritional parameters. Moreover, to compare nutritional evaluation in our study with another same location non-neuroendocrine tumors. MATERIALS AND METHODS: A retrospective observational study, 50 patients with GEPNET, medium age 61 years. It was collected clinico-pathological characteristics and nutritional status was assessed by anthropometric, laboratory test, bioelectrical impedance analysis, phase angle, hand grip strength, and rectus femoris ultrasound for the assessment of muscle mass. We used SPSS statistics to describe and to analyze correlations of different variables. RESULTS: Most frequent location of tumor was pancreas and small bowel, tumor grade 1 and advance stage. In morfo-functional evaluation highlighted: a low fat mass percentage (23,5 vs 34,4%), low muscle mass percentage (36,5 vs 41,2%) and low body mass cell percentage (40,4 vs 48,8%) compared with reference population available by sex and age. Standarized phase angle by sex and age result as positive (+0,851). There was no difference in hand grip strength compared with reference population. After a review of differents studies available, phase angle and hand grip strength of non neuroendocrine tumors in same location, could suggest a minor affection of nutritional state in our GEPNET sample. A positive correlation was observed between non classic methods of nutritional evaluation such as bioelectrical impedance analysis, hand grip strength and rectus femoris ultrasound. CONCLUSION: Patients with gastroenteropancreatic neuroendocrine tumors (GEPNET) show a moderate affection of nutritional status in comparison to general population. Morphofunctional assessment provide us a precise knowledge about nutritional status.

Recommendations for the diagnosis and radiological follow-up of pituitary neuroendocrine tumours.

Pituitary neuroendocrine tumours (PitNETs) constitute a heterogeneous group of tumours with a gradually increasing incidence, partly accounted for by more sensitive imaging techniques and more extensive experience in neuroradiology in this regard. Although most PitNETs are indolent, some exhibit aggressive behaviour, and recurrence may be seen after surgical removal. The changes introduced in the WHO classification in 2017 and terminological debates in relation to neuroendocrine tumours warrant an update of the guidelines for the diagnosis, preoperative and postoperative management, and follow-up of response to treatment of PitNETs. This multidisciplinary document, an initiative of the Neuroendocrinology area of the Sociedad Española de Endocrinología y Nutrición [Spanish Society of Endocrinology and Nutrition] (SEEN), focuses on neuroimaging studies for the diagnosis, prognosis and follow-up of PitNETs. The basic requirements and elements that should be covered by magnetic resonance imaging are described, and a minimum radiology report to aid clinicians in treatment decision-making is proposed. This work supplements the consensus between the Neuroendocrinology area of the SEEN and the Sociedad Española de Anatomía Patológica [Spanish Society of Pathology] (SEAP) for the pathological study of PitNETs.

Chromogranin A (CgA) as a biomarker in carcinoid heart disease and NETG1/G2 neuroendocrine neoplasms of the small intestine (SI-NENs) related carcinoid syndrome.

INTRODUCTION: Progression of carcinoid syndrome (CS) to carcinoid heart disease (CHD) is difficult to predict. This retrospective analysis evaluates the use of chromogranin A (CgA), a biomarker widely used in the diagnosis of neuroendocrine tumours (NET), in monitoring CS and disease progression. PATIENTS AND METHODS: 108 patients with confirmed CS, selected from a group of 351 patients with neuroendocrine neoplasms of the small intestine (SI-NENs), including NETG1 well 40% and NETG2 60% moderately differentiated NET. CgA concentration was measured during initial diagnosis and clinical follow up in 84 patients, 27 of them subsequently developed CHD. The patient's overall survival (OS) was evaluated using the Kaplan-Meier method. RESULTS: Patients with CHD, were found to have significantly shorter OS than patients with CS but without CHD (67.22 vs. 73.03 months). Univariate and multivariate analyses revealed that initial high concentration of CgA and/or increased concentration of CgA is significantly associated with decreased median OS in patients with CS (p<0.05). CONCLUSION: CgA has potential as a clinically useful biomarker in reporting disease status and predicting outcome in patients with CS and with CHD.

Recommendations on the pathological report of pituitary tumors. A consensus of experts of the Spanish Society of Endocrinology and Nutrition and the Spanish Society of Pathology.

Pituitary neuroendocrine tumors (PitNETs) constitute, together with other tumors of the sellar region, 15-25% of intracranial neoplasms. In 2017, the World Health Organization proposed a new classification of PitNETs. The main innovation with respect to the 2004 classification was the recommendation to include in the immunohistochemical evaluation of PitNETs the determination of the transcription factors of the 3 pituitary cell lineages: Pit-1, Tpit and SF-1. Additionally, other clinicopathological classifications with a predictive capacity of tumor behavior during follow-up were proposed. Given these changes, it is appropriate to adapt the knowledge generated during the last 15 years to the daily practice of the treatment and monitoring of PitNETs at the Centers of Excellence in Pituitary Pathology. This document includes the positioning of the Spanish Society of Endocrinology and Nutrition (SEEN) and the Spanish Society of Pathology (SEAP) on the classification and denomination of the PitNETs and the information that the pathologist should provide to the clinician to facilitate the treatment and monitoring of these tumors.

[Recommendations on the pathological report of pituitary tumors. A consensus of experts of the Spanish Society of Endocrinology and Nutrition and the Spanish Society of Pathology]. / Recomendaciones sobre el diagnóstico e informe anatomopatológico de los tumores neuroendocrinos hipofisarios. Consenso de expertos de la Sociedad Española de Endocrinologia y Nutrición y de la Sociedad Española de Anatomía Patológica.

Pituitary neuroendocrine tumors (PitNETs) constitute, together with other tumors of the sellar region, 15-25% of intracranial neoplasms. In 2017, the World Health Organization proposed a new classification of PitNETs. The main innovation with respect to the 2004 classification was the recommendation to include in the immunohistochemical evaluation of PitNETs the determination of the transcription factors of the 3 pituitary cell lineages: Pit-1, Tpit and SF-1. Additionally, other clinicopathological classifications with a predictive capacity of tumor behavior during follow-up were proposed. Given these changes, it is appropriate to adapt the knowledge generated during the last 15 years to the daily practice of the treatment and monitoring of PitNETs at the Centers of Excellence in Pituitary Pathology. This document includes the positioning of the Spanish Society of Endocrinology and Nutrition (SEEN) and the Spanish Society of Pathology (SEAP) on the classification and denomination of the PitNETs and the information that the pathologist should provide to the clinician to facilitate the treatment and monitoring of these tumors.

Recommendations on the pathological report of pituitary tumors. A consensus of experts of the Spanish Society of Endocrinology and Nutrition and the Spanish Society of Pathology. / Recomendaciones sobre el diagnóstico e informe anatomopatológico de los tumores neuroendocrinos hipofisarios. Consenso de expertos de la Sociedad Española de Endocrinologia y Nutrición y de la Sociedad Española de Anatomía Patológica.

Pituitary neuroendocrine tumors (PitNETs) constitute, together with other tumors of the sellar region, 15-25% of intracranial neoplasms. In 2017, the World Health Organization proposed a new classification of PitNETs. The main innovation with respect to the 2004 classification was the recommendation to include in the immunohistochemical evaluation of PitNETs the determination of the transcription factors of the 3 pituitary cell lineages: Pit-1, Tpit and SF-1. Additionally, other clinicopathological classifications with a predictive capacity of tumor behavior during follow-up were proposed. Given these changes, it is appropriate to adapt the knowledge generated during the last 15 years to the daily practice of the treatment and monitoring of PitNETs at the Centers of Excellence in Pituitary Pathology. This document includes the positioning of the Spanish Society of Endocrinology and Nutrition (SEEN) and the Spanish Society of Pathology (SEAP) on the classification and denomination of the PitNETs and the information that the pathologist should provide to the clinician to facilitate the treatment and monitoring of these tumors.

Ultrasonography in infectious and neoplastic diseases of the bowel and peritoneum. / Ecografía de la patología infecciosa y tumoral del intestino y el peritoneo.

Ultrasonography is not the most cited imaging technique for the evaluation of infectious and neoplastic diseases of the gastrointestinal tract and the peritoneum, but it is often the initial technique used in the initial workup for nonspecific clinical syndromes. Despite its limitations, ultrasonography's strengths enable it to provide meaningful diagnostic information. To discuss the most important ultrasonographic, clinical, and epidemiological findings for infectious disease, we follow a topographical approach: stomach (Anisakis), proximal small bowel (Giardia lamblia, Strongyloides stercoralis, Mycobacterium avium-intracellulare complex, and Cryptosporidium), distal small bowel (Yersinia, Salmonella, and Campylobacter), terminal ileum and cecum (tuberculosis), right colon (Entamoeba histolytica), left colon (Shigella), sigmoid colon and rectum, pancolitis (Clostridium difficile, Cytomegalovirus, and Escherichia coli), and peritoneum. To discuss the ultrasonographic and clinical findings of the most common neoplastic diseases, we follow a nosological approach: polyploid lesions as precursors of tumors, carcinomas, neuroendocrine tumors, hematological tumors, mesenchymal tumors, and metastases. We briefly discuss tumors of the peritoneum and the use of ultrasonography to guide percutaneous biopsy procedures.

Is it possible NET-redifferentiation after chemotherapy? 68Ga DOTA-peptide imaging as a game-changer in therapy management. / ¿Es posible la rediferenciación de NET tras la quimioterapia? Captación de imágenes de DOTA-péptidos marcados con 68Ga como cambio de estrategia en el tratamiento terapéutico.

A 64-year-old man with pancreatic grade III neuroendocrine carcinoma underwent 68Ga DOTANOC PET/CT scan for staging. The pancreatic lesion, multiple peripancreatic lymph nodes and multiple gross metastases in both hepatic lobes were revealed with intense uptake. After 3 cycles of chemotherapy containing cisplatin and etoposide the primary and metastatic lesions were decreased in size, however showing higher uptake on follow-up 68Ga DOTANOC PET/CT scan. Another biopsy from liver demonstrated a significant decrease in Ki-67 proliferation index from 35 to 1%. The patient received 2 cycles of peptide receptor-targeted radionuclide therapy with 177Lu DOTANOC.

Factors related to gastric neuroendocrine tumors. / Factores relacionados a tumores neuroendocrinos gástricos.

INTRODUCTION AND AIMS: An association between long-term use of proton pump inhibitors and the development of gastric neuroendocrine tumors has been reported, but it is still a subject of debate. The aims of the present study were to determine the presence of this association in a Mexican population and to identify the risk factors for developing gastric neuroendocrine tumors. MATERIALS AND METHODS: A case-control study was conducted, in which the cases were patients with a histopathologic diagnosis of gastric neuroendocrine tumor and the controls were patients evaluated through upper endoscopy. The controls were paired by age, sex, and endoscopic examination indication. Proton pump inhibitor use was considered prolonged when consumption was longer than 5 years. RESULTS: Thirty-three patients with gastric neuroendocrine tumor and 66 controls were included in the study. Eighteen (54.5%) patients in the case group were women, as were 39 (59%) of the patients in the control group. The median age of the patients in the case group was 55 years (minimum-maximum range: 24-82) and it was 54 years (minimum-maximum range:18-85) in the control group. A greater number of patients in the gastric neuroendocrine tumor group presented with gastric atrophy (p<0.0001) and autoimmune atrophic gastritis (p=0.0002), compared with the control group. No association between gastric neuroendocrine tumor and prolonged proton pump inhibitor use, sex, smoking, gastroesophageal reflux disease, Helicobacter pylori infection, diabetes mellitus, or autoimmune diseases was found in the univariate analysis. CONCLUSIONS: The results of our study showed no association between proton pump inhibitor use for more than 5 years and the development of gastric neuroendocrine tumor. The presence of gastric atrophy and autoimmune atrophic gastritis was associated with gastric neuroendocrine tumor development.

[Tumores neuroendocrinos pancreáticos. Nuestra experiencia]. / Pancreatic neuroendocrine tumors. Our experience.

Pancreatic neuroendocrine tumors are rare. It is a heterogeneous group of neoplasms with very different behavior and prognosis. They can appear sporadically or associated with genetic syndromes. They are divided into functioning and non-functioning. A descriptive retrospective study of patients diagnosed with pancreatic neuroendocrine tumor was performed. The incidental diagnosis of these tumors is increasingly. The only curative treatment for these tumors is surgical excision, depending on the location and characteristics of the tumor and the patient. In selected cases a conservative attitude is recommended.

Los tumores neuroendocrinos pancreáticos son poco frecuentes. Es un grupo heterogéneo de neoplasias con comportamiento y pronóstico muy diferentes. Pueden aparecer de manera esporádica o asociados a síndromes genéticos. Se dividen en funcionantes y no funcionantes. Se realizó un estudio retrospectivo descriptivo de los pacientes diagnosticados de tumor neuroendocrino pancreático. Como se refleja en nuestra serie, cada vez es más frecuente el diagnostico incidental de estos tumores. El único tratamiento curativo de estos tumores es la exéresis quirúrgica, según la localización y las características del tumor y del paciente. En casos seleccionados se puede optar por una actitud conservadora.

PET/TC imaging with somatostatin analogues for assessment of neuroendocrine tumors, initial experience. / PET/TC con análogos de la somatostatina en el estudio de tumores neuroendocrinos, experiencia inicial.

PET with somatostatin analogues (SSA PET/CT) enables the detection of cells with overexpression of somatostatin receptors, especially subtypes 2 and 5; this detection is variable depending on the type of molecule used. This is the basis for its use in the study of neuroendocrine tumours (NETs), which are characterized by an overexpression of these receptors in more than 80% of the subtypes. This PET is now being used in our country supported by the good results published by other groups, that were superior to those of other imaging techniques. We present two of the first cases of SSA-PET/CT with 68Ga-edotreotide (SomaKit TOC®) performed in our centre. SSA-PET/CT was the test that finally determined the clinical management of both patients.

Detection of pancreatic neuroendocrine tumors: 23 years of experience. / Detección de tumores neuroendocrinos pancreáticos: 23 años de experiencia.

INTRODUCTION AND AIMS: Neuroendocrine tumors are of great scientific interest, given that they are difficult to diagnose and treat. Despite being relatively rare (< 1/100,000 individuals, 1-2% of the gastrointestinal neoplasias) and indolent, their potential malignancy must not be forgotten. An increase in the number of diagnosed tumors has been observed in recent years. The aim of the present study was to update a published case series of 19 patients suspected of presenting with pancreatic neuroendocrine tumor with 51 current cases, to study and compare the new results with those of the previous case series, as well as with other recent publications from Spain, the United States, China, and India. MATERIALS AND METHODS: A retrospective, multicenter case series was conducted on 70 patients (19 cases published in 2011), whose data has been collected over a period of 23 years. The variables analyzed were: age, sex, symptomatology, tumor size, location, metastasis, final diagnosis, and surgery, among others. RESULTS: Mean patient age was 55 years and 60% of the patients were men. Disease location was the pancreatic head in 28.5% of the patients and the tail in 27.1%, mean tumor size was 3.9cm (0.2-10cm), 71.4% of the patients had non-functioning tumors, 32.8% had metastases (100% to the liver), 74.2% of the patients were operated on, and actuarial survival was 75%. CONCLUSIONS: Differences were observed between the previously published case series and the current results. There was an increase in incidentalomas and non-functioning tumors, but no variation in the overall survival rate. The differences with other case series (age, sex, and tumor location) were dependent on the country where the cases were compiled. The increase in tumors could be related to a higher number of diagnoses made through imaging studies and to the greater sensitivity of the devices employed.