Carcinoid tumours of the rectum (author's transl)

In three patients aged 21 to 65 rectal polyps diagnosed at rectoscopy were removed by diathermy loop. Histology showed them to be carcinoid tumours, one of tubular type, two of mixed type. Rectal carcinoid rarely presents with typical clinical symptoms. Polyps discovered by digital examination or rectoscopy should be considered to be potentially malignant and removed in toto. The size of the tumour is of decisive prognostic importance. Tumours with a cross section over 2 cm should be treated as malignant. The histological investigation generally gives no indication of clinical prognosis or of the possibility of metastases. Despite invasive growth and despite local or distant metastases carcinoid tumours can be judged more favourably than carcinoma. The plan of treatment should be according to the clinical findings as well, and not only to histological results. Postoperative follow-up is considered imperative.     

Neuroendocrine tumours in various organ systems in a ten-year period

Neuroendocrine cells are present in various organ systems. These widely distributed cells as well as their histogenetically related tumours can produce various peptides and peptide hormones. From 1984 to 1993, 349 neuroendocrine tumours were found among 511,382 histological diagnoses at the Institute of Pathology of the Medical School of the Karl Franzens University in Graz. In 30% carcinoid of the appendix was diagnosed, in 16% carcinoid of the colorectum, in 9% carcinoid of the small intestine and in 2% duodenal carcinoid. Carcinoid of the stomach was detected in 14%. Seven of these cases showed microcarcinoidosis and five of them were combined with an adenocarcinoma. Carcinoid of the oesophagus was present in 1%, neuroendocrine pancreatic tumours in 6%. Neuroendocrine tumours of the bronchial system were found in 12%, medullary thyroid cancer in 5%. In 1% a Merkel-cell tumour was diagnosed. Other more rare localizations of neuroendocrine tumours were the uterus, ovary, breast, testes, epididymis, anal region and the upper respiratory tract.     

Internal jugular to axillary vein bypass for subclavian vein thrombosis in the setting of brachial arteriovenous fistula

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.     

Metastasized carcinoid tumor treated with interferon and octreotide

The carcinoid tumours are rare forms of the gastrointestinal tumours (they make up 2%), though lately they have shown an upward tendency. Among different types of treatments of the carcinoid tumours in non metastatised cases, the surgical intervention stands first on the list. But when symptoms emerge, most of the tumours have metastases, for which there are various types of treatments. The authors introduce the combined therapeutical possibilities in connection with metastatised carcinoid tumours. A combination of octreotid and interferon is used in the course of the treatment. Authors introduce the therapy's indications and experiences gained during the follow up of events.     

Carcinoids of the small intestine. Retrospective study of the radiological diagnosis of 15 patients (author's transl)

The radiological workup of 15 patients with a carcinoid has been evaluated. As 7 of the 15 patients presented for emergency operation, only the remaining 8 had barium studies (7 barium meals and 1 barium enema). Two of the barium meals were even retrospectively, entirely negative. In two others, initially the diagnosis was missed despite radiological signs, which had been overlooked on follow-up films. Fluoroscopic examination of the small bowel would probably have ensured the detection of the lesion. At the time of diagnosis nine tenth of the tumours were already infiltrating and more than half of them metastasizing. The presence of the carcinoid syndrome implies the production of the causative hormones in the liver or the pulmonary circulation. In order to find the primary intestinal carcinoid in a cureable stage- that is, before it has metastasized-, all patients with diarrhoea and repeated bouts of abdominal pain should have a careful follow-up examination of the small bowel. This examination must include fluoroscopies.     

Estradiol receptors expression in nasal polyps and its significance

Nasal polyps are generally considered as a result of extreme nasal mucosal edema induced by long-term recurrent inflammation of the respiratory mucosa. The estradiol (E2) has been demonstrated to play a facilitating role in nasal inflammation. To evaluate the effect of estradiol on the pathogenesis of nasal polyps, the expression of E2 receptors in paraffin section from patients with nasal polyps (84 cases), chronic hypertrophic rhinitis (6 cases) and healthy control subjects were investigated by means of immunohistochemistry for E2 receptors and toluidine blue staining for mast cells. It was shown that there was high expression of E2 receptors in 61 out of 84 cases (male 40, female 44) of nasal polyps and the expression distributed equally among both sexes. Low expression of E2 receptors presents in 2 out of 6 cases of chronic rhinitis and 1 out of 4 healthy subjects. Noteworthily, the E2 receptor expressing cells are similar with the mast cells in shape and distribution. The authors speculate that they may be identical cells. E2 receptor expression in nasal polyps suggests that estradiol plays certain role in the development of nasal polyps.     

Cowden's disease--a report on the first case in Korea and literature review

Cowden's disease, or multiple hamartoma syndrome, is an uncommon condition with characteristic mucocutaneous lesions associated with abnormalities of the breast, thyroid, and gastrointestinal tract. We describe a 32-year-old man with oral mucosal papillomatosis and plantar hyperkeratosis as a definite case of Cowden's disease according to the criteria proposed by Salem and Steck. The patient also had a thyroid mass and numerous gastrointestinal polyps endoscopically. Histologically the polyps were hamartomatous or hyperplastic polyps. The oral papillary lesions were fibroepithelial polyps and the thyroid mass was a follicular adenoma. We review the literature on this entity and summarize the pertinent findings. To the best of our knowledge, this is the first documented case of Cowden's disease in a Korean.     

Carcinoid tumour

Carcinoid tumours are often indolent asymptomatic tumours. However, a small but significant proportion are malignant and difficult to manage. Multiple endocrine neoplasia type 1 (MEN-1) may be associated with carcinoid tumours and should therefore be considered in the investigation of these patients. This review puts into context the use of newer imaging modalities, including octreotide scintigraphy. The therapeutic treatment options are discussed, including the use of octreotide, the role of receptor-targeted therapy, hepatic-artery embolisation, and the arguments against chemotherapy. We review the need for careful patient selection when considering curative and palliative surgery, including liver transplantation. We conclude that there are now better diagnostic tools and therapeutic options available for those patients with malignant carcinoid tumours, and that these patients are best managed by a multidisciplinary approach. Earlier detection and treatment of these tumours should lead to improved quality of life and survival, which, ideally, should be assessed in formal trials.     

Psoriasiform onychopachydermoperiostitis of the large toes: the OP3GO syndrome

OBJECTIVE: To describe our experience with primary appendiceal tumours. DESIGN: Retrospective study. SETTING: University hospital, Israel. SUBJECTS: 2520 patients who had appendectomies during the 14 years, January 1982-December 1996. RESULTS: 22 patients 5 male and 17 female, mean age 56.2 years, had primary neoplasms; 14 were carcinoid tumours and villous adenomas and were treated by appendicectomy only. Adenocarcinoma was diagnosed in 8 patients (0.3%), 5 after appendicectomy (0.2%) which is twice the reported incidence. They were all treated by right hemicolectomy. Seven of the patients were classified as Dukes' B and one as Dukes' C. All patients were alive and disease-free after a mean follow-up period of 57.4 months. CONCLUSION: Right hemicolectomy is the treatment of choice for adenocarcinoma of the appendix.     

Somatostatin receptor scintigraphy of carcinoid tumours using the [111In-DTPA-D-Phe1]-octreotide

Somatostatin-receptor scintigraphy using the 111In-labelled somatostatin-analogue octreotide ([111In-DTPA-D-Phe1]-octreotide) was performed in 40 patients with carcinoid tumours. In 31/40 patients, this scintigraphy proved positive compared with the 33/40 patients whose tumours were disclosed on CT scans. In addition, 18 previously unidentified lesions were detected with this scintigraphy. Two of these lesions represented previously undetectable primary tumours. It is concluded that somatostatin receptor scintigraphy using [111In-DTPA-D-Phe1]-octreotide has a future role in the staging of patients with carcinoid disease.     

Double-contrast barium enema versus colonoscopy in the diagnosis of neoplastic disorders: aspects of decision-making in general practice

A total of 190 patients, referred by general practitioners for a double-contrast barium enema, were subsequently examined with colonoscopy. With colonoscopy and histology as the reference standard, sensitivity, specificity, positive and negative predictive values, and accuracy for the radiological detection of cancer and polyps were calculated. No cancer was overlooked by the radiological examination, but there were four false positives. The overall sensitivity for polyps was 70%, increasing to 81% for polyps > or = 10 mm. The predictive value was 93-97% for the exclusion of polyps. The caecum was reached in 187 patients by double-contrast barium enema (98%) and in 164 patients (86%) by colonoscopy. Lesions in four of 12 patients who had radiological changes were undetected at the first colonoscopy, but a repeat examination showed polyps > or = 10 mm in size. Although colonoscopy is a more sensitive technique for the detection of small mucosal lesions, the general practitioners may, in the vast majority of patients, rely on a negative result for polyps and cancer obtained by the double-contrast barium enema. The latter is linked with a number of false-positive cases, while colonoscopy is associated with technical difficulties; both techniques may lead to repeated examinations, regardless of which was the first choice.     

The vascular effects of topical and intravenous alpha2-adrenoceptor agonist clonidine on canine pial microcirculation

Helicobacter pylori infection of the gastric mucosal surface was investigated in patients with hamartomatous fundic polyps or hyperplastic polyps and in patients without endoscopic evidence of disease (healthy subjects). Presence of H. pylori infection was determined by culture, histologic examination, and the endoscopic phenol red test. Adherence of H. pylori was evaluated with scanning electron microscopic examination of antral biopsy specimens. Both prevalence of H. pylori infection (P < 0.001) and H. pylori adherence (P < 0.05) were less in patients with hamartomatous fundic polyps than in healthy subjects and patients with hyperplastic polyps. However, the percentages of plasma cells in gastric mucosa that contained IgA and of gastric epithelial cells that expressed Lewis b did not differ significantly among the three groups. These findings suggest that defense mechanisms against the attachment of H. pylori other than IgA or Lewis b antigen are present in patients with hamartomarous fundic polyps.     

Carcinoid tumors of the colon and rectum

The article gives a review of the literature concerning carcinoid tumours of the colon and rectum. Carcinoid tumours of the rectum are more common than of the colon, although these tumours are rarities. The carcinoid tumour grows slowly and the patients can survive for years with the disease. Treatment of the rectal carcinoid depends on the size and invasiveness of the primary tumour, as a non-invasive tumour less than 2 cm in diameter can be locally excised. For carcinoids of the colon, the treatment of tumours of less than 2 cm and without invasion will be local excision. For larger and/or invasive tumours in both colon and rectum the treatment is resection. The prognosis for colonic carcinoids is worse than for rectal carcinoids. Adjuvant treatment forms are briefly described. Follow-up programmes for these patients are generally long (over five years) and include recto-/colonoscopy, and search for metastatic spread.     

Carcinoid tumor associated with vascular malformation as a cause of massive gastric bleeding

Massive gastric bleeding in a 28-yr-old woman requiring emergency surgical treatment was found to originate from a polypoid carcinoid tumor 1.3 cm in diameter. Histologically, the tumor was found to be associated with a complex vascular malformation apparently originating from the underlying submucosa, crossing the tumor and ending in large mucosal sinusoids that opened on the mucosal surface. A similar clinical presentation was reported in three previous cases of small gastric carcinoids, one of which revealed an anomalous intratumoral bleeding artery. We recommend that in the absence of more common causative lesions of gastric bleeding, gastric carcinoid be considered in cases of focal massive hemorrhage requiring emergency treatment.     

Confirmation that Thiobacillus halophilus and Thiobacillus hydrothermalis are distinct species within the gamma-subclass of the Proteobacteria

BACKGROUND: There is controversy regarding the most appropriate investigation for suspected colorectal carcinoma. We offered these patients same-day flexible sigmoidoscopy (FS) and double-contrast barium enema (DCBE). METHODS: We reviewed the results of 117 consecutive adult patients. All patients underwent FS followed by DCBE on the same day. The radiographs were reviewed by two of the authors who were blinded to the clinical information, flexible sigmoidoscopy reports, and the original DCBE report. RESULTS: One hundred seventeen patients made up the study population. Thirty-four of the 117 patients had polyps and/or carcinoma. Three malignant tumours were detected by DCBE; one of these was also seen on FS, and the other two cancers were out of FS range. Fifty-three polyps were found by FS; nine were removed by biopsy prior to the enema examination. Of the 44 remaining polyps, DCBE failed to detect 87% of the 0-9-mm group and 67% of the >9-mm group. Ten polyps were seen only on DCBE; seven of these 10 were beyond the range of the sigmoidoscope, and the three remaining polyps were less than 5 mm. CONCLUSION: DCBE is insensitive in the detection of rectosigmoid polyps. FS should continue to be used as a complementary examination to DCBE in the investigation of suspected colorectal carcinoma.     

Nature of juvenile polyps in the large intestine

Forty-five juvenile polyps were found among approximately 390 mucosal polyps of the large intestine. Various degrees of epithelial pseudostratification were found among juvenile polyps and the larger-sized polyps of both juvenile type and adult one tended to have a more advanced degree of epithelial pseudostratification. The glands of both juvenile polyps and adult ones revealed a closely resembled or almost identical pattern of size-distribution-pattern of the glandular size in both normal mucosae and hyperplastic mucosae of long-standing ulcerative colitis. Transition of juvenile polyps into adult ones was suggested by the cases with increase in numbers of glands and their orifices to the surface of the polyp and there were some polyps showing an intermediate form between juvenile polyps and adult ones. On the basis of histologic characteristics and age incidence of the patients, it may be said that a juvenile polyp is an immature variant of an adult polyp.     

Carcinoid tumours. Frequency in a defined population during a 12-year period

The frequency of carcinoids was studied in a 12-year-period (1958-1969) in Malm?, a town with a population of 220,000 at the beginning of the period and 250,000 at the end of it. Of all persons who had died in Malm?, 46 per cent were necropsied in the first year of the study and 70 per cent in the last (altogether 62.6 per cent). The series was examined in a uniform way at one department of pathology. Carcinoid tumours were found in 1.22 per cent (199 patients) of patients comprised in the entire necropsy series (16,294 autopsies). Bronchial carcinoid accounted for 0.1 percent, the remaining lesions were found in the digestive tract. About 90 per cent of the carcinoids were found incidentally at necropsy. During the same period, 44 carcinoids were diagnosed in surgical specimens examined in Malm?. The average annual frequency of carcinoid in the entire series was about 8.4 per 100,000 inhabitants, which is about 7 times as high as that recorded in the National Cancer Register applying to the whole of Sweden. The value of such country-wide reports is discussed. The carcinoid syndrome is extremely rare and was observed only once during the entire 12-year-period. The localization, frequency of metastases and sex-distribution of carcinoids are described and discussed in detail.     

Bronchopulmonary atypical carcinoid tumour metastatic to the orbit

BACKGROUND: Bronchopulmonary carcinoid tumours metastatic to the orbit are rare. A case is presented demonstrating presentation, histopathology and treatment. METHODS: A 64-year-old man with a history of bronchopulmonary atypical carcinoid presented with an orbital mass. The nature of this metastasis was confirmed with histology and it was surgically excised and, in addition, local radiotherapy and chemotherapy were administered. RESULTS: There was a good response to the treatment for the orbital mass but other metastases developed, causing death. CONCLUSIONS: In this case of atypical carcinoid, orbital metastasis was the first sign of disease progression. Histological confirmation of the diagnosis was important in this case to allow planning of oncological therapy and for appropriate patient counselling. A good local response was obtained with a combination of surgical excision, local radiotherapy and chemotherapy. The systemic prognosis remains poor in cases of metastatic bronchopulmonary atypical carcinoid tumour, in contrast to typical carcinoid.     

Carcinoid tumour complicating inflammatory bowel disease. A study of two cases with review of the literature

Two cases of carcinoid tumour complicating inflammatory bowel disease (IBD) are presented. Both tumours were located in the appendiceal tip. The first case occurred in a man with Crohn's disease (CD), and the second one in a woman suffering from ulcerative colitis (UC). Histochemical and immunohistochemical studies were not allowed on case 1 because the tumour was not still present on serial sections of the appendix. On case 2, tumour cells were not reactive with Grimelius and Masson-Fontana stainings, but were strongly stained with anti-keratin and anti-chromogranin monoclonal antibodies (MAb), and faintly expressed neuron specific enolase (NSE), and Leu-7. Both cases occurred in inflammatory or damaged mucosa which exhibited Paneth cell metaplasia and hyperplasia and areas indefinite for dysplasia. Along with these lesions, hyperplasia of enteroendocrine cells was pointed out in the neighbouring appendiceal and colonic mucosa by means of anti-chromogranin MAb. These data suggest that the association of carcinoid tumour with IBD, albeit rare, is not coincidental and is the result of hyperplastic and dysplastic troubles that may involve enteroendocrine cells as well as such other derivatives of digestive stem cells as columnar cells, goblet cells and Paneth cells.     

Joint manifestations of carcinoid tumors of the digestive system

The joint manifestations of carcinoid tumours become integrated into the carcinoid syndrome, the totality of the symptoms occurring at a distance from the tumour and linked to the metabolites secreted by the tumour. These arthropathies have been the subject of only a very limited number of publications. They consist of arthralgia, sometimes with swelling, predominantly in the extremities of the upper limbs, and linked to activity. The radiological picture includes a "band" of demineralization, and less frequently destructive joint lesions. The sedimentation rate is always increased. These arthropathies can be related to their origin only by knowledge of, and demonstration of, the other elements of the carcinoid syndrome and of the tumoro-metastic syndrome. Laboratory diagnosis rests on determination of the 5-hydroxyl derivatives of tryptophane.