Incidence Of Carcinoid Tumors Research Articles

Anesthetic key points in a patient with a terminal ileum neuroendocrine tumor and a rare carcinoid left heart disease presented for non-cardiac surgery: case report

BackgroundCarcinoid tumors are rare neuroendocrine malignancies presenting in an increasing number in our center. The incidence of carcinoid tumors is approximatively between 2.5 and 5 cases per 100,000 people of whom about 50% develop carcinoid syndrome. Once the carcinoid syndrome has developed, a carcinoid cardiomyopathy can occur. Carcinoid heart disease (CaHD) remains a serious and rare complication associated with a significant increase in morbidity and mortality. Although carcinoid tumors have been known and studied for several years, there are still scarce data on the anesthetic management and the peri operative period.Case presentationWe describe a case of a Caucasian 44-year-old woman with an unusual presentation of left CaHD with an ileal neuroendocrine tumor and liver metastases. Our preoperative somatostatin administration protocol, limit the cardiac damage. The maintenance of stable hemodynamics, the use of balanced anesthetic technique, all along with a good understanding of the pathology, played a major role in the successful management of anesthesia. This case report allows us to introduce our decision algorithm for the management of this type of pathology in our tertiary hospital, Cliniques Universitaires Saint-Luc.ConclusionDespite the paucity of data, anesthetic management of patients with carcinoid tumor can be safely performed with effective hemodynamic monitoring and a good understanding of the pathophysiology. Knowledge and application of a clear institutional algorithm for octreotide administration and multidisciplinary consultation at a referral center are essential for the management of these patients.

Inflammatory bowel disease and coexistence of neuroendocrine neoplasms: A review of the literature.

e16206 Background: Inflammatory bowel disease (IBS) has been associated with colorectal adenocarcinoma, but its co-occurrence, causal or coincidental, with the more rare neuroendocrine tumor (NET) histology is not well understood.The incidence of carcinoid tumors has been increasing and chronic inflammation can be theoretically associated with carcinogenesis, including the development of carcinoid tumors. We aim to describe the reported literature on patients with IBS who also developed NET and summarize the characteristic findings reported in eachcase. Methods: This is a descriptive retrospective review of all cases of patients with IBD who developed NET reported in literature. We performed a Pubmed search with select keywords for IBS and NET, including ‘Inflammatory bowel disease’, ‘Ulcerative colitis’, ‘Crohn’s disease, and ‘Neuroendocrine Tumors’, or ‘carcinoid’. Extracted information included age, gender, inflammatory bowel disease type, number of years with IBD, treatment received for IBD, tumor site, indication for surgery, the extent of IBD, coexisting tumors, pathological findings, distant metastasis at diagnosis, and survival. Results: Out of 539 articles we identified 87 cases reports between 1970 and 2021. The mean age of patients was 43.61 years. IBS included ulcerative colitis in 46% (40) and Crohn’s disease in 51.7% (45) of patients. The male to female ratio was 1.7:1. The median number of years with IBS before diagnosis of NET was 12.5 years.Of the patients, 36.6% percent (31) were reported to receive treatment for IBS; of these 61.3%(19) were treated with steroids, 42% (13)mesalamine, 29%(9) azathioprine, 12.9% (4) infliximab, 6.4%(2) adalimumab, 3.2%(1) methotrexate, and 3.2% (1) mercaptopurine. Three patients 3.4% (3) initially underwent colectomy with ileostomy. IBS was diffuse in 20.7%(18) of the cases. The ileum was involved in a total of 35.6% (31) and the rectum in 4.6%(4). A mass was found upon regular screening imaging or regular surveillance in 8%(7) of the cases. The NET site was in the rectum in 20.7%(18), in the ileum 19.5%(17), and in the appendix in 19.5%(17). The indication for surgical intervention was due to refractory symptoms in 19.5% (7) of the cases and obstruction in 12.6%(11). About 35.6%(31) of NET cases were reported as well-differentiated, 5.7% (5) as poorly differentiated. Grade was high in 3.4% (3) and low in 5.7%(5) of the cases. Metastasis was reported to lymph nodes in 11.5% (10) and the liver in 5.7%(5). 13.8%(12) had a coexisting adenocarcinoma and 11.5%(10) died from NET. Conclusions: Most NETs in patients with IBS are in younger patients with non-diffuse disease, take a considerable time to develop, are low grade and localize in ileum, appendix and rectum. It is important to consider NET in patients with IBD who develop refractory or obstructive symptoms, especially when considering imaging / endoscopies to assess IBS status and response.

Incidence of carcinoid tumors in appendectomy specimens: Review of 4485 cases

Incidence of carcinoid tumors in appendectomy specimens: Review of 4485 cases

S2806 A Case of Ileocecal and Distal Sigmoid Carcinoid Tumor With Liver Metastasis

INTRODUCTION: Carcinoid tumors are rare, slow-growing neuroendocrine tumors (NET), often asymptomatic and may not become apparent until there is evidence of metastatic disease or carcinoid syndrome. Digestive involvement is most common in the appendix, mesentery and the ileum. We present a case of carcinoid tumor in the ileocecal valve and distal sigmoid colon, complicated by liver metastasis. CASE DESCRIPTION/METHODS: A 55 year-old African-American with no significant past medical history, presents for eight months of progressive weight loss, subjective fever, chronic watery diarrhea and episodes of flushing. Physical exam was significant for thin habitus. Laboratory tests revealed macrocytic anemia and progressive increase in transaminases (ALT 224 U/L, AST 217 U/L). Right upper quadrant US was showed scattered small indeterminate liver lesions of 1.5 cm. CT abdomen/pelvis revealed a 2.3 × 2.1 cm focal mass and thickening of the terminal ileum and a 2.1 cm mass in the distal sigmoid colon, in addition to multiple liver lesions. Percutaneous liver biopsy consistent with two minute clusters of NET cells at 0.15 mm and 0.10 mm showed positive immunohistochemistry (IHC) positive for synaptophysin an chromogranin. Further laboratory investigation included elevated urine 5-HIAA (60 mg/24 h) and elevated levels of serum chromogranin (167 ng/mL). Colonoscopy with terminal ileum intubation showed a 7 mm pedunculated broad based polyp in the sigmoid colon and a protruding polypoid irregular ileocecal valve. Histopathology from colonic mucosa showed non-specific chronic inflammation and immunoreactivity for CD45, which supports the diagnosis of NET. The patient was treated with Sandostatin injections monthly with significant clinical improvement, and is followed by Oncology and Gastroenterology services. DISCUSSION: Carcinoid tumors although rare, can be found throughout the gastrointestinal tract, generally in the appendix or ileum. Our patient presented with clinical and immunohistochemical characteristics consistent with carcinoid tumor, and apparent unusual localization in the ileocecal valve and distal sigmoid colon. Associated liver metastasis was evident. Recent literature reports that the overall incidence of carcinoid tumors has been steadily increasing likely due to advances in testing, however most cases are being detected after wide-spread disease.CONCLUSION. Further description of the biological and histopathological characteristics of carcinoid tumors is needed for implementation of early detection techniques.

Usefulness of Capsule Endoscopy and Double-balloon Enteroscopy for the Diagnosis of Multiple Carcinoid Tumors in the Small Intestine: Case Reports and a Literature Review

The incidence of carcinoid tumor in the small intestine is increasing; however, its preoperative diagnosis is difficult. We recently experienced three cases of multiple carcinoid tumors in the small intestine successfully detected using capsule endoscopy (CE), followed by a pathological diagnosis using double-balloon enteroscopy (DBE). To diagnose multiple carcinoid in the small intestine appropriately, we reviewed the information of five cases reported to date along with our three recent cases. The literature review demonstrated that CE and DBE are useful for detecting and diagnosing small intestinal carcinoids and tumor multiplicity, which aids in determining the appropriate resection range.

Metastatic carcinoid tumor with severe double valve disease at diagnosis: Case report and review of literature

A 27-year-old woman with a history of anxiety presented with three months of diarrhea. Onphysical examination, she had a grade 3/6 systolic heart murmur at the lower left sternal borderwhich increased with inspiration, a grade 2/6 decrescendo diastolic murmur at the left sternalborder, and lower extremity edema. Stool studies were negative. The abdominal ultrasounddemonstrated multiple complex liver cysts. Transthoracic echocardiogram reported tricuspidvalvular thickening with stenosis and regurgitation, severe pulmonary stenosis, and mild aorticregurgitation with valvular thickening. 5-hydroxyindole acetic acid and chromogranin A levelswere elevated. The patient’s symptoms improved after octreotide therapy, and she underwenttricuspid and pulmonary valve replacement.The incidence of carcinoid tumors is 1.2 to 2.1 per 100,000 people in the general population.Carcinoid heart disease occurs in one-half to two-thirds of patients with carcinoid syndromeand is associated with poorer clinical outcomes. Left side cardiac involvement occurs in lessthan 10% of patients. The presence of both left and right sided valvular disease in the contextof gastrointestinal carcinoid is associated with severe and poorly controlled disease. Up to4% of these cases have metastatic disease on the valves, and 3.8% have direct myocardialinvolvement.

Carcinoid and Inflammatory Bowel Disease: Coincidence or Complication?

Purpose: It is well established that long-standing inflammatory bowel disease (IBD) is associated with an increased risk of small bowel and colorectal adenocarcinoma. The association of neuroendocrine tumors and IBD is less well understood. We report 2 cases of neuroendocrine tumors diagnosed in IBD patients admitted to our institution within days of each other. Case 1 involves a 44 year-old male with a 20-year history of Crohn's. After 8 years of abdominal pain, he developed a small bowel perforation requiring emergency laparotomy with ileocecal resection. Surgical pathology revealed a well-differentiated T2N1M0 carcinoid tumor measuring 12 mm. The remaining excised small bowel had extensive involvement with Crohn's disease with a fistula tract extending into the region of the appendix associated with an abscess and serositis. Post-operatively, his course was complicated by recurrent fistula formation and resultant malabsorption. Eight years after the initial resection, he had a positive octreotide scan but no further radiographic evidence of carcinoid recurrence. He began treatment with IM octreotide but continued to have high-output fistulae and 10 years after his initial surgery, he underwent resection of several fistulae and a previously placed jejunal-colic anastomosis. Small bowel pathology showed evidence of now metastatic, well differentiated neuroendocrine tumor. Case 2 involves a 49 year-old female with a 35-year history of untreated ulcerative colitis, admitted with bloody stools and abdominal pain. Biopsies obtained during diagnostic flexible sigmoidoscopy showed severe colitis and an incidental, well-differentiated neuroendocrine tumor measuring less than 4mm. Further radiographic investigation and repeat colonoscopy failed to reveal a primary carcinoid. Discussion: Gastrointestinal carcinoid tumors are rare, and the concomitant diagnosis of carcinoid and IBD is even rarer. It has been debated whether the incidence of carcinoid tumors is actually increased in patients with IBD or if there are simply more incidental carcinoids diagnosed in IBD patients who are more likely to require surgery for a complication of the intestinal inflammation. The two cases presented had active inflammation at the time the diagnosis of carcinoid tumor was made. They differ in the location of the carcinoid - the patient with Crohn's had evidence of carcinoid in the distal ileum and jejunum, both areas without inflammation, while the patient with ulcerative colitis had carcinoid tumor identified in a random distal colon biopsy. Both cases support the hypothesis that there may be an association between carcinoid and IBD, but Case 1 suggests carcinoids can arise in areas without chronic mucosal inflammation.

Carcinoide gastrointestinal: Experiencia de siete años en el Instituto Nacional del Cáncer (2000-2006)

Carcinoid tumors. Retrospective review of 25 patients Background: The incidence of carcinoid tumors (CT) has increased in the last fi ve decades. These su- pposedly benign tumors may not always behave as such. The largest series of CT show that the most common primary tumor site (appendiceal) changed over the years. Aim: To evaluate the management of gastrointestinal CT at the National Cancer Institute from 2000 to 2006, describe their anatomic location and estimate disease specifi c survival. Material and Methods: Review of the database of the Cancer Committee, during a period of 7 years, looking for patients with a pathological diagnosis of CT, whose clinical records were analyzed. Results: The records of 25 patients with gastrointestinal CT, aged 18 to 79 years (19 females) were analyzed. Five years disease specifi c survival was 91.1%. The most common location was appendiceal in 56% of ca- ses, with a 5 years disease specifi c survival of 100%. Right hemicolectomy was performed in seven patients with appendiceal carcinoid. Other seven patients only underwent appendectomy. Five years disease specifi c survival was 100% in both groups. Conclusions: The appendix is still the most common location of CT in this series and long term survival is acceptable.

Pulmonary tumours in the Netherlands: focus on temporal trends in histology and stage and on rare tumours

Background:Recent temporal trends in histology and stage of pulmonary tumours in the Netherlands were studied. The incidence of rare pulmonary tumours was determined.Methods:All tumours originating from the trachea, bronchus and...

Erfüllen Karzinoidtumoren der Lunge die Legaldefinition für den Begriff „Lungenkrebs” der Berufskrankheitenverordnung? – Untersuchungsergebnisse des Deutschen Mesotheliomregisters

Carcinoid tumours are considered to be malignant epithelial tumours according to the recent WHO classification. This study is based on the examinations of tissue from 108 patients with carcinoid tumours. Our data agree with those of other studies: carcinoid tumours developed mainly in the right lung (40 %) and the lower lobe (30 %), mean age was 56 years, typical carcinoid tumours (74 %) predominated, comparatively high proportion of females (32 %), the mean latency period after asbestos exposure - assuming asbestos as one causal factor - was 35 years. A higher incidence of carcinoid tumours (1.3 %) in the collective of the mesothelioma register compared to the incidence in the collective of all lung carcinomas (1 - 2 %) was not observed. The increased pulmonary asbestos burden analysed in 26 % of patients is explained by the exposure-dependent selection of patients in the register. So far, no association between smoking habits or exposure to other, i. e., occupational pollutions and the development of carcinoid tumours could be established. In the list of occupational diseases (No. 4104) the term "lung cancer" is used without further specification. Thus the following question remains open for discussion: does the term "lung cancer" include carcinoid tumours such as malignant epithelial lung tumours, or is it restricted to the common subtypes such as small cell carcinoma, squamous cell carcinoma, adenocarcinoma, large cell carcinoma with regard to occupational disease and compensation?

Pulmonary carcinoid tumours

<h3>Key points</h3> Pulmonary carcinoid tumours account for 2% of all lung tumours, with an increase in incidence due to more accurate diagnostic techniques. Carcinoid tumours are relatively slow growing, although prognosis depends on histological subtype; atypical carcinoids have worse prognosis Local pulmonary symptoms occur frequently Paraneoplastic syndromes are dependent on the release of hormones or vasoactive peptides into the systemic circulation. Carcinoid syndrome manifests with diarrhoea and flushing, which can lead to dehydration, hypotension and arrhythmias. Carcinoid heart disease is a severe late complication of carcinoid syndrome, manifesting as valvular right-side heart disease, and is a frequent cause of death. Urinary 5-HIAA and CgA are good biochemical markers of carcinoid tumours. Nuclear scintigraphy techniques with ¹¹¹Indium-pentetreotide and ¹³¹I-MIBG are very sensitive in the diagnosis of metastatic carcinoid tumour and are predictive of response to octreotide therapy. Somatostatin analogues are first-line treatment for carcinoid metastatic tumours. Systemic chemotherapy has limited success and is more effective in aggressive variants of carcinoid tumour; IFN-α therapy can also be used with good biochemical and symptomatic responses, but small effects on tumour reduction. <h3>Educational aims</h3> To describe the epidemiology and prognosis of pulmonary carcinoid tumours. To discuss the pathophysiology and clinical presentation of pulmonary carcinoid tumours. To outline the diagnostic procedures and treatment strategies in carcinoid metastatic disease. To highlight the importance of carcinoid-related heart disease and carcinoid syndrome in the prognosis of carcinoid tumours. <h3>Summary</h3> Carcinoid tumours are neuroendocrine tumours derived from enterochromaffin cells, which are widely distributed in the body. Pulmonary carcinoids account for 25% of all carcinoid tumours and they comprise 1–2% of all lung tumours. The incidence of carcinoid tumours seems to be sharply increasing. Carcinoid tumours can be classified as typical or atypical, ranging from low-grade malignant to high-grade malignant, according to histological characteristics and differentiation level. Carcinoids can be placed in a spectrum of neuroendocrine tumours, ranging from low-grade malignant typical carcinoid to intermediate atypical carcinoid, to highgrade large cell neuroendocrine carcinoma and small cell lung carcinoma. Carcinoid pulmonary tumours can present with lymph node or distant metastases, most frequently at the liver. Carcinoid tumours have the ability to secrete many vasoactive peptides, most frequently serotonin. The hormonal secretion is related to systemic manifestations of carcinoid syndrome and carcinoid heart disease. Combining new diagnostic and treatment modalities in metastatic carcinoid patients may result in better prognosis. The review aims to elucidate the reader on the many aspects of carcinoid pulmonary tumours.

A Large Pedunculated Rectal Carcinoid Tumor Resected by Endoscopic Snare Polypectomy

A 53 y/o asymptomatic black female with a history of HTN underwent a routine screening colonoscopy which revealed a 18mm pedunculated rectal polyp as well as a 10mm pedunculated transverse colon polyp. Both were removed by snare cautery. The rectal polyp was grossly atypical. It was pale, firm and nodular with irregular borders on a wide stalk. Pathology reported a rectal carcinoid tumor and a transverse colon tubular adenoma and. An endoscopic rectal ultrasound was performed three weeks later which revealed no invasion of the submucosa. Multiple jumbo biopsies were obtained from the polypectomy site all were negative for any residual carcinoid tumor tissue. Computed tomography of the chest, abdomen and pelvis were negative for metastasis. Discussion: Carcinoid tumors are derived from the neuroendocrine system. They have the capbility of secreting an array of biologically active agents. The incidence of carcinoid tumors has been estimated to be 8.4 per 100,000. 74% of carcinoid tumors are located in the gastrointestinal tract, the ileum, appendix and the rectum are the most common GI sites. The Bronchopulmonary system is responsible for the majority of the other sites. Grossly they are usually submucosal. They are usually yellow/tan or pale/gray secondary to a high lipid content, as well as firm and rubbery. Histologically they appear as nests, cords or rossettes of uniform round cells with rare mitotic figures. Immunohistochemically, they may be differentiated from other neuroendocrine tumors with chromogranins A, B, C, synaptophysin and enolase stains. They are best visualized endoscopicaly using EUS and by Somatostatin scintography, PET scans and CT scans. Tumors less than 1cm < 2% chance of mets, 1–1.9cm 10–15%, >2cm 60–80%. The overall survival rate of rectal carcinoid tumors is 72%. Their prognosis and treatment depend on a variety of factors including: location, size, degree of invasion and possibly by whether they are sessile or pedunculated. Pedunculated rectal carcinoid tumors are extremely rare. This may be the tenth reported case in medical literature. There have been recommendations regarding endoscpopic resection of flat, sessile rectal carcinoid tumors, however in our case of a pedunculated carcinoid tumor, more studies are needed to determine the prognosis and the most appropriate treatment.

Metastatic Carcinoid Tumors: A Clinical Review

Carcinoid tumors are neuroendocrine tumors derived from enterochromaffin cells, which are widely distributed in the body. They can originate from any location in the body, but they are traditionally described as originating from the foregut, midgut, and hindgut. Although the overall incidence of carcinoid tumors appears to have increased in the past decades, the prognosis for patients with metastatic carcinoid tumors has improved during the last decade. Due to longer survival times, complications, such as carcinoid heart disease, and new metastatic patterns, like skin and bone metastases, may become more important features of carcinoid disease. Therapy focused on these complications should be part of the management. Combining new diagnostic and treatment modalities in metastatic carcinoid patients may result in better quality of life and longer survival times. The increasing number of therapeutic options and diagnostic procedures requires a multidisciplinary approach, with decisions made in multidisciplinary meetings focused on "tailor-made" therapy based on patients' specific conditions. Because carcinoid tumors are uncommon, effort should be made to treat these patients in specialized centers and for these centers to join together in multicenter studies.

Cutaneous manifestations of the malignant carcinoid syndrome

The incidence of carcinoid tumours is approximately 1.5 per 100 000 of the population. The malignant carcinoid syndrome, which is caused by circulating neuroendocrine mediators produced by the tumour, occurs in less than 10% of patients. Cutaneous involvement, although recognized in this rare syndrome, has rarely been reported. To examine a series of patients with the malignant carcinoid syndrome, to establish the prevalence and types of cutaneous involvement and to assess whether these could be used as indicators of disease activity, progression and prognosis. Patients with the malignant carcinoid syndrome who attended a multidisciplinary clinic for neuroendocrine tumours over a 23-month period between February 2001 and December 2002 were invited to participate in the study. This involved completion of a standard history proforma and a detailed cutaneous examination with findings recorded by clinical photography. Twenty-five patients were enrolled. All but one had experienced flushing, three had rosacea, five had clinical features of pellagra and two had scleroderma. Flushing was generally an early manifestation of the syndrome, whereas both the pellagra and scleroderma tended to occur in more advanced disease. This descriptive case series indicates that cutaneous features are not uncommon in the syndrome. They are readily diagnosed on clinical examination, and may be useful indicators of disease activity and prognosis.

Carcinoid tumor and carcinoid syndrome

Carcinoid tumors secrete many different types of substances (e.g. serotonin, bradykinin) that may produce potentially fatal intraoperative reactions such as hypotension and bronchoconstriction. The most effective treatment for the deleterious cardiovascular and pulmonary effects of serotonin and bradykinin is octreotide, a somatostatin analogue. Carcinoid heart disease, which develops in the majority of patients with carcinoid syndrome, presents the anesthesiologist with more diagnostic and therapeutic dilemmas. The incidence of carcinoid tumors is much greater than previously recognized. New diagnostic techniques permit identification and localization of carcinoid tumors with greater accuracy. Short term and long term therapy with octreotide significantly improves survival and reduces the severity of 'carcinoid crises'. Echocardiographic studies have demonstrated a very high incidence of carcinoid heart disease (tricuspid and pulmonary insufficiency) in patients with carcinoid syndrome. Cardiac valve surgery is, consequently, becoming much more common in patients with carcinoid syndrome. More patients with carcinoid tumors and carcinoid syndrome are requiring anesthesia and surgery. Specific therapy with somatostatin analogues (octreotide) has replaced older less specific drugs (e.g. antihistamines) for the treatment of adverse effects caused by products of carcinoid tumors.

The management of carcinoid tumours.

The term ‘karzinoide’ was originally used by receptor subtypes, which are members of seven 5HT receptor groups distinguished according to their Obendorfer in 1907 to describe a carcinoma-like lesion without malignant qualities. These tumours coupling to particular signalling pathways and amino acid sequence identity.2 Apart from the 5HT3 recepare uncommon, and attract the interest of clinicians because of the spectacular symptoms that they cause. tor, which is a ligand-gated ion channel, they are all G-protein-coupled receptors. Stimulation of a These symptoms are so striking as to make clinical diagnosis obvious, but because of their range, unfornumber of the 5HT receptor subtypes has the potential to induce malignant transformation. Activation tunately result in patients being cared for by many different medical and surgical specialists. Carcinoid of rat 5-HT2c receptors induces malignant transformation of transfected N1H-323 cells.3 Activation tumours, even when there is metastatic spread, are generally thought to have a good prognosis and as of mouse 5HT2b receptors expressed in L+kD cells activates p21ras and p42mapk/p44mapk and leads to the a result, patients with this condition are managed symptomatically. development of 5HT2b-dependent 5HT-stimulated foci in confluent cells which are tumourigenic in However, Godwin et al. assessed survival in 2837 patients with carcinoids, and in patients with liver nude mice.4 metastases and raised urinary 5HIAA levels he reported a five-year survival of 18–38% and a median survival of 23 months.1 These figures suggest The treatment of metastatic carcinoid that carcinoid tumour is a rather more aggressive tumours disease than is generally appreciated. In this article we review recent clinical developments, make a The incidence of carcinoid tumours is 1.5, and the case for specialist care, and promote the view that prevalence of the carcinoid syndrome 0.5, per chemotherapy given with the aim of reducing tumour 100 000 population. Primary carcinoid tumours are bulk should have a more prominent position in the classified according to their embryonic site of origin therapeutic armamentarium. into foregut, midgut and hindgut tumours. The most common primary site is the appendix followed by the rectum. The carcinoid syndrome is most commonly due to metastatic midgut tumours, 50% of 5HT and its receptors which metastasize to the liver. Patients with carcinoid tumours may be treated The symptoms of carcinoid syndrome are caused by the tumours’ secretory products, and so palliative surgically, medically or radiologically. Where there is metastatic disease, surgery is generally limited to treatment has been designed to reduce circulating levels of these products, Serontonin, 5HT, is the those rare occasions where metastases are confined to one lobe of the liver. The majority of patients major secretory product of carcinoid tumours. Molecular cloning has identified 15 serotonin with metastatic midgut tumours have multiple meta-

The epidemiology of carcinoid tumours in England and Scotland.

Relatively little is known about the epidemiology of carcinoid tumours in contrast to the extensive information available on their biochemical effects and natural history. Accordingly, we have used cancer registrations in England from 1979 to 1987, and in Scotland from 1980 to 1989, to estimate the incidence of carcinoid tumours in Britain. Age-standardised incidence rates for England, based on 3,382 registrations, were 0.71 (0.68-0.75) for men and 0.87 (0.83-0.91) for women, per 100,000 per year. The equivalent rates for Scotland, based on 639 registrations, were 1.17 (0.91-1.44) for men and 1.36 (1.09-1.63) for women. There was a consistent female excess of carcinoid tumours in the reproductive years, which was reversed after the age of 50. The female excess was most striking for gastrointestinal carcinoid tumours in women aged 15-19 years (F:M ratio = 2.14). The sex differences are probably due in part to incidental diagnosis of carcinoid tumours during abdominal procedures, which are more common in women than men at ages 15-49 years. However, there is some evidence to suggest a true sex difference in incidence, particularly the fact that the sex ratio for thoracic tumours varies with age in a similar way to that for gastrointestinal tumours. Hormonal factors may, therefore, be important in the aetiology of carcinoid tumours.

The Frequency of Gastrointestinal Endocrine Tumours in a Well-defined Population – Northern Ireland 1970–1985

The reported incidence of gastrointestinal endocrine tumours is variable. In Northern Ireland circumstances allowing such an assessment are favourable with a central diagnostic laboratory and register established to collect data on tumours from a well-defined population of 1.5 million people. From 1970 to 1985, 368 cases were recorded of which 85 per cent were carcinoid tumours. The annual incidence of carcinoid tumours was 1.3 per 100,000 of the population and the majority occurred in the appendix (61 per cent). No patients presented with the carcinoid syndrome. The annual incidence for other tumours was 0.12 per 100,000 for insulinomas; islet cell tumours of unknown type 0.07; Zollinger-Ellison syndrome 0.05; and multiple endocrine neoplasia (MEN) 0.05. There were two cases of VIPoma, one glucagonoma, one neurotensinoma and one tumour producing ACTH. It is possible that some tumours are more uncommon than others because of difficulty in diagnosis.