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Background Appendiceal carcinoid tumours are found in 0·3–0·9 per cent of patients undergoing appendicectomy. Controversy exists over the management following appendicectomy, especially with regard to the role of right hemicolectomy in patients with tumours smaller than 2 cm in diameter. Methods and results The literature pertaining to the behaviour of appendiceal carcinoids was reviewed in order to formulate indications for right hemicolectomy. Metastatic disease from appendiceal carcinoids is a rare occurrence, but is more common when lesions are larger than 2 cm in diameter. The risk–benefit balance of right hemicolectomy needs to be better defined, and an improved understanding of tumour cell biology may aid prognostic accuracy and decision-making. Conclusion There is limited evidence on which to base clear indications for right hemicolectomy in patients with a diagnosis of appendiceal carcinoid. Acceptable indications are carcinoids larger than 2 cm in size, any high-grade malignant carcinoid (including those with a high mitotic index), mesoappendiceal invasion, lesions at the base of the appendix with tumour-positive margins, and goblet cell adenocarcinoid tumours.

PURPOSE: To describe the clinical and histopathologic findings in a patient with corticosteroid-induced open-angle glaucoma attributable to an adrenocorticotropin-secreting malignant carcinoid of the thymus. METHODS: Case report. In a 33-year-old man, the clinical course, laboratory findings, and imaging results as well as the histopathologic findings are described. RESULTS: Increased intraocular pressure in this patient represented a manifestation of severe hypercortisolism attributable to a malignant adrenocorticotropin-secreting carcinoid tumor. Surgical removal resulted in return of the intraocular pressure values to normal levels. CONCLUSION: Thymic carcinoid is a rare cause of Cushing syndrome, which can lead to increased intraocular pressure.

An undifferentiated small cell tumor of the colon that was found by light- and electron-microscopic study to possess features of a malignant carcinoid merged into keratinizing squamous cell carcinoma. Single cells contained both dense-core secretory granules and tonofilaments. The implications of this combination in relation to theories of tumor origin and differentiation are briefly discussed.

Gastrointestinal carcinoid tumors arising from the foregut and hindgut may metastasize within the abdomen. Bone metastases are rare, but their occurrence is well established. Both osteoblastic and osteolytic metastases can occur in the same patient without any bone symptoms. Three patients with malignant carcinoid tumors had no bone symptoms but demonstrated extensive bone metastases on radionuclide bone scans. Some of these lesions were abnormal on corresponding roentgenograms and some areas were negative on roentgenograms. The use of radionuclide bone scanning in the evaluation of patients with malignant carcinoid tumor is emphasized.

In a retrospective review, we were able to identify records for 18 patients who were treated for malignant carcinoid in our Departments of Radiation Oncology from 1974-1985. At postmortem examination, one was found not to have malignant carcinoid. In 4 cases irradiation was administered postoperatively to patients whose subsequent courses did not permit assessment of objective response. Using standard criteria for objective response, an objective response rate of 54% was observed in the 13 cases whose records were adequate to make this assessment. Of the 7 responses, 3 were complete within the treated field as judged by clinical findings (2 patients) or postmortem examination (1 patient). Of the 6 nonresponders, 2 interrupted treatment prematurely, 2 were treated to a planned dose of only 2000 and 2500 cGy, and 2 were treated palliatively to limited ports which included only partial volumes of tumor. We conclude that radiation therapy is an effective treatment modality with a high rate of objective response in the management of those patients with malignant carcinoid tumors who require non-surgical, anti-neoplastic therapy.

We report a case of a presacral teratoma containing a malignant carcinoid component associated with an anterior sacral meningocele that presented in a 35-year-old woman. The clinical, radiographic, and pathological features of these rare tumors, of presacral meningoceles, and of the hereditary presacral teratoma syndrome are discussed. (Neurosurgery 22:581-588, 1988)

This paper presents a unique apudoma variant--a malignant carcinoid of mid and lower oesophagus in an elderly man. It was remarkable in the short duration of the clinical symptoms, its rather easy resectability despite its large size and the local perigastric lymph node metastases. On light microscopy, the neoplasm was composed entirely of atypical but uniform round cells lacking argentaffin and argyrophilic staining, arranged in a carcinoid pattern. On electron microscopy, they exhibited dense-core neurosecretory granules.

A case of primary malignant laryngeal carcinoid with dual endocrine and mucous differentiation i s reported. Histologically the tumor showed a characteristic organoid pattern and exhibited Alcian-blue, periodic acidschiff, and Grimelius silver positivity. By the immunoperoxidase technique cal citoni n, ACTH, and or-hCG subunit were demonstrated in the tumor cells. ELectron microscopy revealed two different types of endocrinelike cells: mucous cells and occasional cells containing both endocrinelike granules and mucin droplets. Diagnostic morphologic criteria of this rare tumor entity are discussed and reference t o biologic behavior and possible h istogenesis is made.

The malignant carcinoid syndrome (MCS) is a rare complication of malignant carcinoid neoplasms, fewer than 200 cases having been reported since its description by Thorson in 1954. Its signs and symptoms vary with the site of the primary neoplasm, and MCS is now recognized as one member of the large family of endocrine tumors - APUDOMAS). Although MCS remains uniformly fatal, significant palliation can be provided some patients with current surgical and chemotherapeutic management. All signs and symptoms in MCS, except evidence of the destruction of the tricuspid and pulmonic valves, can be reproduced experimentally. The factor causing this lethal complication has not been identified. Two previously unreported cases of MCS, each from the gastrointestinal (GI) tract, are presented. This brings the world's total reported cases to approximately 210.

A case of congenital pulmonary stenosis with tricuspid insufficiency and a peculiar type of cyanosis in an 19-year-old boy is described. The patient died in connection with angiocardiography. At autopsy, the clinical diagnosis was verified; in addition to the cardiac lesion there was a malignant carcinoid of the jejunum with metastases to the liver. The findings are briefly discussed and an explanation of the type of cyanosis is suggested.

During the course of anaesthesia, in patients with malignant carcinoid disease, sudden episodes of hypotension may occur. These are apparently due to the failure of compensatory mechanisms in the cardiovascular system to adjust rapidly to the effects of sudden serotonin release.

WE recently recognized distinctive ophthalmic consequences of the carcinoid syndrome that have not been reported. Patients with malignant carcinoid were studied during their hospital courses at the National Institutes of Health. Their ocular changes form the basis of this report. Material and Methods The subjects were 16 patients from thirty-three to sixty-nine years old. The diagnosis of carcinoid tumor was established histologically by biopsy, pharmacologically by flushes induced with epinephrine1 2 3 and chemically by the demonstration of abnormally large concentrations of serotonin (5-hydroxytryptamine) in platelets and of 5-hydroxyindoleacetic acid (5-HIAA) in the urine. Epinephrine hydrochloride (2 to 10 μg.) was diluted . . .

Of a group of 13 patients with carcinoid tumours, 10 were investigated biochemically during life, and 3 tumours were discovered post mortem. Nine of the 10 patients had significantly elevated levels of 5-hydroxytryptamine in blood platelets, but only 7 showed markedly increased levels of 5-hydroxyindoleacetic acid in urine. One of these patients excreted the precursor 5-hydroxytryptophpn, as well as 5-hydroxytryptamine and 5-hydroxyindoleacetic acid. Analysis of tumour tissue from this patient showed only low levels of 5-hydroxytryptamine (16 μg/g) when compared with the levels from other carcinoid tumours (50-500 μg/g). A primary tumour in this patient could not be located at autopsy. In another patient with widely disseminated metastases, a small primary tumour was found in a Meckel's diverticulum, a rare site of development. Of the 3 carcinoid tumours discovered post mortem, the first involved the appendix and right ovary; in the second case, multiple primary tumours occurred in the jejunum, while in t...

The first case of a gastric carcinoid involving the distal esophagus is reported, with a brief summary of reviewed literature of gastric carcinoids. Emphasis should be made of the relatively benign nature of this malignant condition and, therefore, an aggressive surgical approach should be carried out.

The case of a 26-year-old male with malignant carcinoid of the gallbladder is presented. Extensive review of the literature reveals this patient to be the third case of a metastasizing gall bladder carcinoid and the twelfth reported case in the literature.

Carcinoid tumor of the pancreas is extremely rare. This article describes the case of a 22-year-old woman who manifested a typical carcinoid syndrome; a definitive diagnosis of a metastatic carcinoid tumor was made from needle aspiration of a supraclavicular lymph node using morphologic, immunocytochemical, and ultrastructural criteria. The carcinoid tumor was subsequently shown to be of pancreatic origin.

A case of malignant carcinoid tumor (neuroendocrine carcinoma) of the cecum is described. The neoplasm arose in a patient who had chronic ulcerative colitis for 16 years. There is no previous documentation of this combination, which is surprising, in view of the known association of colonic adenocarcinomas and lymphomas with ulcerative colitis. The reasons for this discrepancy are discussed and it is postulated that ultrastructural examination of poorly differentiated colonic neoplasms may result in a more frequent identification of this association.

This report describes the radiologic findings in the recently described syndrome of multiple colonic polyps and malignant tumors of neurocrest origin. One patient had multiple endocrine neoplasia syndrome type 11B with malignant medullary thyroid carcinoma, another had malignant pheochromocytoma, and the third had malignant carcinoid tumor. All patients had multiple colonic tubular or tubulovillous adenomas. In 2 patients, there were 8 sessile or pedunculated polyps distributed throughout the colon. In the patient with multiple endocrine neoplasia syndrome type 11B, the colon was carpeted with small polyps, 2–6 mm in size.

Eight cases of the rare undifferentiated carcinoma of the large intestine are described. The histological distinction between undifferentiated and poorly differentiated colonic adenocarcinoma and malignant carcinoid is discussed. It is concluded that undifferentiated carcinoma is a variant of adenocarcinoma which tends to grow to a large size before symptoms are produced but which nevertheless has a good prognosis when locally resectable. Five patients survived between 6 and 28 years, one is well 6 months after operation and two cases where local removal could not be achieved, died within a year.

Hepatic arterial embolisation was performed in six patients with malignant carcinoid tumours; five with the carcinoid syndrome and the other with intractable pain due to pleural and hepatic metastases. A total of 11 embolisations was performed, each time producing noticeable symptomatic relief, especially of facial flushing and diarrhoea. Apart from occasional episodes of the post-embolisation syndrome, no significant complications were experienced. Relief of symptoms lasted 2 months to 18 months in all patients with the carcinoid syndrome. Repeat embolisation was performed in three of the six patients. Three of the six patients have died at intervals ranging from 3 weeks to 20 months after the last embolisation. The cause of death was not related to embolisation. Hepatic embolisation is an effective, safe and repeatable method of palliating the symptoms of the carcinoid syndrome.

A clinically and histopathologically well-documented case of malignant carcinoid tumor (with carcinoid syndrome) is described. The presence of numerous psammoma bodies in metastatic carcinoid tumor deposits, confirmed by histochemical and ultrastructural analyses, represents a heretofore unreported finding.

A case is presented in which an adenocarcinoma and a malignant carcinoid tumor occurred in the same area of the transverse colon. Only three cases of carcinoid tumor of the transverse colon have been reported in the literature and in two of these cases the tumor was malignant. The case reported herein is added to the literature.

A twenty-two year old woman with a malignant carcinoid of the stomach, pubertal adrenal hyperplasia and polycystic ovaries is presented. The relationship of carcinoid tumors to other endocrinopathies is reviewed. Suggestions are made as to the operative treatment of this unusual lesion.

A review of the literature on carcinoid with a case of malignant carcinoid of the appendix and of the cecum have been presented. Metastases to the lymph nodes and the liver were present. Although few cases of malignant carcinoids are reported in the literature, the tumor can no longer be regarded as invariably benign. The neoplasm is slow-growing and the presence of metastases should not prevent the surgeon from removing the primary site.

THE relation between malignant carcinoid and various systemic changes has been extensively reviewed1 , 2 since 1953, when Isler and Hedinger3 reported 3 cases and suggested that this constituted a ...

SUMMARY The indications for cutaneous ureterostomy are discussed and a further indication is considered. A brief review of the different methods of approaching the problem is described. A method of collecting the urine from a ureterostomy is reported using a finger cot and polythene tube to a portable thigh urinal. Our thanks are due to Mr G. Y. Feggetter and Mr Fenton Braithwaite for their helpful advice and criticism.

The extremely rare association of a carcinoid tumour with an adenocarcinoma of the stomach is reported. A review of the literature revealed only 1 previous reported instance in which a less intimate relationship of the two lesions was found in a resected stomach.

MOST carcinoid tumors of the gastrointestinal tract are situated in the appendix. The relatively benign nature of appendiceal carcinoids and their frequency contrast with the extra-appendicular variety of carcinoids. In fact, the extra-appendicular carcinoids, in all statistical studies, appear in the ill-defined area between benign and malignant lesions. Some present polyps or well circumscribed lesions; others present patterns of spread similar to adenocarcinomas of the gastrointestinal tract yet differ from them by their remarkably slow rate of growth. The rarity of these lesions, their ill-defined nature, and peculiar biological characteristics create difficult problems in diagnosis and management. The presentation and elucidation of such problems is the purpose of this study, which is based on the review of ten extra-appendicular, gastrointestinal carcinoids removed at the surgical departments of Beilinson Hospital and Meir Hospital during a period of ten years (1955 to 1965). A case in which a malignant carcinoid tumor was

It is only a few years since Thorson 1 and others recognized the association of several unusual signs and symptoms with carcinoid tumors of the small bowel with hepatic metastases. It is apparent now that (1) carcinoid (argentaffinoma) is an endocrine tumor which produces the pharmacologically active agent, serotonin (5-hydroxytryptamine), and (2) the various manifestations of the "carcinoid syndrome" are attributable to secretion of serotonin by the tumors and a disturbance in the metabolism of tryptophan. 2 Since many cases of the disorder have now been recognized, 3,4 both the general practitioner and the specialist should be aware of this condition and become acquainted with the simple chemical test 5 on urine by which it may be diagnosed. Clinical Features A series of baffling illnesses extending over a period of years is typical, coinciding with slow growth of the tumor. Current clinical concepts are derived chiefly from study of advanced

Introduction The association of neuromuscular disorders with malignant tumors has been the subject of increasing comment. 1 Because many of these neuromuscular disorders do not manifest themselves clinically or pathologically as an uncomplicated neuritis or a myositis but as a mixture of the two, the term, carcinomatous neuromyopathy, appears more appropriate. 2 While this association has been noted most frequently with carcinoma of the lung and breast, it has been recognized to occur in many other types of malignancy as well. However, the coexistence of a neuromyopathy and a carcinoid tumor has not to our knowledge been previously noted. This report describes a patient with a confirmed intestinal carcinoid tumor and a severe neuromyopathy. Report of Case The patient, a 60-year-old white woman, was seen in the University Hospital for the first time in May, 1961. She complained of aching pain in the back, spine, knees, and wrists. She had