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Background: The aim of this study was to describe period and cohort effects in incidence and mortality of stomach and pancreatic cancer in England and Wales., Methods: National figures for mortality (1951-2000) and incidence (1971-2000) were analysed using log-linear Poisson regression models to obtain relative risks (RR) for period (year of incidence or death) and cohort (year of birth)., Results: Stomach cancer shows a pronounced cohort effect in mortality with a decline in RR in men from 2.20 (1876) to 0.47 (1946) and a reduction from 2.79 to 0.41 for women. Mortality to incidence ratios are now less than 0.70. Pancreatic cancer mortality (men) RR rose from 0.91 (1951-1955) to a peak 1.11 (1976-1980) and then declined to 0.90 (1996-2000). Women showed a similar pattern. Cohort RR (men) increased to a peak of 1.14 in 1916 and declined to 1.01 in 1946, and continued to fall; the peak occurred slightly later in women. Mortality to incidence ratios were near 1 in the first 20 years, declining to 0.95 in the last 10 years., Conclusion: Stomach cancer incidence has fallen continuously from 19(th) century birth cohorts onwards. Incidence of pancreatic cancer has fallen in successive birth cohorts after 1920; peak period risk was 1976-1990. Age-standardized mortality and case mortality for pancreatic cancer are declining., (Copyright (c) 2007 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd.)

Background/aims: After pancreaticoduodenectomy (PD) patients may be deficient in essential micronutrients. This study was designed to determine if this is a consequence of surgery., Methods: Long-term survivors (>6 months) of PD for peri-ampullary neoplasia and healthy controls (patients' spouse/partner) were enrolled in the study. Specific clinical parameters were recorded, serum micronutrient levels were measured and subjects completed 7-day food diaries., Results: Thirty-seven patients were studied, 25 with paired controls. All were well nourished, as defined by body mass index and food diary analysis. Patients with paired controls were representative of all patients studied. Patients had raised transferrin (median 2.60 vs. 2.16 g/l, p = 0.001) and low ferritin levels (34.9 vs. 119.0 g/l, p < 0.001) indicating relative iron deficiency. Patients also demonstrated lower levels of the anti-oxidants selenium (0.77 vs. 0.93 micromol/l, p < 0.001) and vitamin E (23.2 vs. 35.7 micromol/l, p < 0.001) with 57% of patients having frank selenium deficiencies. Patients had lower levels of vitamin D than controls (15.7 vs. 19.6 micromol/l, p = 0.001) and 30% of patients had a raised parathyroid hormone level, suggesting compensatory mechanisms operate to maintain normocalcaemia., Conclusions: Long-term survivors of PD are relatively deficient in several micronutrients compared to non-operated controls taking the same diet. We recommend that micronutrient status should be regularly checked in these patients and treated where necessary.

Essential fatty acids, especially gamma-linolenic acid (GLA), have been shown to directly inhibit the growth of cancer cell lines in culture. The aim of this study was to see whether an aqueous formulation of GLA works as well as the lithium-based salt. We evaluated the effect of the 1-deoxy-1-methylamino-D-glucitol salt of GLA (MeGLA) on the growth of two human pancreatic cancer cell lines (Panc-1 and MIA PaCa-2) in vitro, and compared its effects with a previously studied formulation, lithium GLA (LiGLA). The effect of time exposure (2-7 days) and difference in concentration (0-1000 micromol/l) were studied using 96-well culture plates. Cell growth was assessed by MTT assay. Control experiments were performed with meglumine alone in similar concentrations. MeGLA had cytostatic and cytotoxic effects on pancreatic cancer cell lines with 50% growth inhibition at 30-100 micromol/l and cytotoxic effects at 60-250 micromol/l. The degree of growth inhibition increased with time of exposure to MeGLA. The anti-proliferative effects of MeGLA were similar to those previously observed with LiGLA. We conclude that MeGLA has equivalent anti-proliferative activity to LiGLA when tested in vitro against pancreatic cancer cell lines and is therefore a suitable alternative to LiGLA for investigation of the in vivo activity of GLA against pancreatic adenocarcinomas.

Background: Gamma-linolenic acid (GLA) is growth inhibitory both in vitro and in vivo, at doses non-toxic to non-cancer cells. Chemotherapeutic agents have limited activity in pancreatic cancer. Interactions between GLA and cytotoxic drugs have not previously been investigated; any synergy might improve the therapeutic effect of these agents., Aim: To investigate possible interactions between GLA and 5-fluorouracil (5-FU) or gemcitabine against pancreatic cancer cell lines in vitro., Methods: Two pancreatic cancer cell lines were exposed to GLA alone and in combination with 5-FU or gemcitabine. Residual viable biomass was measured using the MTT assay and the results analysed by the median effect method of Chou and Talalay [Adv Enzyme Regul 1984;22:27-55]., Results: GLA concentrations of 3.9- 125 microg/ml had a synergistic or additive growth inhibitory effect on all tested concentrations of gemcitabine. Synergism was demonstrated between GLA and 5-FU only at concentrations of 62.5-125 microg/ml of 5-FU., Conclusion: GLA has a synergistic effect with gemcitabine at concentrations that correspond to in vivo therapeutic doses. GLA with 5-FU is synergistic only at a tight range of high concentrations of 5-FU. GLA lacks toxic side effects and may be useful in combination with gemcitabine., (Copyright 2003 S. Karger AG, Basel and IAP)

Pancreatic cancer has a poor response to conventional chemotherapy and radiotherapy. Inhibition of matrix metalloproteinase activity involved in tumour invasion and metastases is a novel biological approach for cancer treatment. This multicentre phase II clinical trial assessed marimastat, an oral matrix metalloproteinase inhibitor, in patients with advanced pancreatic cancer. A total of 113 patients received marimastat for 28 days at 100 mg b.d. (n = 9), 25 mg o.d. (n = 90) or 10 mg b.d. (n = 14). Patients with a response to treatment could continue marimastat beyond 28 days. Of 113 patients, 90 (80%) completed the 28-day study and 83 (73%) continued treatment. The principal side effect was arthralgia in 14 (12%) patients at 28 days and 33 (29%) patients over the whole study. There were 31 patients (27%) who required dose modification. Of 76 patients with evaluable CA19-9 levels, 23 (30%) showed no increase or fall in CA19-9. Of 83 patients with radiologically assessable disease, 41 (49%) had stable disease. The median survival was 245 days for those with a stable or falling CA19-9 level 128 days in those with rising CA19-9. The overall survival was 3.8 months. 5.9 months for stage II, 4.7 months for stage III and 3 months for stage IV disease. Of 90 patients, 46 (51%) had stabilization or reduction in pain, mobility and analgesia scores. Further development and clinical evaluation of matrix metalloproteinase inhibitors for the treatment of pancreatic cancer is warranted.

Background: Chemotherapy for pancreatic cancer offers small survival benefits and considerable side-effects. Unsaturated fatty acids have an antitumour effect in experimental studies; in phase II studies few side-effects were seen., Methods: In this group-sequential, open-label, randomized study, 278 patients with a diagnosis of inoperable pancreatic cancer were treated with either oral (700 mg daily for 15 days), low-dose (0.28 g/kg) or high-dose (0.84 g/kg) intravenous lithium gamolenate (LiGLA). The primary endpoint was survival time from randomization using Kaplan-Meier estimates., Results: Median survival after oral and low-dose intravenous treatment was 129 and 121 days respectively. Median survival after high-dose intravenous treatment was 94 days. A good Karnofsky score and the absence of metastases were associated with increased survival. Haemolysis, a marker of rapid infusion, was associated with a median survival time of 249 days in the low-dose intravenous group., Conclusion: Oral or low-dose intravenous LiGLA led to survival times similar to those of other treatments for pancreatic cancer although one subgroup (low-dose intravenous LiGLA with haemolysis) had longer survival. High-dose intravenous treatment appeared to have an adverse effect. Systemic treatment with LiGLA cannot be recommended for the treatment of pancreatic cancer.

Background: Neuroendocrine tumors of the pancreas are uncommon neoplasms of the pancreas that can occur sporadically or in association with various syndromes such as multiple endocrine neoplasia type 1 (MEN I). Patients can present with a specific clinical syndrome related to biochemically functioning tumors or with nonspecific symptoms related to mass effect or metastases. The size, function, consistency, and malignant behavior of neuroendocrine tumors are integrally related. Imaging has a major role in the preoperative localization of the primary tumor and detection of metastases. Several techniques are available including ultrasound, endoscopic ultrasound, computed tomography (CT), MR, somatostatin receptor scintigraphy, angiography, and arterial stimulation with venous sampling; each with unique advantages and limitations. The reported performance for these techniques vary widely, and as a result, recommended imaging algorithms are controversial. Recent technical advances in ultrasound, CT, and MRI have occurred that may improve the sensitivity of some of the techniques. Further improvements are likely in the future. In this chapter we will review imaging techniques used to study the pancreas as it relates to the detection of neuroendocrine tumors, imaging findings of these tumors using various imaging modalities, and the advantages, limitations and results obtained with each technique.

Neuroendocrine tumors of the pancreas (NETP) are rare. We report our surgical experience of 11 patients with NETP. These included 5 patients with benign insulinomas. Raised serum insulin and C-peptide levels with hypoglycemia were always diagnostic. Ultrasonography, CT, visceral angiography, arterial stimulation and venous sampling, and intraoperative ultrasound localized the tumor in 0/5, 1/5, 3/4, 2/2, 3/3 cases, respectively. The 6 other malignant NETP (one gastrinoma, 2 carcinoids, 3 non-functioning) were managed by pancreatic resection (Whipple's operation = 3, distal pancreatectomy with total gastrectomy = 1, total pancreatectomy = 1, distal pancreatectomy with left nephrectomy and proximal gastrectomy = 1). Two patients died postoperatively. We had 5 major and 2 minor postoperative complications, with 2 deaths. During follow up from 1 to 7 years, one patient with malignant carcinoid tumor died at two and half years, of local recurrence. The other 8 patients are disease-free with good quality of life.

Background: Detection of metastatic liver disease and malignant involvement of major peripancreatic vessels is important to determine resectability of pacreatic malignancy. Computed tomography with arterial portography (CTAP) is the most sensitive method for detection of colorectal liver metastases; it can also detect malignant vascular involvement. We have assessed CTAP in patients with pancreatic cancer considered suitable for resection after standard ultrasonography (US) and computed tomography (CT) examination., Method: CTAP was performed in 18 patients (8 with a biliary stent). All patients had previous US and CT with no clear evidence of irresectability. Findings of CTAP were compared with the prior CT and with findings at operation or clinical progress., Results: CTAP suggested liver metastases in 7 patients. Three were confirmed at operation or at follow-up (sensitivity for detection of metastases in CT negative patients of 75%). There were 4 false-positive assessments (specificity, 71%). One further patient developed liver metastases within 6 mo after resection (1 false-negative). Nine patients had vascular involvement at operation. There was 1 false-positive and one false-negative assessment (sensitivity, 89% and specificity, 89%). CTAP detected vascular involvement in 4 patients in whom it was not detected by CT., Conclusion: This preliminary study suggests that CTAP is a sensitive test for detection of liver metastases and vascular involvement in patients with pancreatic malignancy. This invasive test should be reserved for patients who are considered operable on the basis of other preoperative tests.

Essential fatty acids such as (gamma)linolenic (GLA) and eicosapentaenoic (EPA) acids have been proposed as anticancer drugs. The aim of this study was to test the effect of a lipid emulsion containing both GLA and EPA in a novel chemical formulation of 1-(gamma)linolenyl-3-eicosapentaenoyl propane diol on the growth of human pancreatic carcinoma in vitro and in nude mice. This compound had a dose-dependent growth-inhibitory effect on human pancreatic cancer cell lines MIA PaCa-2 and Panc-1 in vitro. The concentration necessary for 50% growth inhibition was 25 micromol/l for MIA PaCa-2 and 68 micromol/l for Panc-1 (95% CI 20-29 and 59-77 micromol/l respectively). Nude mice bearing subcutaneous pancreatic tumours produced with the MIA PaCa-2 cell line were treated with the maximum tolerated dose (6.75 mg GLA and 7.3 mg EPA per g of body weight) administered over 10 days by daily intravenous (i.v.) bolus injections. No antitumour effect or major alteration in tumour lipid fatty acid composition was seen in comparison with control animals. Concurrent treatment with parenteral iron (iron saccharate, 5 microg/gram body weight daily) did not make a significant difference. Further improvements in fatty acid delivery mechanisms are necessary before they can become useful anticancer agents.

There is overwhelming consensus that quality of life assessment is urgently required in pancreatic cancer, yet little research has been conducted. We report on the development of a disease specific questionnaire module to supplement the EORTC core cancer module, the QLQ-C30 in patients with pancreatic cancer, using EORTC quality of life study group guidelines for module development. Relevant QoL issues were generated from literature searches and interviews with health professionals and patients with pancreatic cancer. Issues were constructed into items and provisionally translated. The provisional module was pretested in patients in 8 European centres. The resulting module the QLQ-PAN26 includes 26 items related to disease symptoms, treatment side-effects and emotional issues specific to pancreatic cancer. This should ensure that the module will be sensitive to assess the small but important disease and treatment related QoL changes in pancreatic cancer. The use of the QLQ-C30 and QLQ-PAN26 will provide a comprehensive system of QoL assessment in international trials of pancreatic cancer.

During the initial stages of development of a pancreatic cancer quality of life (QoL) module to supplement the EORTC core QoL module, the QLQ-C30, a qualitative study was undertaken to explore observed differences in the health professionals' and patients' perception of the illness, treatment and care of pancreatic cancer. Semi-structured one-to-one interviews were conducted with a range of six health professionals and 21 pancreatic cancer patients from two acute general hospitals and one teaching hospital within the Wessex Region. Grounded theory guided data collection and analysis. There was good agreement between the content of issues generated by professionals and patients with 42 relevant and specific issues identified. However, subtle differences in perception were observed when the context of why such issues were important was examined between the two groups. Health professionals took a mechanistic view and saw the impact of each symptom or problem as directly affecting quality of life perception. Patients' perception of quality of life was mediated by the process of coping. This was grounded by two linked factors. First, the perceived threat of each symptom or problem to the patient and second, the success or otherwise of coping strategies employed to maintain control. Five main coping strategies were identified. This study highlighted that there are important and specific quality of life issues which warrant a need for a pancreatic cancer QoL module. This study emphasises the fact that patients are the best source to describe their quality of life. Health professionals should acknowledge the effect of coping strategies when assessing the impact of symptoms and their treatment on patients with pancreatic cancer.

Background: The lithium salt of gamma-linolenic acid (Li-GLA) is growth inhibitory to pancreatic cancer cells in vitro and is reported to prolong the survival of patients with pancreatic cancer. The effect of Li-GLA on the growth of human pancreatic carcinoma in vivo is not known. In this study the effect of parenterally administered Li-GLA on the growth of human pancreatic carcinoma in nude mice was tested., Methods: Pancreatic tumours were produced in nude mice by subcutaneous implantation of MIA PaCa-2 cells. This cell line is sensitive to Li-GLA in vitro. Mice were randomly treated with intraperitoneal, intravenous or intratumoral Li-GLA. Each group also had controls., Results: Both intravenous and intraperitoneal administration of Li-GLA had no significant effect on tumour growth or tumour phospholipid fatty acid composition. Intratumoral administration of Li-GLA was, however, associated with a significant antitumour effect., Conclusion: Within the limitations of this tumour model, the benefit seen with intravenous Li-GLA in patients with pancreatic carcinoma cannot be explained by tumour growth inhibition. Local administration appears to be more effective than intravenous or intraperitoneal therapy.

Background: The proper evaluation of patients with pancreatic cystic neoplasms remains controversial. Although computed tomography (CT) and sonography can differentiate the majority of these tumors, needle aspiration and biopsy can be helpful in selected patients. The purpose of this study was to (1) determine the sensitivity of needle aspiration and biopsy of cystic pancreatic neoplasms, (2) assess the complications after needle aspiration and biopsy, and (3) assess the indications for needle aspiration and biopsy by using the radiographic appearance as a guide., Methods: The histories, imaging studies, and pathologic specimens of 18 patients with serous cystadenoma (10 patients) or mucinous cystic tumor (eight patients) of the pancreas who also underwent CT-guided (n = 16), sonographically guided (n = 3), or intraoperative (n = 4) needle biopsy (n = 23 total biopsies) between 1976 and 1996 were retrospectively reviewed., Results: The histologic diagnosis of the serous subtype was made with needle aspiration and biopsy in five of 10 patients with serous cystadenoma. The histologic diagnosis of the mucinous subtype was made with needle aspiration and biopsy in six of eight patients with mucinous cystic neoplasms. Of the 23 biopsies performed, one patient experienced significant intraperitoneal hemorrhage. Indications for needle biopsy include an atypical cystic mass on diagnostic imaging studies, confirmation of a serous cystadenoma before observation, and confirmation of mucinous cystadenocarcinoma in patients with nonresectable disease., Conclusion: Needle biopsy and aspiration is a helpful and safe procedure to aid in the diagnosis of cystic pancreatic neoplasms before surgery.

In the past decade there has been increasing focus on assessment of the quality of life (QoL) of patients with cancer. QoL is now a mandatory endpoint in many cancer clinical trials, particularly in interventions with palliative intent alone. Although QoL is difficult to define, a variety of specially designed and validated questionnaires has been developed to measure QoL in the clinical setting. In pancreatic cancer, assessment (QoL) is of paramount importance; however, little research has been conducted and until recently no disease-specific QoL questionnaire existed. In this review we consider the quality of life of patients with pancreatic cancer, with particular reference to the symptoms and psychosocial consequences of the disease. The literature is critically examined on the approaches to evaluation of QoL in previous pancreatic cancer clinical trials. We then describe the impact on QoL following both "curative" and palliative surgical interventions and medical treatment, including endoscopic palliation and chemotherapy for pancreatic cancer. In the European Organisation for Research and Treatment of Cancer (EORTC) the questionnaire specific for pancreatic cancer, the QLQ-PAN26, is discussed as a standardised and valid assessment of QoL in international clinical trials. Finally, recommendations are made for future QoL research in pancreatic cancer.

Essential fatty acids, especially gamma linolenic (GLA) and eicosapentaenoic acids, have been proposed as potential anticancer drugs. Our aim was to study the effect of the lithium salt of gamma linolenic acid (LiGLA) on the growth of two human pancreatic cancer cell lines (MIA PaCa2 and Panc 1) and primary human fibroblasts (HFF 5) in vitro. Cell growth was assessed by a microculture tetrazolium (MTT) assay. LiGLA had a selective growth inhibitory effect on pancreatic cancer cell lines with 50% growth inhibition (IC50) at approximately 6-16 mumol/l compared with approximately 111 mumol/l for the fibroblasts. The degree of growth inhibition increased with the time of exposure to LiGLA. Special attention was paid to the influence of albumin and iron on LiGLA-mediated growth inhibition. Albumin incorporated into essentially serum-free culture medium inhibited the effect of LiGLA in a dose-dependent manner, associated with reduced GLA uptake by cancer cells. Ferric ions were confirmed as potentiators of the growth inhibitory effect of LiGLA but more physiologically relevant transferrin-bound iron was ineffective. With further improvements in the fatty acid delivery mechanism, LiGLA may become a useful adjunct in the management of pancreatic cancer patients.

We present a case of pancreatic malignancy which presented as chronic pancreatitis. The diagnostic difficulties are discussed. We recommend that when there is any doubt about a diagnosis of chronic pancreatitis with a pancreatic mass, then early resection is appropriate.

Adenocarcinoma of the pancreas is a common and dreadful disease with an extremely poor prognosis. In practice, only a few patients are cured but surgical resection, although feasible in less than 20% of patients, offers maximum prolongation of life and provides good palliation of symptoms. This can now be performed safely, even in elderly patients, in specialist units. Better radiological imaging and laparoscopy allow selection of resectable tumours effectively. All patient with pancreatic cancer should now be assessed for surgical resection and potentially suitable patients should be referred to a specialist team at an early stage.

Cystic lymphangioma of the pancreas is a rare condition. A 14-year-old girl presented with a cystic abdominal mass and abdominal pain. She was initially treated by biopsy and cyst drainage, and subsequently with partial excision of the cystic mass. The mass, which was a cystic lymphangioma involving the pancreas, recurred after each operation. Persisting symptoms led to a pylorus-preserving pancreatectomy and hepaticojejunostomy. Total excision is the only effective method of treating this benign tumour. The patient remains symptom free 2 1/2 years after pancreatectomy.

A survey was carried out by postal questionnaire of the attitudes of British surgeons to pancreatic resection as palliation for ductal adenocarcinoma of the pancreas. Replies from 24 surgeons related to experience in over 700 resections. The incidence of estimated residual local disease after resection was median 12.5 percent, range 0-35 percent. Half (12) of the surgeons felt that pancreatic resection with residual macroscopic disease was justified. Only 3 (12.5 percent) surgeons accepted that palliative resection in the presence of liver metastases was sometimes justifiable. Further evidence is required of improved quality of life after resection before the majority of surgeons will accept palliative resection in the management of pancreatic ductal adenocarcinoma.

Objective: The purposes of this study were to determine the nature, prevalence, and CT findings of pancreatic lesions in patients with von Hippel-Lindau disease and to determine whether identification of pancreatic cysts and neoplasms is important in establishing the diagnosis of von Hippel-Lindau disease., Subjects and Methods: The medical records and radiologic images of 52 patients with von Hippel-Lindau disease who were evaluated at our institution between 1976 and 1992, and who at some stage underwent abdominal CT, sonography, or MR imaging, were reviewed. The nature, prevalence, and CT findings of the pancreatic lesions were determined, and the role of the pancreatic abnormalities in establishing the diagnosis of von Hippel-Lindau disease was studied., Results: Twenty-nine (56%) of the 52 patients had pancreatic lesions. Nineteen patients had pancreatic cysts and no other pancreatic lesion. Four patients had islet cell tumors only, one had a microcystic adenoma only, and three had indeterminate pancreatic masses. One patient had cysts and an islet cell tumor, and another patient had cysts, an islet cell tumor, and a microcystic adenoma. In six patients (12%), pancreatic lesions were the only abdominal manifestation of von Hippel-Lindau disease. In three patients screened because of a family history of von Hippel-Lindau disease, no CNS abnormalities were present, and the only abdominal lesions were in the pancreas (cysts in two cases, islet cell carcinoma in the other). Thus, the pancreatic lesion was an important factor in establishing a diagnosis of von Hippel-Lindau disease in these patients., Conclusion: Pancreatic lesions may be the only abdominal manifestation of von Hippel-Lindau disease. CT findings include cysts, islet cell tumors, and microcystic adenomas. Pancreatic lesions, including cysts, may precede any other manifestation of von Hippel-Lindau disease by several years, and recognition permits earlier diagnosis in patients being screened for von Hippel-Lindau disease.

Adenocarcinoma of the pancreatic body and tail often presents late and is widely regarded as incurable by surgical resection; long-term survivors are rare. Thirteen patients underwent left resection (n = 7) or total pancreatectomy (n = 6) in a consecutive series of 105 patients with carcinoma of the body or tail of the pancreas. Comparison was made with 17 patients with locally advanced or metastatic disease. Preoperative computed tomography predicted irresectable disease when a large perivascular lymph node mass was demonstrated. Preoperative angiography predicted irresectable disease when there was encasement or obliteration of the coeliac axis or its major branches, or of the superior mesenteric artery or vein. Splenic vessel involvement was sometimes compatible with resection. After resection, median survival was 13 (range 3-50) months, with a minimum follow-up of 2 years. Five patients survived more than 2 years, and three are still alive 30, 43 and 50 months after resection. Resection of carcinoma of the body or tail of the pancreas was possible in 12 per cent of patients and long-term survival was observed in some of these.

Objective: The purpose of this study is to report the spectrum of MR findings of pancreatic islet cell carcinoma., Materials and Methods: The MR scans of 33 patients with islet cell carcinoma were retrospectively reviewed. Magnetic resonance detected the primary tumor in 21 of 27 patients (78%) who had not had prior resection of their primary tumor. Mean tumor diameter was 7.1 cm (range 3.5-13.0 cm)., Results: In all patients, the primary tumor on T1-weighted images (TR/TE = 250/15) was of signal intensity equal to or lower than that of the adjacent normal pancreas. The primary tumor on T2-weighted images (TR/TE = 2,000/> or = 100) was of signal intensity the same as or higher than fat in 18 of 21 patients (86%) and had mixed signal intensity in the other 3 (14%). Hepatic metastases were found in 28 of 33 patients (85%). Liver metastases were categorized as "usual" (variably circumscribed, homogeneous lesions of medium signal intensity on T2-weighted images) in 19 of 28 patients (68%), necrotic in 8 of 28 (29%), hemorrhagic in 3 of 28 (11%), and calcified in 1 of 28 (4%). Extrahepatic metastases were found in 18 of 33 patients (55%)., Conclusion: We conclude that MRI is an excellent modality for the diagnosis and routine follow-up of patients with islet cell carcinoma.

This debate discusses the palliative management of pancreatic cancer. The arguments in favour of surgical palliation are that this approach allows all symptoms to be treated or prevented, the diagnosis can be confirmed histologically and a final assessment of resectability can be made. The arguments against the use of surgery are that survival is short and that effective alternative therapies are available: endoscopic intubation, percutaneous coeliac plexus block and pancreatic enzyme supplements. The most appropriate policy, however, is to tailor the management plan to suit the individual patient.

Von Hippel-Lindau disease is a rare, autosomal-dominant disorder characterized by CNS hemangioblastomas, retinal angiomas, renal cell carcinomas, pheochromocytomas, and visceral cysts. The occurrence of islet cell tumors in von Hippel-Lindau disease has been noted recently. Because of the coexistence of both islet cell tumors and pheochromocytomas in some patients with this disorder, it has been proposed that there may be a continuum of the multiple endocrine neoplasias. However, no large, multifamily study has been published evaluating the prevalence of islet cell tumors and pheochromocytomas in von Hippel-Lindau disease. To assess the frequency of islet cell tumors in this disorder and its relationship to the multiple endocrine neoplasias, we reviewed the clinical and imaging findings of all patients with von Hippel-Lindau disease evaluated at the Mayo Clinic between January 1979 and December 1989. Forty-three patients with von Hippel-Lindau disease from over 25 kindreds were found. Cross-sectional imaging of the pancreas had been performed in 35. Islet cell tumors were found in six (17%) of these, three islet cell adenomas and three islet cell carcinomas. No patient presented with endocrine-related symptoms; four tumors were detected during screening examinations of the abdomen. Two (33%) of these six patients had a coexisting pheochromocytoma. Our review of a large number of patients from many different families with von Hippel-Lindau disease revealed a high prevalence of islet cell tumors and the frequent coexistence of islet cell tumors and pheochromocytomas. This latter finding supports a continuum of the multiple endocrine neoplastic syndromes.

Three patients with profuse diarrhea, hypokalemia, metastatic nonbeta islet cell carcinoma, and the absence of gastric hypersecretion were found to have elevated levels of vasoactive intestinal peptide. One patient also had elevated serum gastrin levels. Two patients experienced prolonged remissions of diarrhea after operations in which only tumor biopsies were done. These cases further implicate vasoactive intestinal peptide as the agent mediating the diarrhea in the syndrome of pancreatic cholera.

Thirty-five CT scans and 23 sonograms of 45 pathologically proved pancreatic cystic neoplasms (16 microcystic adenomas, 17 mucinous [macrocystic] cystadenomas, and 12 mucinous [macrocystic] cystadenocarcinomas) in 43 patients were retrospectively and blindly reviewed. Radiologic findings and their usefulness in differentiating microcystic from mucinous subtypes were assessed. The number of cysts within the tumors (more than six in microcystic adenomas and six or fewer in mucinous cystadenomas and cystadenocarcinomas) and the diameter of the majority of cysts within the tumor (less than or equal to 2 cm in microcystic adenomas and greater than 2 cm in mucinous tumors) were the most helpful radiologic findings in differentiating tumor types. Calcification was present in 38% of microcystic adenomas, 18% of mucinous cystadenomas, and 8% of mucinous cystadenocarcinomas by CT. Calcification was not definitely identified on any of the sonograms. A central scar was identified in only two (13%) of 16 microcystic adenomas. Blind retrospective review was often able to correctly classify tumors as either microcystic (CT, 93%; sonography, 78%) or mucinous (CT, 95%; sonography, 93%). All tumors with a typical appearance on either CT or sonography were categorized correctly. Cystic pancreatic tumors may be difficult to prospectively separate from other types of pancreatic cysts. Assuming a cystic neoplasm is present, it often can be classified correctly as microcystic or mucinous (macrocystic) by using the above criteria.