1/14. pancreatic polypeptide hyperplasia causing watery diarrhea syndrome: a case report.Neuroendocrine tumours of the pancreas can secrete numerous peptides, leading to various recognizable clinical syndromes. The secretion of pancreatic polypeptide has been used as a marker for neuroendocrine tumours but is considered to be a biologically inert peptide. A 37-year-old woman had watery diarrhea syndrome from pancreatic polypeptide hyperplasia. Only 2 other reported cases in the literature have described pancreatic polypeptide hyperplasia; however, this is the first reported case in which the patient was successfully treated by surgical resection, with a 2-year follow-up. This report and review of the literature illustrate that pancreatic polypeptide hypersecretion may present as a clinical endocrinopathy.- - - - - - - - - - ranking = 1keywords = neuroendocrine tumour, neuroendocrine (Clic here for more details about this article) |
2/14. Overview of chronic diarrhea caused by functional neuroendocrine neoplasms.Eight different neoplastic disorders can cause chronic diarrhea attributable to humoral-mediated diarrhea. These include pancreatic endocrine tumor (PET) syndromes (gastrinomas, VIPomas, glucagonomas, somatostatinomas, PET's releasing calcitonin), carcinoid syndrome, medullary thyroid cancer, and systemic mastocytosis. Because these disorders are an uncommon cause of all chronic diarrheas (<1%), they are not often considered in the differential diagnosis, leading to a delay in diagnosis. This is problematic not only because all are treatable, but also because the neoplasm is frequently malignant. In this article, the characteristics and pathogenesis of the diarrhea, important clinical and diagnostic laboratory features, and treatment of each disorder are briefly reviewed, with an emphasis on recent insights.- - - - - - - - - - ranking = 0.41703302868603keywords = neuroendocrine (Clic here for more details about this article) |
3/14. Metastatic jejunal vipoma: beneficial effect of combination therapy with interferon-alpha and 5-fluorouracil.The vipoma syndrome is rare. It is usually caused by a neuroendocrine tumor located in the pancreas. Somatostatin analogs and interferon-a can be helpful in the symptomatic control of the disease, but the efficacy of chemotherapy in metastatic disease is limited. We report the case of a 32-yr-old patient who had a primary intestinal vipoma with peritoneal carcinomatosis and hepatic metastases. Somatostatin analogs and conventional chemotherapy regimens were not effective on vipoma syndrome and tumor progression. The combination of 5- fluorouracil and interferon-alpha was associated with a major clinical improvement and tumor regression. Further investigations should evaluate the place of such a combination as a first line treatment for patients with metastatic neuroendocrine tumors.- - - - - - - - - - ranking = 0.20851651434302keywords = neuroendocrine (Clic here for more details about this article) |
4/14. 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 = 0.20851651434302keywords = neuroendocrine (Clic here for more details about this article) |
5/14. Large cell carcinoma with calcitonin and vasoactive intestinal polypeptide-associated Verner-Morrison syndrome.Verner-Morrison syndrome, characterized by diarrhea, hypokalemia, and hypochlorhydria, is caused most commonly by vasoactive intestinal polypeptide-secreting islet cell tumors of the pancreas. Verner-Morrison syndrome has not been described as a paraneoplastic syndrome in non-small cell lung cancer. We describe a 38-year-old man with metastatic non-small cell lung cancer of large cell carcinoma with neuroendocrine differentiation who presented with bone metastasis and intractable secretory diarrhea that was unresponsive to pharmacological treatment, including octreotide. Laboratory evaluation indicated elevated serum calcitonin and vasoactive intestinal polypeptide levels. Chemotherapy resulted in a transient response in the patient's diarrhea and neuroendocrine markers. The patient did not respond to further therapy and died 5 months after onset of back pain. To our knowledge, this is the first published case of large cell carcinoma with neuroendocrine differentiation associated with treatment-responsive paraneoplastic Verner-Morrison syndrome.- - - - - - - - - - ranking = 0.31277477151452keywords = neuroendocrine (Clic here for more details about this article) |
6/14. Laparoscopic distal pancreatectomy for neuroendocrine tumors of the pancreas.Although neuroendocrine tumors of the pancreas are traditionally managed by laparotomy, these rare neoplasms may be amenable to laparoscopic surgical resection. We present our experience with laparoscopic distal pancreatectomy in two such patients, and discuss the operative technique with emphasis on organ preservation. Two female patients aged 63 and 69 years presented with clinical and biochemical features of an insulinoma and a vasoactive intestinal peptide secreting tumor (vipoma), and were found on cross-sectional imaging to have 1.2-cm and 4.5-cm solitary tumors in the tail of the pancreas. They underwent laparoscopic distal pancreatectomy with and without preservation of splenic vessels and spleen respectively. Both procedures were completed laparoscopically. The operating time was 180 and 210 minutes respectively. There were no postoperative complications. The postoperative hospital stay was 4 and 14 days respectively. histology revealed a benign insulinoma and a malignant vipoma with lymph node metastases respectively. Laparoscopic distal pancreatectomy for neuroendocrine tumors of the pancreas may be accomplished safely, with preservation of the spleen and splenic vessels in benign disease, and with benefits to the patients in terms of postoperative recovery.- - - - - - - - - - ranking = 0.62554954302905keywords = neuroendocrine (Clic here for more details about this article) |
7/14. Neuroendocrine tumours of the gut: long-term therapy with the somatostatin analogue SMS 201-995.Nine patients with metastatic neuroendocrine tumours of the gut were treated for over 100 days with the long-acting octapeptide of somatostatin, SMS 201-995. Two patients had unusually aggressive tumours (neuroendocrine tumours are typically slow-growing) with only modest elevation of tumour secretory products. SMS 201-995 was given in an attempt to slow tumour growth, but tumour size continued to increase. Three patients had glucagonomas and uncontrolled skin rashes, and four patients had VIPomas, which caused uncontrolled watery diarrhoea. SMS 201-995 was given principally to reduce hypersecretion of hormones from their tumours and to relieve symptoms. All the patients experienced rapid relief of their symptoms when SMS 201-995 treatment was introduced, and symptomatic control was maintained throughout treatment except in one patient, who required a supplementary procedure (pancreatic primary tumour embolization). Circulating peptide levels were initially suppressed, but continued suppression did not occur in every case despite good clinical control. It seems likely that SMS 201-995 has beneficial effects on target organs as well as suppressing the release of peptides from tumour tissue. The tumours did not grow during treatment. The analogue was well tolerated, most adverse effects being minor. Somatostatin inhibited insulin release, but in only one patient was there a slight deterioration of glucose tolerance. SMS 201-995 is therefore a useful therapeutic tool in the long-term management of patients with symptoms due to uncontrolled hormone secretion from neuroendocrine tumours.- - - - - - - - - - ranking = 3keywords = neuroendocrine tumour, neuroendocrine (Clic here for more details about this article) |
8/14. Clinical evaluation of SMS 201-995. Long-term treatment in gut neuroendocrine tumours, efficacy of oral administration, and possible use in non-tumoural inappropriate TSH hypersecretion.Long-acting somatostatin analogues such as SMS 201-995 (Sandoz) are being evaluated in a wide range of clinical indications, including gut neuroendocrine tumours and acrogemaly. Long-term continuous SMS 201-995 treatment has achieved useful symptomatic improvement in diarrhoea in 4 patients with metastatic VIPomas who had relapsed following previous treatment. Clinical improvement has outlasted suppression of VIP secretion (suggesting an additional direct antisecretory action of SMS 201-995) and has occurred despite expansion of hepatic metastases. In 6 patients with tumours secreting gastrin and/or glucagon, secretion of these peptides was acutely inhibited by SMS 201-995. However, endocrine and clinical responses to chronic treatment have been less consistent. SMS 201-995 is active orally at doses of 4-8 mg and when given thrice-daily to 6 patients with active acromegaly, suppressed mean 24-h growth hormone levels by 51-88%. Despite significantly reduced plasma insulin concentrations, glucose tolerance did not deteriorate. SMS 201-995 was also effective in suppressing thyroid-stimulating hormone (TSH) and thyroid hormone secretion in a patient with mild thyrotoxicosis due to non-tumoural inappropriate TSH hypersecretion. In all cases SMS 201-995 treatment has been well tolerated and has few side-effects.- - - - - - - - - - ranking = 5keywords = neuroendocrine tumour, neuroendocrine (Clic here for more details about this article) |
9/14. Neuroendocrine gut neoplasms. Important lessons from uncommon tumors.OBJECTIVE: To review our experiences in order to high-light some important lessons learned in the treatment of patients with neuroendocrine gut neoplasms. DESIGN: Retrospective analysis of case series of 70 patients with neuroendocrine gut neoplasms treated between 1983 and 1993. The clinical features of individual patients illustrate lessons in surgical treatment. SETTING: University hospitals with tertiary care referral practice. INTERVENTIONS: The main intervention was abdominal exploration in 43 patients, with resection of the primary tumor in 39 and of hepatic metastases in four. MAIN OUTCOME MEASURES: To describe the tumors seen and to identify major lessons learned. RESULTS: Of 70 patients with neuroendocrine tumors treated, 31 had carcinoid tumors, 10 each had insulinomas and gastrinomas, five had vipomas, nine had non-functioning islet cell tumors, three had glucagonomas, and one each had somatostatinoma and a possible cholecystokinin-secreting tumor (or CCKoma). Important lessons learned include: (1) the importance of preoperative tumor localization; (2) in multiple endocrine neoplasia, type I syndrome, the tumor found may not be the one responsible for the patient's symptoms; (3) solitary sporadic tumors secreting multiple peptides may mimic multiple tumors in multiple endocrine neoplasia, type I syndrome; (4) one needs to be prepared for the unexpected, such as the carcinoid crisis; (5) resection may sometimes be necessary even with advanced local disease; and (6) selected patients may benefit from pancreaticoduodenectomy. CONCLUSIONS: These rare tumors are interesting in their clinical presentation and can be challenging in their treatment.- - - - - - - - - - ranking = 0.31277477151452keywords = neuroendocrine (Clic here for more details about this article) |
10/14. liver vipoma: report of two cases and literature review.Two cases of neuroendocrine tumor in the liver, positive for VIP, without evidence of a primary tumor outside the liver is presented. One patient had a vipoma syndrome with diarrhea, hypokalemia, and hypercalcemia, all symptoms were reversible after treatment consisting of somatostatin analogue and arterial liver embolization followed by liver resection. The other patient showed no endocrine symptoms. To the best of our knowledge, VIPomas apparently primary in the liver have not been previously described.- - - - - - - - - - ranking = 0.10425825717151keywords = neuroendocrine (Clic here for more details about this article) |
| | Next -> |