- Email: [email protected]
Clinical presentation of neuroendocrine tumours: British Society of Gastroenterology (BSG) survey 1998–2000
1326 Epidemiological Study of Risk Factors for Gastric Carcinoids Edward A. Low, Brigham & Women's Hosp and VA Boston HealthcareSystem, Boston, MA; HarveyA. Risch, Yale Univ, New Haven, CT; Wong-Ho Chow, National Cancer Institute, Bethesda, MD; Marilie D. Gammon, Univ of North Carolina, Chapel Hill, NC; Thomas L. Vaughan, Fred Hutchinson Cancer Research Ctr, Seattle, WA; Janet B. Schoenberg, New Jersey Dept of Health and Senior Service, Trenton, NJ; Janet L. Stanford, Diana Farrow, Fred Hutchinson Cancer ResearchCtr, Seattle, WA; A. Brian West, NYU Medical Ctr, New York, NY; Heidi Rotterdam, Columbia Univ, New York, NY; Willliam J. Blot, Intl Epidemiology Institute, Rockville, MD; Joseph F. Fraumeni Jr, National Cancer Institute, Bethesda,MD
Registries were availablein 619 patients, diagnosedbetween1980 and 1997. ResultsBetween 1989 and 1996, overall incidence was 1 95/100,000 population. Under the age of 50 years a significant female predominancefor appendicealand lung carcinoids was observed. Under the age of 35 years appendicealcarcinoid was the most frequently diagnosed primary site. Incidence of distant metastases at diagnosis for appendix and lung primary sites was 1.6% and 5.5%, compared to 40% in the other primary sites. Survival analysis of 619 patients revealedthat age, stage and appendix Iocalisation independentlypredicted survival. In metastatic disease however, year of diagnosis after 1992 was the only independentpredictor of survival (p =0.012). Conclusions The female predominancefound under the age of 50 years suggests a hormonal influence. Improved survival in metastatic carcinoid disease may be related to the nationwide introduction and use of octreotide for carcinoid syndrome related complaints since 1992.
Background: Recent studies suggest that the incidence of gastric carcinoids has increased over the last 20 years. Although gastric carcinoids may occur in condrdons associatedwith hypergastrinemia such as chronic atrophic gastritis or Zollinger Ellison Syndrome, little is known about other risk factors for this tumor, if is also unclear whether the use of add suppressive medications which often leads to mild elevations in gastrin levels increasesthe risk of developinggastric Carcinoids.Methods: We studied 22 patients ~ newiy-d/agonsed gastric carcinoids who were identified as part of a population-basedcase-control study of gastric and esophagealcancersconducted in Connecticut,New Jersey,and Washington State, Two hundred randomly selected population controls were matched to the cases by age, gender, and study center. Face-to-faceinterviews were conducted to obtain information on medication use, dietary history, lifestyle and other potential risk factors. Adjusted odds ratios (OR) with 95 percent confidence intervals (CI) were calculated by multivariate conditional logistic regressionanalysesfor matcheddata. Results: Chronic use of histamine2 antagonists was not significantly associated with risk of gastric carcinoids (OR, 1.02 per year of use; 95% CI, 0.81 to 129). None of the cases had been on proton pump inhibitor therapy of at least 1 year duration prior to diagnosis. However, regular aspirin use appeared to convey increased risk (OR, 5.00; 95% CI, 1.23 to 20.41). A prior history of gastric ulcers was also nonsigniticantiy associatedwith cancer risk (OR, 3.07; 95% CI, 0.35 to 26.61). Interestingly, dietary intakes of soluble fiber (OR for trend, 0.23 per 3 gram/day consumed; 95% CI, 0.09 to 0.61) and Vitamin C (OR for trend, 0.30 per 90 rag/day consumed; 95% CI, 0.12 to 0.76) were significantly associatedwith a reduced risk for this tumor. Conclusions:Chronic use of histamine2 antagonists and a history of gastric ulcers were not significantly associatedwith risk of gastdc caroinoids, whereas regular aspirin use did seem to be associated with risk. Dietary intakes of soluble fiber and Vitamin C appearedto lower dsk. Although these are novel findings, it is important to emphasizethat gastric carcinoids are overall very rare and that additional larger studies are neededto confirm these results.
lm Prima of IMmafy Small Bowel Malignancy in the UK-a Praspectiva National s m ~ o ~ t4 math= ( l m - Z ~ ) P K. Ja/al, St Jamas's Univ Hosp, Leeds United Kingdom; G K T. Holmes, Derbyshire Royal Infirmary, Derby United Kingdom; R S. Houlston, Institute of Cancer Research, Surrey United Kingdom; P D. Howdle, St James's Univ Hosp, Leeds United Kingdom Objective: Primary small bowel malignancy is rare and little is known about its incidence in the UK. This prospective survey aimed to obtain information regarding its presentationand incidence.Study design:A four-step datacollection procedurewas adopted underthe auspices of the British Society of Gastroenterologyand data was collected over a 24 month period (June 98-May 00). Step 1: A card was posted by the BSG Research Unit to consultant physicians, surgeons, histopathologists and paedlatricianson the BSG databaseevery month for the reporting of any case of primary small bowel malignancy,Step 2: Following a positive report, a preliminary form was sent to the reporting consuitant requesting an anonymous identifier and the type of small bowel malignancy. Step 3: A detailed questionnaire was sent to these consultantS asking for specific clinical details of reported patients. Step 4: Contact by telephonewas madeto obtain clarification of details. Results:A mean of 1362 cards (13171428) was sent out each month; the mean response rate of returned cards was 45%. A total of 552 cases were reported (mean 23 per month). The preliminary form was returned in 424 cases (77%). Primary small bowel malignancy was confirmed in 373 cases (88%) and 51 (12=/o) were excludeddue to incorrect diagnosis or duplication in reporting, Adenocarcinoma was diagnosed in 163 patients (44%), lymphoma in 99 (26%), carcinoid in 79 (21%) and leiomyocarcoma in 32 (9%). Detailed information was obtained in 282 (83%) of the 341 cases of edonncardnoma, lymphoma and carcinoid. The number of patients reported in each year was similar. Accepting that there was possible under-reporting of cases, the annual incidence rate of primary small bowel malignancy in the UK was calculated to be 3.4 per million population. The commonest type was adenocarcinomawith an annual incidence rate of 1.5 per million, followed by lymphoma (annual incidence rate of 0.9 per million) and carcinoid (annual incidence rate of 0.72 per million). A significant number of cases presented acutely (56%, 44% and 47% in patients with edenocarcinoma, lymphoma and carcinoid respectively). Patients with adenncarcinoma and carcinoio had a hatter survival rate (85% in both) compared to those with lymphoma (62%). Further clinical information about these tumours will be presented.Conclusion: Primary small bowel malignancyis rare. This national data collection system has been useful in obtaining clinical data in a large number of patients with a rare diagnosis.
1327 Clinical Presentation of Neuraendocdne Turnouts: British Society of Gastroenterology (BSG) Sunmy 1998-2000. Martin W. James, Nottingham City Hosp, Nottingham United Kingdom; Sarah Mian, British Society of GastroenterologyResearch Unit, London United Kingdom; Richard F.A. Logan, Nottingham Univ Hosp, Nottingham United Kingdom; Richard G. Long, Nottingham City Hosp, Nottingham United Kingdom BACKGROUNDNeuroendocrinefumours can secrete hormomes that have an effect on the gastrointestinal tract, producing classical syndromes. Clinical series have demonstratedthe rarity of these tumoura yet they presenta considerablediagnostic challenge.The BSG research unit surveillance scheme offered the opportunity to identity a representativeseries of such cases. METHODSIncident cases diagnosed from June 1998 to June 2000 were notified on the monthly report card distributed to all consultant gastroenterologistsand gastrointestinal surgeons in the United Kingdom.The reporting doctor then completeda questionnairedetailing the age and clinical presentationof the patients,tumour site, hormone assaylevel, and method of treatment. Survival data were obtained at the end of the study period. RESULTS244 cases were reported, including 32 prevalentcases and 2 inappropriatetumoura. 57% of these cases had questionnairescompleted,producing 106 casesfor analysis.64 had symptoms attributable to the hormonal effects o1 the tumours, such as peptic ulceration or diarrhoea, and 42 had non-hormonal symptoms such as bowel obstruction, or extra-gastrointeetinalsymptoms such as rash or confusion. The commonest tumours producing hormonal GI symptoms were carcinoids (n=44,69%), presenting with diarrhoea as the main symptom in 83% Other symptoms included flushing, weight loss, or abdominal pain. 80% had liver metastases demonstrated at presentation. Gastrinomas accounted for 20% (n=13) of the tumours, presenting with peptic ulceration in 77%, and diarrhoeain 62%. Other tumoura identified were VIPomas, phaeochromocytomas,thyroid medullary tumoura, insulinomas, glucagonomas, somatostatinomas, parathyroid tumoura, and spindle cell tumoura. 78% of all patients were alive at the end of the study period. CONCLUSIONSCarcinoid syndrome was the commonest neoroendncdnecondition causing gastrointestinalsymptoms. Diarrhoeaappearsto be a more common presenting symptom of gastrinomas than has previously been appreciated.With a series as large as in any previous UK survey, the BSG monthly surveillance system shows promising potential.
Adenomyoma of the Vaterian System. Clinical and Morphological Characteristics of 11 Cases and Discussion of Histogenesis Adnana Handra-Luca, Benoit Terris, Anne Couvelard,Georgas Kharss, Hosp 8eaujon, Clichy France; Helene Bonte, Hosp Saint-Antoine, Paris France; Claude Degott, Hosp Beaujon, Clichy France; Jean-Francois Flejou, Hosp Saint-Antoine, Paris France BACKGROUND:Adenomyomais a benign lesion of the hepatobillaryand gastrointestinaltraCt, with most casesdescribedthe gallbladder.Amongtumors of the vateriansystem, adenomyoma is an exceptional benign, tumor-like lesion as only single cases have been reported in the literature (the total number of reported cases does not exceed20). OBJECTIVE: The study presents the main clinical and morphological cbaracteristics of 11 cases of adenomyomaof the vatedansystem. The histogenesisof this rare lesion is discussed. METHODS: A retrospective study of clinical, morphological, histochemical(PAS and Alcian blue stains) and immunohistochemical characteristics (Mib 1, smooth muscle actin) of 11 cases of adenomyoma surgically resected between 1989-2000 was made. RESULTS : The age of the patients was between 39 and 78 years and the sex ratio male:female = 5:6. In 2 cases the tumour was an incidental finding on echoendoscopy,in the other casesthe main clinical complaints were jaundice and abdominal pain. In all casesthe preoperativediagnosis was of ampul/arytumour and all cases were treated by pancreaticoduodenalresection. Macroscopically, there was a white, firm lesion whose size ranged between 10 and 30 mm, involving the ampullary region and stenosing the common bile duct. In 5 cases congenital anomaliesof the pancreaswere observed: pancreatic heterotopy in the duodenal wall (2 cases), pancreas divisum (2 cases) and annular pancreas (1 case). At microscopy, the lesion consisted in multiple Iobules composed of g/ands, sometimes cystically dilated, surrounded by a myofibroblastic proliferation and resulting in a "pseudohypertrophy" of the vatefian system. The glandular cells stained with PAS and Alcian blue and showed low proliferative activity (Mibl index). The myotibroblastic component consisted variable proportions of cells expressingsmooth muscle actin. CONCLUSION : The location in the vaterian system of adenomyoma, is of clinical importance because, in spite of its benign nature and small size, it is associatedwith biliary obstruction and is usually confused with carcinoma, often leading to extensivesurgery. Preoperative diagnosis on biopsy or frozen section specimen could lead to a limited surgical resection instead of pancreaticoduodenalresection.
1328 Epidemiology and Survival in Patients with Carcinoid Disease in The Nofhodands. An Epidemiological Study with 2391 Patients. Pascal F.H.J. Quaedvlieg,NKI/AvL, Amsterdam Netherlands;Otto Visser, IKA, Amsterdam Netherlands; Cornelis B.H.W Lamers, LUMC, Leiden Netherlands; Hank Boot, Babs G. Tsal, NKI/AvL, Amsterdam Netherlands Background Carcinoidtumors are rare malignant neuro-endncrinetumors. In 1992 octreotide has been introduced in The Netherlands as palliative treatment for the carcinoid syndrome in carcinoid disease.The aims of this epidemiologicalstudy were to evaluateepidemiological data and the impact of octreotide on survival in metastatic carcinoiq disease. Methods The database of the National Cancer Registry revealed 2391 patients with caroinoid disease, diagnosedbetween 1989 and 1996. Survival data from the Eindhovenand Amsterdam Cancer
A-256
Registries were availablein 619 patients, diagnosedbetween1980 and 1997. ResultsBetween 1989 and 1996, overall incidence was 1 95/100,000 population. Under the age of 50 years a significant female predominancefor appendicealand lung carcinoids was observed. Under the age of 35 years appendicealcarcinoid was the most frequently diagnosed primary site. Incidence of distant metastases at diagnosis for appendix and lung primary sites was 1.6% and 5.5%, compared to 40% in the other primary sites. Survival analysis of 619 patients revealedthat age, stage and appendix Iocalisation independentlypredicted survival. In metastatic disease however, year of diagnosis after 1992 was the only independentpredictor of survival (p =0.012). Conclusions The female predominancefound under the age of 50 years suggests a hormonal influence. Improved survival in metastatic carcinoid disease may be related to the nationwide introduction and use of octreotide for carcinoid syndrome related complaints since 1992.
Background: Recent studies suggest that the incidence of gastric carcinoids has increased over the last 20 years. Although gastric carcinoids may occur in condrdons associatedwith hypergastrinemia such as chronic atrophic gastritis or Zollinger Ellison Syndrome, little is known about other risk factors for this tumor, if is also unclear whether the use of add suppressive medications which often leads to mild elevations in gastrin levels increasesthe risk of developinggastric Carcinoids.Methods: We studied 22 patients ~ newiy-d/agonsed gastric carcinoids who were identified as part of a population-basedcase-control study of gastric and esophagealcancersconducted in Connecticut,New Jersey,and Washington State, Two hundred randomly selected population controls were matched to the cases by age, gender, and study center. Face-to-faceinterviews were conducted to obtain information on medication use, dietary history, lifestyle and other potential risk factors. Adjusted odds ratios (OR) with 95 percent confidence intervals (CI) were calculated by multivariate conditional logistic regressionanalysesfor matcheddata. Results: Chronic use of histamine2 antagonists was not significantly associated with risk of gastric carcinoids (OR, 1.02 per year of use; 95% CI, 0.81 to 129). None of the cases had been on proton pump inhibitor therapy of at least 1 year duration prior to diagnosis. However, regular aspirin use appeared to convey increased risk (OR, 5.00; 95% CI, 1.23 to 20.41). A prior history of gastric ulcers was also nonsigniticantiy associatedwith cancer risk (OR, 3.07; 95% CI, 0.35 to 26.61). Interestingly, dietary intakes of soluble fiber (OR for trend, 0.23 per 3 gram/day consumed; 95% CI, 0.09 to 0.61) and Vitamin C (OR for trend, 0.30 per 90 rag/day consumed; 95% CI, 0.12 to 0.76) were significantly associatedwith a reduced risk for this tumor. Conclusions:Chronic use of histamine2 antagonists and a history of gastric ulcers were not significantly associatedwith risk of gastdc caroinoids, whereas regular aspirin use did seem to be associated with risk. Dietary intakes of soluble fiber and Vitamin C appearedto lower dsk. Although these are novel findings, it is important to emphasizethat gastric carcinoids are overall very rare and that additional larger studies are neededto confirm these results.
lm Prima of IMmafy Small Bowel Malignancy in the UK-a Praspectiva National s m ~ o ~ t4 math= ( l m - Z ~ ) P K. Ja/al, St Jamas's Univ Hosp, Leeds United Kingdom; G K T. Holmes, Derbyshire Royal Infirmary, Derby United Kingdom; R S. Houlston, Institute of Cancer Research, Surrey United Kingdom; P D. Howdle, St James's Univ Hosp, Leeds United Kingdom Objective: Primary small bowel malignancy is rare and little is known about its incidence in the UK. This prospective survey aimed to obtain information regarding its presentationand incidence.Study design:A four-step datacollection procedurewas adopted underthe auspices of the British Society of Gastroenterologyand data was collected over a 24 month period (June 98-May 00). Step 1: A card was posted by the BSG Research Unit to consultant physicians, surgeons, histopathologists and paedlatricianson the BSG databaseevery month for the reporting of any case of primary small bowel malignancy,Step 2: Following a positive report, a preliminary form was sent to the reporting consuitant requesting an anonymous identifier and the type of small bowel malignancy. Step 3: A detailed questionnaire was sent to these consultantS asking for specific clinical details of reported patients. Step 4: Contact by telephonewas madeto obtain clarification of details. Results:A mean of 1362 cards (13171428) was sent out each month; the mean response rate of returned cards was 45%. A total of 552 cases were reported (mean 23 per month). The preliminary form was returned in 424 cases (77%). Primary small bowel malignancy was confirmed in 373 cases (88%) and 51 (12=/o) were excludeddue to incorrect diagnosis or duplication in reporting, Adenocarcinoma was diagnosed in 163 patients (44%), lymphoma in 99 (26%), carcinoid in 79 (21%) and leiomyocarcoma in 32 (9%). Detailed information was obtained in 282 (83%) of the 341 cases of edonncardnoma, lymphoma and carcinoid. The number of patients reported in each year was similar. Accepting that there was possible under-reporting of cases, the annual incidence rate of primary small bowel malignancy in the UK was calculated to be 3.4 per million population. The commonest type was adenocarcinomawith an annual incidence rate of 1.5 per million, followed by lymphoma (annual incidence rate of 0.9 per million) and carcinoid (annual incidence rate of 0.72 per million). A significant number of cases presented acutely (56%, 44% and 47% in patients with edenocarcinoma, lymphoma and carcinoid respectively). Patients with adenncarcinoma and carcinoio had a hatter survival rate (85% in both) compared to those with lymphoma (62%). Further clinical information about these tumours will be presented.Conclusion: Primary small bowel malignancyis rare. This national data collection system has been useful in obtaining clinical data in a large number of patients with a rare diagnosis.
1327 Clinical Presentation of Neuraendocdne Turnouts: British Society of Gastroenterology (BSG) Sunmy 1998-2000. Martin W. James, Nottingham City Hosp, Nottingham United Kingdom; Sarah Mian, British Society of GastroenterologyResearch Unit, London United Kingdom; Richard F.A. Logan, Nottingham Univ Hosp, Nottingham United Kingdom; Richard G. Long, Nottingham City Hosp, Nottingham United Kingdom BACKGROUNDNeuroendocrinefumours can secrete hormomes that have an effect on the gastrointestinal tract, producing classical syndromes. Clinical series have demonstratedthe rarity of these tumoura yet they presenta considerablediagnostic challenge.The BSG research unit surveillance scheme offered the opportunity to identity a representativeseries of such cases. METHODSIncident cases diagnosed from June 1998 to June 2000 were notified on the monthly report card distributed to all consultant gastroenterologistsand gastrointestinal surgeons in the United Kingdom.The reporting doctor then completeda questionnairedetailing the age and clinical presentationof the patients,tumour site, hormone assaylevel, and method of treatment. Survival data were obtained at the end of the study period. RESULTS244 cases were reported, including 32 prevalentcases and 2 inappropriatetumoura. 57% of these cases had questionnairescompleted,producing 106 casesfor analysis.64 had symptoms attributable to the hormonal effects o1 the tumours, such as peptic ulceration or diarrhoea, and 42 had non-hormonal symptoms such as bowel obstruction, or extra-gastrointeetinalsymptoms such as rash or confusion. The commonest tumours producing hormonal GI symptoms were carcinoids (n=44,69%), presenting with diarrhoea as the main symptom in 83% Other symptoms included flushing, weight loss, or abdominal pain. 80% had liver metastases demonstrated at presentation. Gastrinomas accounted for 20% (n=13) of the tumours, presenting with peptic ulceration in 77%, and diarrhoeain 62%. Other tumoura identified were VIPomas, phaeochromocytomas,thyroid medullary tumoura, insulinomas, glucagonomas, somatostatinomas, parathyroid tumoura, and spindle cell tumoura. 78% of all patients were alive at the end of the study period. CONCLUSIONSCarcinoid syndrome was the commonest neoroendncdnecondition causing gastrointestinalsymptoms. Diarrhoeaappearsto be a more common presenting symptom of gastrinomas than has previously been appreciated.With a series as large as in any previous UK survey, the BSG monthly surveillance system shows promising potential.
Adenomyoma of the Vaterian System. Clinical and Morphological Characteristics of 11 Cases and Discussion of Histogenesis Adnana Handra-Luca, Benoit Terris, Anne Couvelard,Georgas Kharss, Hosp 8eaujon, Clichy France; Helene Bonte, Hosp Saint-Antoine, Paris France; Claude Degott, Hosp Beaujon, Clichy France; Jean-Francois Flejou, Hosp Saint-Antoine, Paris France BACKGROUND:Adenomyomais a benign lesion of the hepatobillaryand gastrointestinaltraCt, with most casesdescribedthe gallbladder.Amongtumors of the vateriansystem, adenomyoma is an exceptional benign, tumor-like lesion as only single cases have been reported in the literature (the total number of reported cases does not exceed20). OBJECTIVE: The study presents the main clinical and morphological cbaracteristics of 11 cases of adenomyomaof the vatedansystem. The histogenesisof this rare lesion is discussed. METHODS: A retrospective study of clinical, morphological, histochemical(PAS and Alcian blue stains) and immunohistochemical characteristics (Mib 1, smooth muscle actin) of 11 cases of adenomyoma surgically resected between 1989-2000 was made. RESULTS : The age of the patients was between 39 and 78 years and the sex ratio male:female = 5:6. In 2 cases the tumour was an incidental finding on echoendoscopy,in the other casesthe main clinical complaints were jaundice and abdominal pain. In all casesthe preoperativediagnosis was of ampul/arytumour and all cases were treated by pancreaticoduodenalresection. Macroscopically, there was a white, firm lesion whose size ranged between 10 and 30 mm, involving the ampullary region and stenosing the common bile duct. In 5 cases congenital anomaliesof the pancreaswere observed: pancreatic heterotopy in the duodenal wall (2 cases), pancreas divisum (2 cases) and annular pancreas (1 case). At microscopy, the lesion consisted in multiple Iobules composed of g/ands, sometimes cystically dilated, surrounded by a myofibroblastic proliferation and resulting in a "pseudohypertrophy" of the vatefian system. The glandular cells stained with PAS and Alcian blue and showed low proliferative activity (Mibl index). The myotibroblastic component consisted variable proportions of cells expressingsmooth muscle actin. CONCLUSION : The location in the vaterian system of adenomyoma, is of clinical importance because, in spite of its benign nature and small size, it is associatedwith biliary obstruction and is usually confused with carcinoma, often leading to extensivesurgery. Preoperative diagnosis on biopsy or frozen section specimen could lead to a limited surgical resection instead of pancreaticoduodenalresection.
1328 Epidemiology and Survival in Patients with Carcinoid Disease in The Nofhodands. An Epidemiological Study with 2391 Patients. Pascal F.H.J. Quaedvlieg,NKI/AvL, Amsterdam Netherlands;Otto Visser, IKA, Amsterdam Netherlands; Cornelis B.H.W Lamers, LUMC, Leiden Netherlands; Hank Boot, Babs G. Tsal, NKI/AvL, Amsterdam Netherlands Background Carcinoidtumors are rare malignant neuro-endncrinetumors. In 1992 octreotide has been introduced in The Netherlands as palliative treatment for the carcinoid syndrome in carcinoid disease.The aims of this epidemiologicalstudy were to evaluateepidemiological data and the impact of octreotide on survival in metastatic carcinoiq disease. Methods The database of the National Cancer Registry revealed 2391 patients with caroinoid disease, diagnosedbetween 1989 and 1996. Survival data from the Eindhovenand Amsterdam Cancer
A-256