1/10. Long-term survival after diagnosis of hepatic metastatic vipoma: report of two cases with disparate courses and review of therapeutic options.This report describes two patients with pancreatic cholera caused by vasoactive intestinal polypeptide (VIP)-producing tumors, which originated in the pancreas and showed metastases in both hepatic lobes at time of diagnosis. However, the two tumors displayed remarkably disparate clinical courses. Due to the protracted but progressive course over more than 10 years, a multifaceted therapeutic approach was performed to control symptoms and to improve quality of life. The long-acting somatostatin analog octreotide was the most effective treatment for relieving symptoms and correcting fluid and electrolytes disturbances. The effects of complementary treatments, including systemic chemotherapy and hyperselective chemoembolization, as well as concurrent application of octreotide and prednisolone or interferon with respect to clinical symptoms, VIP levels, and tumor growth are reviewed. Our experience, although small, emphasizes the need for an expert, well-planned, adaptive, and multidisciplinary approach in the care of these complex patients.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/10. Vasoactive intestinal polypeptide-secreting tumor (vipoma) with liver metastases: dramatic and durable symptomatic benefit from hepatic artery embolization, a case report.neuroendocrine tumors often manifest an excess production of hormones that create severe metabolic abnormalities resulting in significant patient morbidity, independent of the tumor burden itself. VIPomas are rare neuroendocrine tumors arising from the pancreas and are associated with secretory diarrhea and electrolyte disturbances. We present a patient with vipoma and hepatic metastases who had greater than 10 loose stools a day for 4 yr since diagnosis, despite debulking surgery, multiple antidiarrheal medications, large doses of octreotide, and targeted radioisotope injections. The patient required several hospitalizations for treatment of dehydration and electrolyte disturbances, despite receiving daily intravenous fluids at home. hepatic artery embolization (HAE) immediately stopped the patient's diarrhea and provided a return to normal formed stools without any other symptom-support measures. One year after HAE, the patient remains asymptomatic and has returned to a productive life. HAE can be a very effective and durable treatment modality for patients with metastatic VIPomas (or other neuroendocrine tumors) and who are clinically symptomatic from the effects of hormone hypersecretion.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/10. Radiofrequency ablation has a valuable therapeutic role in metastatic vipoma.BACKGROUND: vasoactive intestinal peptide-secreting tumours (VIPomas) are rare islet cell tumours of the pancreas that can result in life-threatening biochemical abnormalities. The optimal intervention for metastatic vipoma remains undecided. This case history documents the clinical role of radiofrequency (RF) ablation in the treatment of metastatic vipoma. CASE history: A primary pancreatic vipoma was diagnosed in a 61-year-old female in 1998 and a distal pancreatectomy and splenectomy were performed. She remained disease-free for 44 months when she presented as an emergency with watery diarrhoea, hypokalaemia, renal failure and an elevated serum VIP level. CT scanning showed a liver metastasis and open RF ablation was performed with complete resolution of symptoms and biochemistry within 48 h. Post-ablation imaging confirmed complete ablation of the metastasis. She remained disease-free until 22 months later when watery diarrhoea resumed and a new hepatic metastasis was seen on CT. Percutaneous RF ablation was performed and follow-up CT scan showed complete ablation of the metastasis. The patient remains disease- and symptom-free 10 months after the second RF ablation. CONCLUSION: This case illustrates that the pronounced clinical and biochemical upset caused by metastatic vipoma can be resolved safely, quickly and repeatedly by RF ablation.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
4/10. Four year treatment with a long acting somatostatin analogue in a patient with Verner-Morrison syndrome.The case is described of a woman with a Verner-Morrison syndrome of extreme severity, caused by an occult vipoma. Administration of SMS 201-995 (Sandoz) (SMS) at the dose of 150 and subsequently of 250 micrograms daily, decreased plasma levels of vasoactive intestinal polypeptide (VIP) from about 500 to 100 pg/ml (highest normal limit 60 pg/ml). This was associated with complete regression of the diarrhea and normalization of serum potassium levels and hence with the return of the patient to a fully normal life. After 36 months of clinical remission, watery diarrhea recurred together with elevation of VIP plasma levels and appearance of liver metastases. Laparotomic exploration led to the removal of a pancreatic vipoma and its liver secondarisms, which was followed by a second remission. Reappearance of the symptoms and development of new liver metastases 8 months later required reinstitution of SMS therapy, which allowed once again to control the clinical picture. Anterior pituitary function, assessed by dynamic testing, was unaffected by chronic SMS administration with the exception of the stimulated growth hormone secretion that was inhibited. Glucose tolerance and insulin secretion remained normal during treatment. glucose intolerance ensued after pancreatectomy and was not worsened by reintroduction of SMS. Treatment with SMS may allow long-lasting remission of Verner-Morrison syndrome associated to vipoma, though it does not arrest the progression of the tumor.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
5/10. A case of the watery diarrhea-hypokalemia-achlorhydria syndrome: successful preoperative treatment of watery diarrhea with a somatostatin analogue.A 35-year-old man presenting with severe watery diarrhea was diagnosed as having the watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome with the elevation of plasma vasoactive intestinal peptide (VIP) level. Imaging diagnostic techniques revealed a hypervascular tumor at the tail of the pancreas as well as a solitary liver metastasis. During the patient's stay in hospital, he developed acute renal failure probably due to persistent dehydration and severe hypokalemia. Although these complications improved with artificial dialyses, severe watery diarrhea continued, which made it difficult to achieve surgical resection of the tumor. A new long-acting and potent somatostatin analogue, SMS 201-995 (Sandoz Ltd, Basel, switzerland), was tested and was shown to be effective; after a few hours of subcutaneous injection of this agent, the watery diarrhea disappeared, which in turn improved the patient's hypokalemia, hypercalcemia and metabolic acidosis. Three weeks later, distal pancreatectomy with splenectomy and hepatic lobectomy were successfully performed, and the patient resumed his normal life. The somatostatin analogue has been reported to be useful in the long-term treatment of patients with inoperable WDHA syndrome. The present case demonstrated that short-term administration of this agent is also useful for improving the condition of WDHA patients at the preoperative stage.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
6/10. Successful treatment of therapy-resistant pancreatic cholera with human leucocyte interferon.The pancreatic cholera syndrome is a serious and potentially fatal disease found in patients with endocrine pancreatic tumours and ganglioneuromas. Two patients with therapy-resistant pancreatic cholera syndrome were successfully treated with human leucocyte interferon given intramuscularly in a dose of 3 X 10(6)-6 X 10(6) IU per day. This produced a reduction in stool volume and plasma vasoactive intestinal polypeptide (VIP) within 3-5 days of the start of treatment. Tumour mass decreased in one of the patients after 3 months of treatment but some tumour tissue remained after 15 months' observation, although circulating concentrations of VIP are normal. The mechanisms of action of interferon are not known but a direct inhibition of tumour-cell hormone production and perhaps of tumour-cell proliferation might account for the rapid clinical response.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/10. Secretory diarrhea with islet cell hyperplasia and increased immunohistochemical reactivity to serotonin.Two patients with secretory diarrhea and signs and symptoms consistent with the Verner-Morrison syndrome and islet cell hyperplasia are described. Both patients responded well to subtotal pancreatectomies. The morphologic changes in the pancreata were characterized by proliferation of islets associated with periductal and interstitial fibrosis. Immunohistochemical stains demonstrated increased staining for serotonin in islet cells. A few islet cells also stained for vasoactive intestinal polypeptide. The significance of these results is discussed.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/10. Pathophysiology and management of vipoma: a case study.PURPOSE/OBJECTIVES: To review the pathophysiology and clinical treatment of VIPomas, neuroendocrine tumors that secrete vasoactive intestinal polypeptide, and vipoma syndrome. DATA SOURCES: Published clinical treatments and case studies in the medical literature and case study data from a patient's medical record. DATA SYNTHESIS: VIPomas are rare neuroendocrine tumors that cause a syndrome of life-threatening symptoms. Clinical management is complex, yet little information is available in the medical and nursing literature to guide the clinician. CONCLUSIONS: Information about the pathophysiology and management of the disease may facilitate care of these patients. IMPLICATIONS FOR nursing PRACTICE: A case study presentation provides an example of the care required by one patient with vipoma. The nurse has an important role in assessing, educating, and caring for the patient with vipoma. Interventions include managing complex fluid and electrolyte imbalances, chemotherapy administration and management of side effects, activity and rest alterations, safety issues, altered social roles, and educational needs regarding medications, central lines, and follow-up care.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
9/10. Metastatic pancreatic vipoma: deteriorating clinical course and successful treatment by liver transplantation.Gastrointestinal neuroendocrine tumors are slowly growing and metastases are often limited to the liver. As a result of their favorable biological behavior these tumors have a relatively good prognosis even in metastatic stage. Due to a variety of therapeutic options patients with malignant neuroendocrine tumors may survive for extended periods of time up to ten years. Often a combination of different treatments and also alternation between the different therapeutic regimes is needed. A patient with excessive WDHA-syndrome and severe metabolic disturbances due to a pancreatic vipoma with metastatic spread into the liver and abundant hormonal secretion is presented. Cytotoxic agents (streptozocin, 5-fluorouracil and adriamycin) were able to alleviate clinical symptoms and to control tumor growth for six years. Analogues of somatostatin (octreotide) and interferon alpha had been very useful in controlling clinical symptoms and tumor progress for 18 months. Cytotoxic agents or octreotide were not able, however, to achieve any permanent cure. Eventually, treatment failure occurred with dramatic progression of symptoms and tumor growth, unresponsive to any medical therapy. Consequently, total hepatectomy and liver transplantation together with extirpation of the pancreatic primary tumor was performed and succeeded in providing a normal life to the patient. In our opinion the overall outcome of patients with metastatic vipoma may be improved best by maintaining the patients on medical therapy until treatment failure occurs. In case of extended hepatic metastases orthotopic liver transplantation might be considered for patients with symptomatic disease who no longer respond to conventional treatment modalities.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
10/10. Life threatening diarrhoea ultimately cured by surgery.A 30-year-old previously healthy woman was diagnosed as having a vasoactive intestinal polypeptide (VIP)-producing tumour of the pancreas. Her medical history was typical for neuroendocrine gastrointestinal tumours, presenting initially with non-specific symptoms but eventually she developed life-threatening manifestations requiring intensive care due to severe dehydration. She immediately recovered following surgical resection. The patient had elevated serum concentrations of VIP as well as pancreastatin, and post-operatively elevated concentrations of three growth factors, IGF-I, EGF and TGF-alpha, were seen. The importance of the alterations in plasma concentrations of the different peptides for her symptomatology are discussed.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
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