Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia

Verner, John V.; Morrison, A. B.

doi:10.1016/0002-9343(58)90075-5

Cited by 579 publications

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“…At contrast-enhanced MR the lesion exhibited a mild and inhomogeneous enhancement in the arterial phase which could be appreciated despite the presence of motion artifacts ( Figure 3A) with a rim of peripheral enhancement in the delayed phase ( Figure 3B). Although similar enhancement patterns can also be observed in other neoplastic pancreatic lesions such as the acinar cell carcinoma [15] and the solid pseudopapillary tumor [16] , MR findings were finally considered consistent with the diagnosis of a pancreatic vipoma as the patient had the typical symptoms of the Verner-Morison syndrome [2] and most vipomas are indeed localized at the level of the pancreatic tail [7] . As the tumor was prospectively missed on contrastenhanced multi-detector CT, the present case underscores the relevance of a multidisciplinary approach in the detection of pancreatic neuro-endocrine tumors and the superior contrast resolution of MR which turns out to be particularly helpful whenever the administration of iodinated contrast media is either contraindicated or suboptimal [17] .…”

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confidence: 80%

“…They may show a malignant behaviour in up to 70% of cases, mostly with evidence of hepatic metastases at the time of the diagnosis [1] . From a clinical point of view the diagnosis of a pancreatic VIPoma is straightforward in typical cases presenting with the Verner-Morrison triad first described in 1958 [2] . The syndrome, also named WDHA (Watery Diarrhoea, Hypokalaemia, Achlorhydria), is caused by the excessive production of the Vasoactive Intestinal Peptide (VIP) which stimulates fluid and electrolyte secretion in the intestinal epithelium through the activation of cyclic adenosine mono-phosphate pathway [3] .…”

Section: Introductionmentioning

confidence: 99%

“…Up to 30%-50% of PNENs are functioning neoplasms which manifest with typical clinical syndromes with insulinomas and gastrinomas being the most common followed by glucagonomas, somatostatinomas and vipomas [1] . These latter represent only 2% of functional PNENs and usually manifest with the classic WDHA syndrome first described by Verner et al [2] in 1958. Such was the case of our patient who had a two years history of watery diarrhea which was thought to be related to an infective or a parasitic disease.…”

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Contrast enhanced multi-detector CT and MR findings of a well-differentiated pancreatic vipoma

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Core tip: Pancreatic vipoma is an extremely rare tumor accounting for less than 2% of endocrine pancreatic neoplasms. While cross-sectional imaging findings are usually not specific, exact localization of the tumor by means of either computed tomography (CT) or magnetic resonance (MR) is pivotal for surgical planning. We report the case of a 70 years old female with a two years history of watery diarrhoea who was found to have a solid, inhomogeneously enhancing lesion at the level of the pancreatic tail at Gadolinium-enhanced MR. The tumor had been prospectively overlooked at a contrast-enhanced multi-detector CT but it was subsequently confirmed by somatostatin-receptor scintig- CASE REPORT

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Contrast enhanced multi-detector CT and MR findings of a well-differentiated pancreatic vipoma

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“…Initially, VIP was characterized as a vasodilatory substance [28] responsible for the watery diarrhoea syndrome in patients with VIP-secreting tumours [29]. More recent studies suggest that, in addition, VIP promotes growth and proliferation of normal and malignant cells [20,30].…”

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Mack Forster Award Lecture Receptor nuclear medicine: vasointestinal peptide and somatostatin receptor scintigraphy for diagnosis and treatment of tumour patients

Virgolini

1997

Eur J Clin Investigation

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“…P ancreatic cholera syndrome, also known as Verner-Morrison syndrome 1 and WDHA syndrome, is a constellation of symptoms caused by VIPomas, a rare subtype of neuroendocrine tumor (NET) that typically originates in the pancreas. In this syndrome, excessive production of vasoactive intestinal polypeptide (VIP) provokes secretory diarrhea with associated electrolyte imbalance.…”

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An¹,

Singh²

2016

Synopsis of Spine Surgery

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Hospital Physician July 2008 35P ancreatic cholera syndrome, also known as Verner-Morrison syndrome 1 and WDHA syndrome, is a constellation of symptoms caused by VIPomas, a rare subtype of neuroendocrine tumor (NET) that typically originates in the pancreas. In this syndrome, excessive production of vasoactive intestinal polypeptide (VIP) provokes secretory diarrhea with associated electrolyte imbalance. VIPomas account for less than 1% of all pancreatic tumors and are usually solitary lesions larger than 3 cm, with 75% occurring in the tail of the pancreas. More than 60% of VIPomas metastasize by the time of diagnosis. 2 Diagnosis is dependent on confirming the presence of hormone hypersecretion and localizing the tumor using available imaging modalities. This article presents the case of a woman with the classic presentation of VIPoma who required multiple hospitalizations due to life-threatening hypokalemia. The approach to diagnosis of VIPoma and management of components of pancreatic cholera syndrome are also discussed. CASE PRESENTATION History and Physical ExaminationA 42-year-old woman with no significant past medical history presented to her primary care physician with abdominal pain 15 days after an elective tubal ligation. Initially, the symptoms were thought to be related to the postoperative course and the patient was treated with opiates. However, symptoms persisted for 2 months and were associated with bouts of copious, watery, nonbloody diarrhea (occurring 3-4 times/day). She denied recent use of antibiotics or laxatives. Current medications were acetaminophen/oxycodone to control pain. She had no known drug allergies and denied a history of smoking or illicit drug use. Previous surgeries included the recent laparoscopic tubal ligation, cholecystectomy, breast augmentation, and tonsillectomy. Family history was noncontributory. The primary care physician ordered esophagogastroduodenoscopy and colonoscopy, both of which were unremarkable. Abdominal computed tomography (CT) performed 6 months prior as part of the preoperative evaluation for tubal ligation was also normal. The patient's symptoms worsened, with 8 to 10 bowel movements per day, generalized fatigue, and a 26-lb weight loss, leading to an emergency department (ED) visit.In the ED, the patient's vital signs included a temperature of 99.8°F, blood pressure of 109/60 mm Hg, a regular heart rate of 98 bpm, respiratory rate of 20 breaths/min, and oxygen saturation of 99% on room air. Physical examination revealed abdominal distension, increased bowel sounds with no palpable masses or organomegaly, and a normal rectal examination. Diagnostic StudiesLaboratory values on admission were notable for elevated alkaline phosphatase (348 U/L), aspartate aminotransferase (83 U/L), and alanine aminotransferase (49 U/L) levels. The potassium level was low at 2.4 mEq/L (normal, 3.5-5.0 mEq/L) and remained low despite daily oral potassium replacement. Stool cultures and samples for ovae, parasites, and Clostridium difficile toxin were negative.Repeat abdo...

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Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia

Cited by 579 publications

References 12 publications

Contrast enhanced multi-detector CT and MR findings of a well-differentiated pancreatic vipoma

Contrast enhanced multi-detector CT and MR findings of a well-differentiated pancreatic vipoma

Mack Forster Award Lecture Receptor nuclear medicine: vasointestinal peptide and somatostatin receptor scintigraphy for diagnosis and treatment of tumour patients

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