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Case 14
Published in Andrew Solomon, Julia Anstey, Liora Wittner, Priti Dutta, Clinical Cases, 2021
Andrew Solomon, Julia Anstey, Liora Wittner, Priti Dutta
VIPoma (vasoactive intestinal polypeptide secreting tumours)Severe watery diarrhoea leading to abdominal pain, weakness, hypotension, weight loss and hypokalaemiaAchlorhydria
Gastrointestinal cancer
Published in Peter Hoskin, Peter Ostler, Clinical Oncology, 2020
Gallstones are a common cause of obstructive jaundice and abdominal pain, although they are not commonly associated with systemic symptoms and are not a cause of glucose intolerance. Benign tumours such as a glucagonoma, gastrinoma or VIPoma should also be considered.
Fluids and electrolyte management and nutritional support
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
Craig R Nemechek, Onyebuchi Ukabiala
VIPoma (Verner–Morrison’s syndrome) was originally described as a rare non-betacell pancreatic tumour. It can be associated with multiple endocrine neoplasia type 1 syndrome. More than 20 cases have been described in association with ganglioneuroma and ganglioneuroblastoma as well as the maturation process often induced by chemotherapy for neuroblastoma. Being a neural crest tumour, this is easily understood since the cells in the APUD (amine precursor uptake and decarboxylation) system are often capable of secretions in common with one another. Prolonged/profuse watery diarrhoea, hypokalaemia with dehydration, lethargy, hypotonia and abdominal pain are common. Occasionally, carcinoid syndrome-type flushing with impaired glucose tolerance is seen. Treatment is directed to correction of the water and the electrolyte abnormalities. Pharmacological suppression with somatostatin is helpful but the only cure is surgical resection, even if the residual tumour is benign.
Recent advances in our understanding of mast cell activation – or should it be mast cell mediator disorders?
Published in Expert Review of Clinical Immunology, 2019
Theoharis C. Theoharides, Irene Tsilioni, Huali Ren
Diseases such as carcinoid syndrome, pheochromocytoma, gastrinoma, and VIPoma, which could explain many of the symptoms have to be ruled out through specific diagnostic tests. For instance, serum chromogranin A is a biomarker for carcinoid syndrome, but not for SM [154]. If mast cell clonality is absent, mast cell mediators (e.g. serum tryptase, and 24-h urine N-methylhistamine, PGD2) are measured in symptomatic patients (Figure 1). If these are not elevated, further clinical evaluation is necessary [118]. Idiopathic MCAS may be an underlying cause of various other frequent clinical presentations, like in subsets of patients with ME/CFS [81], fibromyalgia syndrome [82], and IBS [155,156].
Surgical management of pancreatic neuroendocrine tumors: an introduction
Published in Expert Review of Anticancer Therapy, 2019
Elisabeth Hain, Rémy Sindayigaya, Jade Fawaz, Joseph Gharios, Gaspard Bouteloup, Philippe Soyer, Jérôme Bertherat, Frédéric Prat, Benoit Terris, Romain Coriat, Sébastien Gaujoux
VIPoma are rare tumors characterized by WDHA syndrome including watery secretory diarrhea, hypokalemia, and achlorhydria or hypochlorhydria caused by an increased secretion of vasoactive intestinal peptide [16] by the tumor. Approximately 70% of the patients are reported to have metastasis at the time of diagnosis, leading to a poor prognosis [47]. To control hormone secretion, long-acting somatostatin analogs are considered as first-line treatment. It has been reported that the status of 80–90% of patients with VIPoma improves promptly after administration of somatostatin analogues [48].
Vasoactive intestinal peptide-oma causing refractory diarrhea in a young woman
Published in Baylor University Medical Center Proceedings, 2020
Nagasri Shankar, Catherine Linzay, Kyle Rowe
In conclusion, VIPoma is a rare cause of chronic severe diarrhea and presents with a classic triad of persistent secretory diarrhea, hypokalemia, and achlorhydria. Patients presenting with this syndrome should receive appropriate pancreatic imaging. Treatment with somatostatin analogues is key to improving symptom burden and may have antitumor effects. Patients should also be considered for surgical resection, which may be curative and prolong survival.