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Neuroendocrine neoplasms (NENs) encompass a heterogeneous spectrum of tumors originating from the diffuse neuroendocrine cell system. Approximately 30% of NEN exhibit functional activity with clinical syndromes through hormone-mediated effects. Synchronous and metachronous functioning syndromes, resulting from the simultaneous release of distinct hormones, are exceptionally rare. Of note, hormonal excess syndromes can have a greater effect on patients' morbidity and mortality than the tumor mass itself. We present the case of a 49-year-old male patient affected by an oligo-metastatic ileal NEN, concurrently demonstrating vasointestinal peptide (VIP) and serotonin excretion, complicated by pulmonary tuberculosis. After the first cycle of Lutetium-177-DOTATATE peptide-radio-receptor therapy, the patient developed a severe watery diarrhea, hypokalemia, and achlorhydria (WDHA) syndrome, despite receiving high-dose somatostatin analogues, everolimus, and telotristat ethyl, without any surgical options. The WDHA syndrome necessitated intensive-care-unit (ICU) admission with continual intravenous administration of electrolytes and fluids. With limited alternatives, an off-label intervention using the enkephalinase inhibitor racecadotril was initiated. After 5 days of treatment, the WDHA syndrome exhibited sufficient control, facilitating the patient's discharge from the ICU. This case report underscores racecadotril as an individualized, off-label treatment strategy for patients with severe VIPoma and serotonin-driven WDHA syndrome, where conventional therapeutic avenues have been exhausted. [ABSTRACT FROM AUTHOR]

We present a rare case of a 17-month-old child diagnosed with MS-stage neuroblastoma and associated chronic diarrhea due to elevated vasoactive intestinal peptide (VIP) levels. The unusual occurrence of a VIP-secreting tumor in a patient with MS neuroblastoma is a rare combination, not previously reported in the literature. The patient underwent exploratory laparotomy and excision of the tumor, leading to a significant decline in VIP levels and resolution of symptoms.

Abstract Functioning pancreatic neuroendocrine neoplasms other than insulinomas and gastrinomas (rf-pNENs) are exceptionally rare tumours. Thus, their characteristics and long-term prognosis have not been well defined. This article aims to present data and experience from a single institution concerning this topic. Twelve of 216 (5.5%) patients with pNENs operated between 2002 and 2022 in the ENETS Centre of Excellence Marburg had rf-pNENs and their data were retrospectively analysed. We identified three vasoactive intestinal polypeptide producing pNENs, four glucagonomas and five calcitoninomas. The tumour could be visualised by preoperative imaging in all 12 patients, and six patients had distant metastases at the time of diagnosis. The tumour was located in the pancreatic tail in nine patients and the median tumour size was 82 (range 12–220) mm. Eleven patients underwent tumour resections (two robotic, nine conventional), nine of which were R0. After a median follow-up of 75 (range 1–247) months, six patients were alive, five of whom had no evidence of disease. All patients who remained disease-free had an initial R0 resection of the primary tumour and no initial liver involvement. This study sheds light on the distinct characteristics and outcomes of these exceedingly rare tumours, offering insights for improved understanding and management.

Functioning pancreatic neuroendocrine neoplasms other than insulinomas and gastrinomas (rf-pNENs) are exceptionally rare tumours. Thus, their characteristics and long-term prognosis have not been well defined. This article aims to present data and experience from a single institution concerning this topic. Twelve of 216 (5.5%) patients with pNENs operated between 2002 and 2022 in the ENETS Centre of Excellence Marburg had rf-pNENs and their data were retrospectively analysed. We identified three vasoactive intestinal polypeptide producing pNENs, four glucagonomas and five calcitoninomas. The tumour could be visualised by preoperative imaging in all 12 patients, and six patients had distant metastases at the time of diagnosis. The tumour was located in the pancreatic tail in nine patients and the median tumour size was 82 (range 12–220) mm. Eleven patients underwent tumour resections (two robotic, nine conventional), nine of which were R0. After a median follow-up of 75 (range 1–247) months, six patients were alive, five of whom had no evidence of disease. All patients who remained disease-free had an initial R0 resection of the primary tumour and no initial liver involvement. This study sheds light on the distinct characteristics and outcomes of these exceedingly rare tumours, offering insights for improved understanding and management. [ABSTRACT FROM AUTHOR]

ABSTRACT: We present a rare case of a 17-month-old child diagnosed with MS-stage neuroblastoma and associated chronic diarrhea due to elevated vasoactive intestinal peptide (VIP) levels. The unusual occurrence of a VIP-secreting tumor in a patient with MS neuroblastoma is a rare combination, not previously reported in the literature. The patient underwent exploratory laparotomy and excision of the tumor, leading to a significant decline in VIP levels and resolution of symptoms. [ABSTRACT FROM AUTHOR]

Neuroendocrine tumors (NETs) are a heterogeneous group of tumors originating from cells of the dispersed endocrine system. They vary in their degree of differentiation and malignancy. They are primarily located in the gastrointestinal tract, pancreas, and lungs. This article will present the most common pancreatic neuroendocrine tumors, their specific characteristics, diagnostic methods including imaging diagnostics, and treatment options. NETs account for approximately 0.5% of all malignant tumors. Pancreatic neuroendocrine tumors include insulinoma, gastrinoma, glucagonoma, vipoma, and somatostatinoma. They secrete hormones specific to each type, chromogranins, and pancreatic polypeptide. However, only some of these tumors have a secretory function. They are usually well-differentiated tumors. Neuroendocrine tumors are difficult to diagnose due to their specific nature. They are typically diagnosed at an advanced stage, except for insulinoma. The most commonly used imaging methods are CT, MRI, PET, and SPECT. Treatment is extremely challenging due to symptoms caused by excessive hormone secretion. Usually, the first step in treatment is to alleviate symptoms. The only curative option is the removal of the tumor and its metastases. Unfortunately, this option is not available for many patients due to the late detection of the disease. Currently, many methods are used to slow the progression of tumors, including peptide receptor radionuclide therapy, mTOR inhibitors (everolimus), sunitinib, and chemotherapy. Detection and treatment are extremely difficult due to the specific nature of this group of tumors.

Cyclic nucleotide elevation in intestinal epithelial cells is the key pathology causing intestinal fluid loss in secretory diarrheas such as cholera. Current secretory diarrhea treatment is primarily supportive, and oral rehydration solution is the mainstay of cholera treatment. There is an unmet need for safe, simple and effective diarrhea treatments. By promoting cAMP hydrolysis, extracellular calcium-sensing receptor (CaSR) is a regulator of intestinal fluid transport. We studied the antidiarrheal mechanisms of FDA-approved CaSR activator cinacalcet and tested its efficacy in clinically relevant human cell, mouse and intestinal organoid models of secretory diarrhea. By using selective inhibitors, we found that cAMP agonists-induced secretory short-circuit currents (I sc) in human intestinal T84 cells are mediated by collective actions of apical membrane cystic fibrosis transmembrane conductance regulator (CFTR) and Clc-2 Cl− channels, and basolateral membrane K+ channels. 30 μM cinacalcet pretreatment inhibited all 3 components of forskolin and cholera toxin-induced secretory I sc by ∼75%. In mouse jejunal mucosa, cinacalcet inhibited forskolin-induced secretory I sc by ∼60% in wild type mice, with no antisecretory effect in intestinal epithelia-specific Casr knockout mice (Casr-flox; Vil1-cre). In suckling mouse model of cholera induced by oral cholera toxin, single dose (30 mg/kg) oral cinacalcet treatment reduced intestinal fluid accumulation by ∼55% at 20 hours. Lastly, cinacalcet inhibited forskolin-induced secretory I sc by ∼75% in human colonic and ileal organoids. Our findings suggest that CaSR activator cinacalcet has antidiarrheal efficacy in distinct human cell, organoid and mouse models of secretory diarrhea. Considering its excellent clinical safety profile, cinacalcet can be repurposed as a treatment for cyclic nucleotide-mediated secretory diarrheas including cholera. [ABSTRACT FROM AUTHOR]

Introdução/Justificativa: Os Vipomas são tumores neuroendócrinos raros do pâncreas, caracterizados pela produção excessiva de peptídeo intestinal vasoativo (VIP), levando a diarreia aquosa, desequilíbrios eletrolíticos, desidratação e consequentes graves complicações potenciais. O tratamento convencional envolve a terapia com análogos de somatostatina e cirurgias. No entanto, a disponibilidade irregular de medicamentos pode comprometer o controle da doença. Relato: Um homem de 56 anos apresentou-se com diarreia intensa, perda de peso significativa e desidratação. Exames revelaram hipocalemia grave, elevação de enzimas pancreáticas e escórias nitrogenadas renais. O tratamento de urgência envolveu terapia renal substitutiva, reposição eletrolítica, com recuperação completa do quadro clínico. A tomografia computadorizada (TC) de abdome total evidenciou um tumor neuroendócrino em cauda pancreática medindo 4 centímetros com metástases hepáticas, linfonodais e peritoneais. Iniciou-se tratamento empírico com Octreotida de liberação rápida, resultando em melhora sintomática significativa. A investigação complementar revelou dosagem de VIP 79,5 pmol/l (valor de referência até 30 pmol/l), ressonância de sela túrcica e dosagem de paratormônio normais. A cirurgia de debulking foi realizada para diagnóstico e citoredução tumoral, confirmando o diagnóstico de tumor neuroendócrino grau 2 com extensão para órgãos adjacentes. Após irregularidade do fornecimento do análogo de somatostatina de longa duração, o paciente retornou a apresentar sintomatologia com risco elevado de complicações agudas. Optou-se pela terapia teranóstica com Octreotato-Dota-177Lutécio (Lu) devido a sua eficácia comprovada para terapia de tumores neuroendócrinos gastroenteropancreáticos bem diferenciados com expressão comprovada para receptores de somatostatina. O paciente permaneceu em uso regular mensal de análogo de somatostatina durante cinco anos. Desde então continua assintomático, últimas tomografias realizadas sem evidência de recidiva ou de doença mensurável. Conclusão: O tratamento com Octreotato-Dota-177Lu em um paciente com vipoma metastático demonstrou eficácia na ausência de acesso regular a análogos de somatostatina de longa duração. Este caso destaca a importância de alternativas terapêuticas em contextos em que a disponibilidade de medicamentos é limitada, resultando em controle da doença e melhora significativa na qualidade de vida do paciente. A terapia com Octreotato-Dota-177Lu mostrou-se uma opção viável e eficaz em casos semelhantes, merecendo consideração em pacientes com dificuldades de acesso a tratamentos convencionais.

This case report presents a 40-year-old patient with a vasoactive intestinal peptide (VIP) secreting high grade (Ki-67 39%) neuroendocrine tumor (NET) from the pancreas, for whom successful liver transplantation (LT) was carried out 8 years after resection of the primary tumor due to massive liver metastases. The transplantation was done as rescue therapy due to rapid progression and a devastating clinical condition requiring intravenous supplementation for 20 hours daily. The latest imaging carried out 18 months after transplantation is without signs of recurrence, and the patient is in good health with undetectable levels of VIP. According to the guidelines, LT is only recommended if Ki-67 is <20% and if there has been tumor control for more than 6 months prior to transplantation. Our case illustrates that LT is an option that should be considered for selected NET patients without extrahepatic involvement regardless of tumor grade and clinical condition. [ABSTRACT FROM AUTHOR]

This ENETS guidance paper aims to provide practical advice to clinicians for the diagnosis, treatment and follow‐up of functioning syndromes in pancreatic neuroendocrine tumours (NET). A NET‐associated functioning syndrome is defined by the presence of a clinical syndrome combined with biochemical evidence of inappropriately elevated hormonal levels. Different hormonal syndromes can be encountered in pancreatic NET patients, including insulinoma, gastrinoma as well as the rare glucagonoma, VIPoma, ACTHoma, PTHrPoma, carcinoid syndrome, calcitoninoma, GHRHoma and somatostatinoma. The recommendations provided in this paper focus on the biochemical, genetic and imaging work‐up as well as therapeutic management of the individual hormonal syndromes in well‐differentiated, grade 1‐3, functioning NET with the primary tumour originating in the pancreas, and for specific subtypes also in the duodenum. [ABSTRACT FROM AUTHOR]

Functional neuroendocrine neoplasms (NENs) are those associated with specific symptoms related to the hormonal secretion of the NENs. Although less than 25 % of NENs are functional at diagnosis, 1 the associated syndromes significantly increase the patient burden of disease. Management of hormonal NEN symptoms may involve tumor resection or other reduction strategies (e.g., chemotherapy, embolotherapy, etc), but also specific therapies directed at decreasing hormonal synthesis, secretion, or end-organ effects. In this review, we focus on specific symptomatic management of many of the NEN syndromes, which may be pursued in addition to management primarily directed at tumor bulk and growth. A continued focus on symptom management related to the hormonal secretions of NENs, in the context of other efforts to reduce tumor bulk and growth, could significantly improve patient wellbeing. [ABSTRACT FROM AUTHOR]

Vasoactive intestinal peptide (VIP) secreting tumors (VIPomas) are insidious functional neuroendocrine tumors originating mainly from pancreatic islet cells. Hepatic localization is considered exceedingly rare as only few cases have been reported in the literature. Diagnostic and therapeutic management of this tumor is still not clearly codified and therefore represents a real challenge for clinicians. Herein we report a unique case of a primary hepatic VIPoma recurrence in a female patient 22 years after curative resection. The patient had two sessions of transarterial chemoembolization. Complete symptomatic improvement was achieved since the first day after the first session. This case highlights that long-term follow-up for patients with hepatic VIPoma is mandatory as recurrence could occur several years after curative surgical treatment.

Purpose: VIPoma belongs to the group of neuroendocrine neoplasms. These tumours are located mostly in the pancreas and produce high levels of vasoactive intestinal peptide (VIP). In most cases, a metastatic state has already been reached at the initial diagnosis, with high levels of VIP leading to a wide spectrum of presenting symptoms. These symptoms include intense diarrhoea and subsequent hypopotassaemia but also cardiac complications, with life-threatening consequences. Treatment options include symptomatic therapy, systemic chemotherapy and targeted therapy, as well as radiation and surgery. Due to the low incidence of VIPoma, there are no prospective studies or evidence-based therapeutic standards to date. Methods: To evaluate the possible impact of different therapy strategies, we performed literature research using PubMed. Results: All possible treatment modalities for VIPoma have at least one of two therapy goals: antisecretory effects (symptom control) and antitumoural effects (tumour burden reduction). Symptomatic therapy is the most important in the emergency setting to rehydrate, balance electrolytes and stabilise the patient. Symptomatic therapy is also of great importance perioperatively. Somatostatin analogues play a major role in symptom control, although their efficiency is often limited. Chemotherapy may be effective in reaching stable disease for a certain time period, although its impact on symptom control is limited and often delayed. Among targeted therapy options, the usage of sunitinib appears to be the most effective in terms of symptom control and showing antitumoural effects at the same time. Experience with radiation is still limited; however, local ablative procedures seem to be promising options. Peptide receptor radiotherapy (PRRT) with radiolabelled somatostatin analogues (SSAs, 177Lu-DOTATATE) offers a targeted approach, especially in patients with high somatostatin receptor density. Surgery is the first-line therapy for nonmetastatic VIPoma. Additionally, if the resection of all visible tumour lesions is possible, the surgical approach seems preferable to other strategies in highly symptomatic patients. The role of surgery in very advanced stages where only tumour debulking is possible remains debatable. However, a high rate of immediate symptom control can be achieved by tumour debulking followed by somatostatin therapy, although the impact on survival remains unclear. Conclusion: Surgery is the only curative option for nonmetastatic VIPoma. Additionally, surgery should be a first-line therapy option for highly symptomatic patients, especially if the resection of all tumour lesions (primary tumour and metastasis) is achievable. In frail patients, other modalities can be used. [ABSTRACT FROM AUTHOR]

Abstract Background Neuroendocrine tumors are rare, heterogeneous neoplasms that produce a wide variety of clinical symptoms. Diarrhea in neuroendocrine tumors is incredibly common and is usually benign in nature. We report two extreme cases of diarrhea in metastatic neuroendocrine tumors that threatened fatality and provide evidence for steroids as a novel agent in the management of vasoactive intestinal peptide tumors. Case presentation A 63-year-old Caucasian male with a grade 2 (Ki-67 17%) metastatic small bowel neuroendocrine tumor, and a 43-year-old female with a grade 2 (Ki-67 5%) metastatic pancreatic vasoactive intestinal peptide tumor. Both patients suffered life-threatening diarrhea despite extensive treatment modalities, including new systemic agents. This case explains how a lack of compliance and patient under-reporting of symptoms contributed to their challenging clinical course. Only steroids had a significant sustained effect on the diarrhea of the patient with vasoactive intestinal peptide tumor. Conclusions This report discusses two rare cases of life-threatening diarrhea in neuroendocrine tumors and stresses the importance of accurate clinical history taking, patient education, and compliance for symptom control. The report suggests steroids as a potential novel pharmaceutical option in the management of vasoactive intestinal peptide tumors; this is of great significance as it may provide a new approach to their management and potentially act as a life-saving agent in other oncology patients.

A 35‐year‐old man with known human immunodeficiency virus experienced chronic diarrhoea for 18 months. He presented to multiple hospitals with profuse secretory diarrhoea and life‐threatening electrolyte disturbances. Infectious and non‐infectious aetiologies were considered, with focussed history and investigations ultimately leading to a diagnosis of VIPoma. Initiation of somatostatin analogue therapy followed by surgical resection led to complete resolution of symptoms and markedly improved quality of life. [ABSTRACT FROM AUTHOR]

Neuroblastic tumors are the most common malignant extracranial solid tumors of childhood. A small subgroup presents chronic incoercible diarrhea due to the tumor's production of vasoactive intestinal peptide (VIP). The hypothesis of an occult tumor is not always considered, which delays and impairs treatment. We aim to identify these patients' characteristics and help alert health professionals to the hypothesis of a neuroblastic tumor in children with chronic diarrhea refractory to the usual approach. We carried out an epidemiological study on all retrievable reports of neuroblastic tumors between 1975 and 2021 described in the Medical Literature Analysis and Retrieval System Online (MEDLINE), Excerpta Medica database (EMBASE), and Latin American & Caribbean Health Sciences Literature (LILACS) databases. Patient information was divided into categories, and we performed a descriptive analysis. We analyzed 96 cases; 83 (86.5%) cases had diarrhea prior to the diagnosis of the neoplasm, 49 (51%) were ganglioneuroblastomas, 69 (71.8%) were abdominal, and 59 of the 60 patients (98%) with reported acid-base disorders had hypokalemia. When serum VIP was reported, the majority of values varied between one and 20 times the upper reference limit. Seventy-two (75%) patients underwent complete tumor resection, and the overall survival rate was 70%. Serum VIP production by neuroblastic tumors is related to cell differentiation and better prognosis. Such children often require intensive hospital support to reverse the malnutrition and acid-base disorders related to this paraneoplastic syndrome. Its early diagnosis and treatment significantly change the prognosis and quality of life. We, therefore, suggest screening for neuroblastic tumors when health professionals encounter unmanageable chronic secretory diarrhea in children with no defined etiology in the usual investigations., Competing Interests: Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Dornelles Penteado Pacheco e Silva et al.)

Vasoactive intestinal peptide-secreting tumor (VIPoma) is a very rare, life-threatening, functioning pancreatic neuroendocrine tumor (pNET). The efficacy of antitumor therapies against functioning symptoms and tumor burden have been poorly described in VIPoma. Describe the impact of treatments on the secretory syndrome, tumor burden and survival in patients with VIPoma. We retrospectively reviewed the records of patients with VIPoma treated in seven French expert centers between 1990 and 2016. Diagnostic of VIPoma was reassessed using strict criteria. We evaluated the antisecretory efficacy (>50 % decrease of daily bowel movements), and antitumor efficacy (RECIST 1.1) of all treatments received. Twenty-two patients were included. pNETs were mostly metastatic (77 %) and classified as grade 2 (83 %). Median follow-up was 78.2 months. Surgical excision of nonmetastatic VIPoma effectively controlled the secretory syndrome. Although 4/5 patients had metastatic recurrences, all patients were alive after median post-operative follow-up of 171 months. Among the 87 treatments received for metastatic VIPoma, curative-intent surgery (n = 14), somatostatin analogs alone (n = 11), chemotherapy (n = 23), transarterial liver embolization (TALE) (n = 14), everolimus (n = 10) and sunitinib (n = 7) achieved, respectively, 100 %, 67 %, 83 %, 50 %, 20 % and 100 % antisecretory efficacy. The 5-year OS rate was 63.6 %, with pejorative impact of higher Ki-67 index (P = 0.045) and higher plasma VIP concentration (P = 0.025). Surgical resection of localized VIPoma is effective but rarely curative. For metastatic VIPoma, curative-intent surgery, chemotherapy and sunitinib are the therapeutic options that best combined antitumor and antisecretory efficacies. [ABSTRACT FROM AUTHOR]

Background: Neuroendocrine tumors are rare, heterogeneous neoplasms that produce a wide variety of clinical symptoms. Diarrhea in neuroendocrine tumors is incredibly common and is usually benign in nature. We report two extreme cases of diarrhea in metastatic neuroendocrine tumors that threatened fatality and provide evidence for steroids as a novel agent in the management of vasoactive intestinal peptide tumors.Case Presentation: A 63-year-old Caucasian male with a grade 2 (Ki-67 17%) metastatic small bowel neuroendocrine tumor, and a 43-year-old female with a grade 2 (Ki-67 5%) metastatic pancreatic vasoactive intestinal peptide tumor. Both patients suffered life-threatening diarrhea despite extensive treatment modalities, including new systemic agents. This case explains how a lack of compliance and patient under-reporting of symptoms contributed to their challenging clinical course. Only steroids had a significant sustained effect on the diarrhea of the patient with vasoactive intestinal peptide tumor.Conclusions: This report discusses two rare cases of life-threatening diarrhea in neuroendocrine tumors and stresses the importance of accurate clinical history taking, patient education, and compliance for symptom control. The report suggests steroids as a potential novel pharmaceutical option in the management of vasoactive intestinal peptide tumors; this is of great significance as it may provide a new approach to their management and potentially act as a life-saving agent in other oncology patients. [ABSTRACT FROM AUTHOR]

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Vasoactive intestinal polypeptide-secreting tumors (VIPoma) are a rare pancreatic neuroendocrine tumor that can cause chronic diarrhea with 1 case per 10 million people per year. Diagnosis is made based on a combination of laboratory evaluation (serum VIP level), imaging findings (functional positron emission tomography-computed tomography [PET-CT]), and histological analysis (chromogranin A stain). We present a case of a male with 6 months of diarrhea and malaise who was found to have significant kidney injury and hypokalemia requiring admission to the medical intensive care unit. Subsequent laboratory evaluation while admitted eventually showed a low stool osmotic gap (–11 mOsm/kg) consistent with secretory diarrhea, in addition to significantly elevated VIP levels at 940 pg/mL (normal

Introduction: VIPoma is a neuroendocrine tumor that secrets vasoactive intestinal peptide and produces a well-defined clinical syndrome characterized by watery diarrhea, hypokalemia, hypochlorhydria and metabolic acidosis. The aim of this study to investigate clinical studies about diagnostic and therapeutic modalities of vipoma patients. In this retrospective study, all patients of vipoma were investigated. Clinical features, laboratory data at initial presentation, management and outcomes were collected. Subjects and Methods: The paper has written based on searching PubMed and Google Scholar to identify potentially relevant articles or abstracts. Categorical variables as percentage and continuous variables were reported as mean ± standard deviation (SD). Results: All the patients presented with watery diarrhea (30/30, 100%) and dehydration was reported in 33.3% of them. Prevalence of laboratory findings in these patients were assessed for hypokalaemia (25/30, 83.3%), metabolic acidosis (9/30, 33.6%), hypochloremia and achlorhydria (2/30, 6.6%). Elevated VIP levels have been seen in 73.3% patients with mean values of 882.85 ± 1134.87 pg/ml. Prevalence of diagnostic methods included CT scan in 19 patients (19/30, 63.3%), ultrasonography (15/50, 50%), and somatostatin receptor scintigraphy (8/30, 26.6%). Medical treatments included somatostatin and analogues in 18 patients (18/30, 60%). Surgery included less percentage of treatment in these patients. Conclusion: CT scan can be used as a reliable modality for diagnosis of vipoma and somatostatin analogues can be used as the most effective treatment in vipoma patients. Surveillance of these patients needs to close monitoring of patients via history, physical examination, laboratory and imaging.

Introduction: Octreotide acetate subcutaneous injection is indicated to treat acromegaly and the symptoms of carcinoid tumors and vasoactive intestinal peptide tumors (VIPomas). This formative human factors study assessed the octreotide acetate pen injector and accompanying instructions for use (IFU) with self-trained participants.Methods: The study enrolled patients with diagnoses of acromegaly, carcinoid tumors, or VIPomas and healthcare practitioners (HCPs) who treat patients with these diagnoses. The IFU provided a stepwise process with illustrations to train participants on using the pen injector. Participants familiarized themselves with the pen injector and the IFU before administering 2 unaided injections into skin-like pads; administering the full dose into the pad was considered a successful injection. The investigators evaluated each injection by performance measures-specific tasks necessary to safely and correctly administer the medication-and subjective measures, which included participant comments, feedback from questions, and suggestions for improvements.Results: The study enrolled 11 participants-8 patients and 3 HCPs. Participants had a success rate of 100% for both injections. Errors included 1 participant priming the pen with the incorrect dose and 2 participants not holding the injector button for 10 s after the injection. Neither error led to a failed injection. To improve the IFU, participants suggested changing the order of wording on the priming step, clarifying illustrations of the plunger, and stronger indications to hold the injector button.Conclusion: The octreotide pen injector and IFU were usable by self-trained participants. Participant errors and suggestions provided a foundation for recommendations to improve the IFU. [ABSTRACT FROM AUTHOR]

Final diagnosis VIPoma, also termed pancreatic cholera, Verner-Morrison syndrome or WDHH (watery diarrhea, hypokalemia, hypochlorhydria) syndrome due to VIP-expressing tumor tissue in the liver with unknown primary but most likely originating from neuroendocrine differentiation in posttreatment prostate cancer. Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID Study Group. Keywords: VIPoma; Pancreatic cholera; Verner-Morrison syndrome; WDHH (watery diarrhea, hypokalemia, hypochlorhydria) syndrome; Hypokalemia EN VIPoma Pancreatic cholera Verner-Morrison syndrome WDHH (watery diarrhea, hypokalemia, hypochlorhydria) syndrome Hypokalemia 515 522 8 05/17/21 20210501 NES 210501 Information Information on Clinical-Pathological Conferences from the Medical University of Graz are also available under https://www.medunigraz.at/cpc-klinischpathologischekonferenz/ (in German). One of the key symptoms in the discussed patient is a 2-month history of watery diarrhea (4-6 weeks of diarrhea is defined as chronic diarrhea), which may be due to osmotic, secretory, functional or inflammatory causes; however, since endoscopy with biopsies was unremarkable in the discussed patient diagnoses, such as celiac disease, microscopic colitis, inflammatory bowel disease and eosinophilic enteritis can be ruled out. [Extracted from the article]

Introduction: Vasoactive intestinal peptide-secreting tumor (VIPoma) is a rare functional pancreatic neuroendocrine tumor (F-PNET) characterized by secretory diarrhea, hypokalemia, and hypochlorhydria. Its low incidence and high risk of malignancy pose a clinical challenge that requires a high degree of clinical suspicion. Case presentation: A 61-year-old woman visited the emergency department of a tertiary care hospital in Medellín, Colombia, due to chronic diarrhea (7 months) that led to dehydration, renal failure, metabolic acidosis, and hypokalemia. As a result, a treatment based on loperamide, intravenous fluids and broad-spectrum antibiotics was started. In addition, chromogranin A levels of 477 ug/L (

We present the case of a 73-year-old female who underwent percutaneous cryoablation for recurrent life-threatening pancreatic vasoactive intestinal polypeptide-producing tumor (VIPoma) following a pancreaticoduodenectomy and chemotherapy 5 years earlier. She presented with profuse watery diarrhea causing severe electrolyte and acid–base abnormalities, along with acute kidney injury. Cryoablation was successful in treating her profound symptoms, completely reversing her clinical course. The patient has made a successful recovery for the last 1.5 years since the procedure. [ABSTRACT FROM AUTHOR]

Vasoactive intestinal peptide secreting tumor (VIPoma) is a rare mostly malignant neuroendocrine tumor that is characterized by watery diarrhea, hypokalemia and achlorhydria due to the nonregulated increased secretion of VIP. VIPomas ar diagnosed by the presence of the most common symptoms, laboratory analysis of blood and stool, radiological imaging and immunohistochemical findings. Primary treatment includes fluid replacement, electrolyte balance correction, pharmacological treatment with somatostatin analogs, surgical resection and chemotherapy. This review aims to provide an insight into the latest research on VIPoma epidemiology, pathophysiology, diagnostics and treatment., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Las neoplasias neuroendocrinas (Neuroendocrine Neoplasms, NEN) constituyen un grupo heterogéneo de neoplasias poco frecuentes, que se originan en las células endocrinas, con la capacidad de secretar aminas y polipéptidos hormonales. Las NEN de localización pancreática (pNEN) pueden ser funcionales o no funcionales. Las pNEN funcionales secretan hormonas como la gastrina, la insulina y el glucagón y otras menos frecuentes como el péptido intestinal vasoactivo (PIV), por lo que sus características sindromáticas dependen del péptido secretado. Los vipomas se manifiestan con diarrea crónica de características secretoras, que usualmente conducen a trastornos hidroelectrolíticos e incluso a complicaciones serias asociadas como la falla renal. A continuación, se describe el caso de un hombre de 37 años con diarrea crónica de 6 meses de evolución y frecuentes hospitalizaciones por trastornos hidroelectrolíticos, generados por hipocalemia severa y lesión renal aguda por deshidratación. Después de múltiples estudios, se considera el diagnóstico de una diarrea secretora por NEN funcional, secretora de PIV. Por tanto, se inicia una terapia empírica con octreotida y se logra controlar la diarrea, así como corregir el trastorno hidroelectrolítico. Además, se amplían los estudios, para documentar las pNEN tratadas mediante intervención quirúrgica, con respuesta clínica favorable y remisión completa de la sintomatología.

VIPoma is an unusual neuroendocrine neoplasm that autonomously secretes VIP. It is associated with secretory diarrhea and electrolyte disturbances. Herein we report a case of a male patient, who was hospitalized in the Department of Endocrinology in Hedi Chaker Hospital, Sfax, Tunisia. He presented VIPoma syndrome, with hepatic metastases at diagnosis. He had a history of chronic, watery diarrhea. He was dehydrated with many electrolytic disorders as hypokalemia, hyponatremia and metabolic acidosis. Abdominal CT scan showed a heterogeneous mass in the pancreatic head with multiple hepatic lesions. A high VIP hormone level was found. Histological study of a liver biopsy revealed hepatic metastases of neuroendocrine carcinoma. The patient received analogues of somatostatin and systemic chemotherapy, with a transient symptomatic relief. Sadly the patient was lost to follow-up.

Neuroendocrine tumors (NETs) are a heterogeneous group of rare neoplasms that originate in endocrine cells with the ability to secrete amines and hormonal polypeptides. Pancreatic neuroendocrine tumors (PNETs) can be functional or non-functional. Functional PNETs secrete common hormones such as gastrin, insulin and glucagon and much less frequent hormones such as vasoactive intestinal peptide (VIP). Their characteristics depend on the peptide secreted. Vipomas are characterized by chronic diarrhea of secretory characteristics that usually lead to hydroelectrolytic disorders and can lead to serious complications associated with renal failure. This article describes the case of a 37-year-old man who had suffered chronic diarrhea with frequent hospitalization for hydroelectrolytic disorders for six months due to severe hypokalemia and acute renal damage due to dehydration. After multiple studies, a diagnosis of secretory diarrhea due to a VIP secretory functional NET was considered. Empirical therapy with Octreotide was begun to control diarrhea and correct the hydroelectrolytic disorder. More studies of PNETS are being published. They have been treated surgically intervention with favorable clinical results and complete remission of symptoms. [ABSTRACT FROM AUTHOR]