Presentation
In most cases, metastatic lesions are present at VIPoma diagnosis. [unboundmedicine.com]
VIPomas can be associated with multiple endocrine neoplasia 1 (MEN1), but they remain rare presenting in less than 1% of patients with MEN1 (12). [ncbi.nlm.nih.gov]
Because this condition resembles cholera, Matsumoto et al suggested the alternative term pancreatic cholera. [3] (See Etiology, Presentation, Treatment, and Medication.) [emedicine.staging.medscape.com]
Gastrointestinal
- Diarrhea
C Although chronic diarrhea is frequent, some of its causes are rare, namely, neuroendocrine tumors (NETs). Due to their rarity and non-specific symptoms, such as diarrhea, they are often underdiagnosed. [unboundmedicine.com]
Said, S.I.: Author's reply re: intractable diarrhea. Gastroenterology75:153, 1978 Google Scholar 35. [link.springer.com]
At that time, the syndrome was named watery diarrhea, hypokalemia, achlorhydria (WDHA)-syndrome or Verner-Morrison syndrome. [ncbi.nlm.nih.gov]
Poor quality of life from severe diarrhea Priest WM, Alexander MK. [emedicine.staging.medscape.com]
- Nausea
Sub-acute toxicity of PRRT mainly includes nausea, vomiting and CTCAE grade 3/4 toxicity of hematologic parameters. [ncbi.nlm.nih.gov]
- Vomiting
Sub-acute toxicity of PRRT mainly includes nausea, vomiting and CTCAE grade 3/4 toxicity of hematologic parameters. [ncbi.nlm.nih.gov]
Skin
- Flushing
Nearly 15-30% of patients present with facial flushing (10). The cause of this patchy erythematous flushing is not clear, but it has been attributed to VIP, or prostaglandins, which may be co-secreted by the tumor. [ncbi.nlm.nih.gov]
A small percentage of patients with WDHA syndrome also have hypercalcemia, hyperglycemia, hypochlorhydria, and flushing. [emedicine.staging.medscape.com]
Urogenital
- Renal Insufficiency
Moderately elevated plasma VIP levels can incidentally also be caused by gastrointestinal ischemia, renal insufficiency, or congestive heart failure (15-17). [ncbi.nlm.nih.gov]
Treatment
TREATMENT The first step in the treatment of a patient with a VIPoma is to correct the fluid and electrolyte deficits. In severe cases, intravenous resuscitation with potassium and bicarbonate can be required. [ncbi.nlm.nih.gov]
No… PMC Free Full TextPMC Free PDF Efficacy of treatments for VIPoma: A GTE multicentric series. [unboundmedicine.com]
(See Treatment and Medication.) In contrast to pancreatic carcinoma treatment, an aggressive surgical approach in WDHA syndrome appears warranted in light of improved 5-year survival rates with palliative debulking of tumors. [emedicine.staging.medscape.com]
Vasoactive intestinal peptide, the major mediator of the WDHA (pancreatic cholera) syndrome: value of measurement in diagnosis and treatment. Am. J. Dig. Dis.23:373, 1978 Google Scholar 7. [link.springer.com]
Prognosis
Depending on primary location and hormonal status, they range in terms of clinical presentation, prognosis and treatment. [unboundmedicine.com]
A proper assessment of the prognosis for WDHA syndrome is limited because of the rarity of the condition. [emedicine.staging.medscape.com]
Pancreatic neuroendocrine tumors (PNETs): incidence, prognosis and recent trend toward improved survival. Ann Oncol. 2008;19(10):1727–1733. [PMC free article: PMC2735065] [PubMed: 18515795] 12. [ncbi.nlm.nih.gov]
Etiology
(See Etiology.) VIP-secreting tumors are rare, affecting 0.05-0.2 per million adults, and these tumors most often originate in the pancreas (approximately 95%). [emedicine.staging.medscape.com]
Epidemiology
Epidemiology United States data Pancreatic endocrine tumors are uncommon, with a prevalence of less than 10 cases per million population. [emedicine.staging.medscape.com]
Pathophysiology
Patients need to be aware that dehydration and acute renal failure are significant complications and that they must seek hospitalization if they are not doing well at home. [10] Pathophysiology The pathophysiology of WDHA syndrome is best understood by [emedicine.staging.medscape.com]
Prevention
Efficacy of octreotide in the prevention of complications of elective pancreatic surgery. Italian Study Group. Br J Surg. 1994 Feb. 81(2):265-9. [QxMD MEDLINE Link]. Pratz KW, Ma C, Aubry MC, Vrtiska TJ, Erlichman C. [emedicine.staging.medscape.com]