{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:25Z","timestamp":1784557045375,"version":"3.55.0"},"reference-count":19,"publisher":"Bioscientifica","license":[{"start":{"date-parts":[[2022,8,1]],"date-time":"2022-08-01T00:00:00Z","timestamp":1659312000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["edm.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,8,1]]},"abstract":"<jats:sec>\n                    <jats:title>Summary<\/jats:title>\n                    <jats:p>Vasoactive intestinal peptide-secreting tumours (VIPomas) are an extremely rare form of functional pancreatic neuroendocrine tumour with an estimated annual incidence of 1 in 10 million. Associated tumour hypersecretion of other peptides, including pancreatic polypeptide (PPomas), may also be seen. These malignancies classically present with a defined triad of refractory diarrhoea, hypokalaemia and metabolic acidosis known as Verner\u2013Morrison syndrome. Diagnosis is frequently delayed, and the majority of patients will have metastatic disease at presentation. Symptoms are usually well controlled with somatostatin analogue administration. Here we report a case of metastatic mixed VIPoma\/PPoma-induced diarrhoea causing renal failure so severe that ultrafiltration was required to recover adequate renal function.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Learning points<\/jats:title>\n                    <jats:list list-type=\"bullet\">\n                      <jats:list-item>\n                        <jats:p>Profuse, watery diarrhoea is a common presenting complaint with a multitude of aetiologies. This, combined with the rarity of these tumours, makes diagnosis difficult and frequently delayed. A functional neuroendocrine tumour should be suspected when diarrhoea is unusually extreme, prolonged and common causes have been promptly excluded.<\/jats:p>\n                      <\/jats:list-item>\n                      <jats:list-item>\n                        <jats:p>These patients are likely to be profoundly unwell on presentation. They are extremely hypovolaemic with dangerous electrolyte and metabolic abnormalities. Aggressive initial rehydration and electrolyte replacement are imperative. A somatostatin analogue should be commenced as soon as the diagnosis is suspected.<\/jats:p>\n                      <\/jats:list-item>\n                      <jats:list-item>\n                        <jats:p>This is an extreme example of Verner\u2013Morrison syndrome. We are unaware of another case where renal failure secondary to diarrhoea and dehydration was so severe that renal replacement therapy was required to restore adequate renal function, further emphasising how critically unwell these patients can be.<\/jats:p>\n                      <\/jats:list-item>\n                      <jats:list-item>\n                        <jats:p>Both the primary tumour and metastases showed a remarkably good and rapid response to somatostatin analogue administration. Cystic change and involution were noted on repeat imaging within days.<\/jats:p>\n                      <\/jats:list-item>\n                      <jats:list-item>\n                        <jats:p>Prior to his illness, this patient was extremely high functioning with no medical history. His diagnosis was an enormous psychological shock, and the consideration and care for his psychological well-being were a crucial part of his overall management. It highlights the importance of a holistic approach to cancer care and the role of the clinical nurse specialist within the cancer multidisciplinary team.<\/jats:p>\n                      <\/jats:list-item>\n                    <\/jats:list>\n                  <\/jats:sec>","DOI":"10.1530\/edm-22-0231","type":"journal-article","created":{"date-parts":[[2022,8,24]],"date-time":"2022-08-24T10:06:14Z","timestamp":1661335574000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-2","source":"Crossref","is-referenced-by-count":3,"title":["Metastatic mixed VIPoma\/PPoma-induced diarrhoea causing renal failure"],"prefix":"10.1530","volume":"2022","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6325-824X","authenticated-orcid":true,"given":"George","family":"Brown","sequence":"first","affiliation":[{"name":"Department of Hepatobiliary & Pancreatic Surgery, University Hospital Southampton, Southampton, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony Mark","family":"Monaghan","sequence":"additional","affiliation":[{"name":"Department of Hepatobiliary & Pancreatic Surgery, University Hospital Southampton, Southampton, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Fristedt","sequence":"additional","affiliation":[{"name":"Department of Hepatobiliary & Pancreatic Surgery, University Hospital Southampton, Southampton, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma","family":"Ramsey","sequence":"additional","affiliation":[{"name":"Department of Hepatobiliary & Pancreatic Surgery, University Hospital Southampton, Southampton, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ma\u2019en","family":"Al-Mrayat","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, University Hospital Southampton, Southampton, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rushda","family":"Rajak","sequence":"additional","affiliation":[{"name":"Department of Cellular Pathology, University Hospital Southampton, Southampton, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Armstrong","sequence":"additional","affiliation":[{"name":"Department of Hepatobiliary & Pancreatic Surgery, University Hospital Southampton, Southampton, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arjun","family":"Takhar","sequence":"additional","affiliation":[{"name":"Department of Hepatobiliary & Pancreatic Surgery, University Hospital Southampton, Southampton, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"Intractable and Rare Diseases Research","doi-asserted-by":"publisher","first-page":"21","DOI":"10.5582\/irdr.2017.01007","article-title":"Pancreatic neuroendocrine tumors","volume":"6","author":"Sun","year":"2017","unstructured":"Sun JPancreatic neuroendocrine tumors. Intractable and Rare Diseases Research2017621\u201328. (https:\/\/doi.org\/10.5582\/irdr.2017.01007)10.5582\/irdr.2017.01007"},{"key":"bib2","series-title":"Endocrine-Related Cancer","doi-asserted-by":"publisher","first-page":"R423","DOI":"10.1530\/ERC-16-0200","article-title":"Medical management of secretory syndromes related to gastroenteropancreatic neuroendrocrine tumours","volume":"23","author":"Dimitriadis","year":"2016","unstructured":"Dimitriadis GKWeickert MORandeva HSKaltsas GGrossman A. Medical management of secretory syndromes related to gastroenteropancreatic neuroendrocrine tumours. Endocrine-Related Cancer201623R423\u2013R436. (https:\/\/doi.org\/10.1530\/ERC-16-0200)10.1530\/ERC-16-0200"},{"key":"bib3","series-title":"New England Journal of Medicine","doi-asserted-by":"publisher","first-page":"287","DOI":"10.1056\/NEJM198607313150504","article-title":"Secretion of pancreatic polypeptide in patients with pancreatic endocrine tumors","volume":"315","author":"Adrian","year":"1986","unstructured":"Adrian TEUttenthal LOWilliams SJBloom SR. Secretion of pancreatic polypeptide in patients with pancreatic endocrine tumors. New England Journal of Medicine1986315287\u2013291. (https:\/\/doi.org\/10.1056\/NEJM198607313150504)10.1056\/NEJM198607313150504"},{"key":"bib4","series-title":"American Journal of Medicine","doi-asserted-by":"publisher","first-page":"374","DOI":"10.1016\/0002-9343(58)90075-5","article-title":"Islet cell tumor and a syndrome of refractory watery diarrhoea and hypokalemia","volume":"25","author":"Verner","year":"1958","unstructured":"Verner JVMorrison AB. Islet cell tumor and a syndrome of refractory watery diarrhoea and hypokalemia. American Journal of Medicine195825374\u2013380. (https:\/\/doi.org\/10.1016\/0002-9343(5890075-5)10.1016\/0002-9343(58)90075-5"},{"key":"bib5","series-title":"Neuroendocrinology","doi-asserted-by":"publisher","first-page":"153","DOI":"10.1159\/000443171","article-title":"ENETS consensus guidelines update for the management of patients with functional pancreatic neuroendocrine tumors and non-functional pancreatic neuroendocrine tumors","volume":"103","author":"Falconi","year":"2016","unstructured":"Falconi MEriksson BKaltsas GBartsch DKCapdevila JCaplin MKos-Kudla BKwekkeboom DRindi GKl\u00f6ppel GENETS consensus guidelines update for the management of patients with functional pancreatic neuroendocrine tumors and non-functional pancreatic neuroendocrine tumors. Neuroendocrinology2016103153\u2013171. (https:\/\/doi.org\/10.1159\/000443171)10.1159\/000443171"},{"key":"bib6","series-title":"Pancreas","doi-asserted-by":"publisher","first-page":"87","DOI":"10.1097\/00006676-199201000-00012","article-title":"Are pancreatic vipomas paraneuron neoplasms? A clue to neuroectodermal origin of these tumors","volume":"7","author":"Bani Sacchi","year":"1992","unstructured":"Bani Sacchi TBani DBiliotti G. Are pancreatic vipomas paraneuron neoplasms? A clue to neuroectodermal origin of these tumors. Pancreas1992787\u201397. (https:\/\/doi.org\/10.1097\/00006676-199201000-00012)10.1097\/00006676-199201000-00012"},{"key":"bib7","series-title":"Journal of B.U.ON.","first-page":"415","article-title":"Clinicopathological data and treatment modalities for pancreatic vipomas: a systematic review","volume":"24","author":"Schizas","year":"2019","unstructured":"Schizas DMastoraki ABagias GPatras RMoris DLazaridis IIArkadopoulos NFelekouras E. Clinicopathological data and treatment modalities for pancreatic vipomas: a systematic review. Journal of B.U.ON.201924415\u2013423. (PMID: https:\/\/pubmed.ncbi.nlm.nih.gov\/31127985\/)"},{"key":"bib8","series-title":"Chinese Journal of Cancer","doi-asserted-by":"publisher","first-page":"312","DOI":"10.5732\/cjc.012.10295","article-title":"Pancreatic neuroendocrine tumors: biology, diagnosis and treatment","volume":"32","author":"Ro","year":"2013","unstructured":"Ro CChai WYu VEYu R. Pancreatic neuroendocrine tumors: biology, diagnosis and treatment. Chinese Journal of Cancer201332312\u2013324. (https:\/\/doi.org\/10.5732\/cjc.012.10295)10.5732\/cjc.012.10295"},{"key":"bib9","series-title":"Seminars in Oncology","first-page":"282","article-title":"Vipoma syndrome","volume":"14","author":"Mekhijian","year":"1987","unstructured":"Mekhijian HSO\u2019Dorisio TM. Vipoma syndrome. Seminars in Oncology198714282\u2013291. (https:\/\/pubmed.ncbi.nlm.nih.gov\/2820063\/)"},{"key":"bib10","series-title":"Hospital Practice","doi-asserted-by":"publisher","first-page":"109","DOI":"10.1080\/21548331.2016.1210474","article-title":"Pancreatic neuroendocrine tumors: contemporary diagnosis and management","volume":"44","author":"Parbhu","year":"2016","unstructured":"Parbhu SKAdler DG. Pancreatic neuroendocrine tumors: contemporary diagnosis and management. Hospital Practice201644109\u2013119. (https:\/\/doi.org\/10.1080\/21548331.2016.1210474)10.1080\/21548331.2016.1210474"},{"key":"bib11","series-title":"Journal of Gastrointestinal Surgery","doi-asserted-by":"publisher","first-page":"382","DOI":"10.1007\/s11605-007-0177-0","article-title":"Pancreatic vipomas: subject review and one institutional experience","volume":"12","author":"Ghaferi","year":"2008","unstructured":"Ghaferi AAChohnacki KALong WDCameron JLYeo CJ. Pancreatic vipomas: subject review and one institutional experience. Journal of Gastrointestinal Surgery200812382\u2013393. (https:\/\/doi.org\/10.1007\/s11605-007-0177-0)10.1007\/s11605-007-0177-0"},{"key":"bib12","series-title":"Regulatory Peptides","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1016\/0167-0115(84)90066-1","article-title":"Vasoactive intestinal peptide (VIP) in the pig pancreas: role of VIPergic nerves in the control of fluid and bicarbonate secretion","volume":"8","author":"Holst","year":"1984","unstructured":"Holst JJFahrenkrug JKnuhtsen SJensen SLPoulsen SSNielsen OV. Vasoactive intestinal peptide (VIP) in the pig pancreas: role of VIPergic nerves in the control of fluid and bicarbonate secretion. Regulatory Peptides19848245\u2013259. (https:\/\/doi.org\/10.1016\/0167-0115(8490066-1)10.1016\/0167-0115(84)90066-1"},{"key":"bib13","series-title":"Pancreas","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1097\/00006676-200401000-00015","article-title":"Diagnosis and treatment of vipoma in China: (case report and 31 cases review) diagnosis and treatment of vipoma","volume":"28","author":"Peng","year":"2004","unstructured":"Peng SYLi JTLiu YBFang HQWu YLPeng CHWang XBQian HR. Diagnosis and treatment of vipoma in China: (case report and 31 cases review) diagnosis and treatment of vipoma. Pancreas20042893\u201397. (https:\/\/doi.org\/10.1097\/00006676-200401000-00015)10.1097\/00006676-200401000-00015"},{"key":"bib14","series-title":"Frontiers in Endocrinology","article-title":"Frequency and causes of false-positive elevated plasma concentrations of fasting gut hormones in a specialist neuroendocrine tumor center","volume":"11","author":"Butler","year":"2020","unstructured":"Butler OLMekhael MMAhmed ACuthbertson DJPritchard DM. Frequency and causes of false-positive elevated plasma concentrations of fasting gut hormones in a specialist neuroendocrine tumor center. Frontiers in Endocrinology202011 606264. (https:\/\/doi.org\/10.3389\/fendo.2020.606264)"},{"key":"bib15","series-title":"International Journal of Gastrointestinal Cancer","doi-asserted-by":"publisher","first-page":"153","DOI":"10.1385\/IJGC:32:2-3:153","article-title":"Symptomatic pancreatic polypeptide-secreting tumor of the distal pancreas (PPoma)","volume":"32","author":"Mortenson","year":"2002","unstructured":"Mortenson MBold RJ. Symptomatic pancreatic polypeptide-secreting tumor of the distal pancreas (PPoma). International Journal of Gastrointestinal Cancer200232153\u2013156. (https:\/\/doi.org\/10.1385\/IJGC:32:2-3:153)10.1385\/IJGC:32:2-3:153"},{"key":"bib16","series-title":"World Journal of Surgery","doi-asserted-by":"publisher","first-page":"1815","DOI":"10.1007\/s00268-008-9499-7","article-title":"Sporadic pancreatic polypeptide secreting tumors (PPomas) of the pancreas","volume":"32","author":"Kuo","year":"2008","unstructured":"Kuo SCGananadha SScarlett CJGill ASmith RC. Sporadic pancreatic polypeptide secreting tumors (PPomas) of the pancreas. World Journal of Surgery2008321815\u20131822. (https:\/\/doi.org\/10.1007\/s00268-008-9499-7)10.1007\/s00268-008-9499-7"},{"key":"bib17","series-title":"Standards and Datasets for Reporting Cancers Dataset for Histopathological Reporting of Neuroendocrine Neoplasms of the Gastroenteropancreatic Tract","author":"Luong","year":"2019","unstructured":"Luong TWatkins JChakrabarty BWang L. Standards and Datasets for Reporting Cancers Dataset for Histopathological Reporting of Neuroendocrine Neoplasms of the Gastroenteropancreatic Tract. London: Royal College of Pathologists, 2019. (available at: https:\/\/www.rcpath.org\/profession\/guidelines\/cancer-datasets-and-tissue-pathways.html). Accessed on 12 May 2022."},{"key":"bib18","series-title":"WHO Classification of Tumours of Endrocrine Organs","author":"Lloyd","year":"2017","unstructured":"Lloyd ROsamura RKloppel GRosai JEds. WHO Classification of Tumours of Endrocrine Organs, 4th ed.Lyon, France: InternationalAgencyforResearchonCancer, 2017. (ISBN: 978-92-832-4493-6)"},{"key":"bib19","series-title":"Gastroenterology","doi-asserted-by":"publisher","first-page":"185","DOI":"10.1016\/S0016-5085(85)80153-0","article-title":"Longterm treatment of a VIPoma with somatostatin analogue resulting in remission of symptoms and possibly shrinkage of metastases","volume":"88","author":"Kraenzlin","year":"1985","unstructured":"Kraenzlin MCh\u2019ng JWood SCarr DBloom S. Longterm treatment of a VIPoma with somatostatin analogue resulting in remission of symptoms and possibly shrinkage of metastases. Gastroenterology198588185\u2013187. (https:\/\/doi.org\/10.1016\/s0016-5085(85)80153-0)10.1016\/S0016-5085(85)80153-0"}],"container-title":["Endocrinology, Diabetes &amp; Metabolism Case Reports"],"original-title":[],"link":[{"URL":"https:\/\/edm.bioscientifica.com\/view\/journals\/edm\/2022\/1\/EDM22-0231.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/edm.bioscientifica.com\/downloadpdf\/journals\/edm\/2022\/1\/EDM22-0231.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/edm.bioscientifica.com\/downloadpdf\/journals\/edm\/2022\/1\/EDM22-0231.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T13:47:54Z","timestamp":1784555274000},"score":1,"resource":{"primary":{"URL":"http:\/\/journals.bioscientifica.com\/edm\/article\/3522"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,1]]},"references-count":19,"URL":"https:\/\/doi.org\/10.1530\/edm-22-0231","relation":{},"ISSN":["2052-0573"],"issn-type":[{"value":"2052-0573","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,8,1]]}}}