A total of 80 individuals in 4 kindreds with multiple endocrine neoplasia type 1 (MEN 1) have been subjected to repeated biochemical screening during a 10-yr period with the principal aim being to analyze characteristics of the developing pancreatic lesion. Age at presentation of the MEN 1 trait averaged 18 yr in 7 previously unaffected individuals, and this effect of the screening procedure represented a lowering by almost 2 decades. Pancreatic endocrine involvement was recognized at a mean age of 25 yr and constituted the presenting lesion in a majority of the patients. A standardized meal test and basal values of serum pancreatic polypeptide, insulin, proinsulin, and gastrin were the most efficient markers for the pancreatic lesion and preceded signs of pancreatic tumors upon radiological examinations by a mean of 3.5 yr. A 75% penetrance of the islet cell disease and 90% for primary hyperparathyroidism within the affected individuals equalled the prevalences reported in autopsy studies. Two of the kindreds showed signs of intrafamilial homogeneity with respect to the profile of peptide excess (P less than 0.05) and considerable discrepancy in the malignant potential of the pancreatic lesions. The results of early detection and surgical intervention of the pancreatic tumors in MEN 1 suggested an impact on morbidity, while any effect on the mortality of these individuals remains to be clarified.

Coincident with medical antitumor treatment of 138 patients suffering from mid-gut carcinoid tumors, 51 patients were subjected to surgery with the principal aims of removing primary tumors and debulking mesenteric or liver metastases. Sixteen patients had previously been operated with intestinal resection or, when the tumors had been considered inexcisable, with intestinal bypass or laparotomy alone. Apart from exhibiting symptoms related to the carcinoid syndrome, the majority (approximately 60%) of the 51 patients had generally intermittent, subileus-like abdominal pain and weight loss. In 18 patients, these symptoms were pronounced and associated with intestinal obstruction or severe malnutrition. Computed tomography and arteriography efficiently demonstrated mesenteric and liver metastases. At laparotomy, the primary intestinal tumors were small, mainly less than 1 cm in diameter, and they were multiple in 39% of the patients. Mesenteric metastases measuring up to 12 cm in diameter were present in 86% of the patients. These metastases were frequently associated with a pronounced mesenteric and retroperitoneal fibrosis causing fixation, angulation, and obstruction of the bowel as well as incipient intestinal gangrene in 8 patients. In all but 6 patients, the primary tumors could be removed by comparatively limited intestinal resections although bulky mesenteric metastases were often dissected from the mesenteric vessels. Liver metastases, found in 49% of the patients, were generally bilateral and multiple, and major hepatic metastases were resected in 6 patients. The results support a role for surgery also in the more compromised patients with mid-gut carcinoid tumors and that such intervention may be associated with considerable symptomatic relief and substantial periods of survival.

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A prospective study has been performed on 84 patients with endocrine pancreatic tumours evaluated at the Medical Department in Uppsala. Available information concerning the patients' presenting symptoms, age at diagnosis, clinical syndrome, tumour location, location of metastases, diagnostic radiology, biochemical and histopathological findings has been analysed. Our results indicate that most patients initially show rather vague and non-specific symptoms, with dyspepsia and pain being the most frequent presenting features. The median delay between appearance of the first symptom and diagnosis was 2 years; the delay was 35 months in sporadic cases and 14.5 months in familial cases. In spite of improvements in diagnostic methods, the median age at diagnosis (53 years) has not been reduced, and most patients are encountered when the tumour has reached an advanced stage. There is a need for a method of screening patients with still uncharacteristic abdominal symptoms for a neuroendocrine tumour. The presence of elevated levels of plasma chromogranin in all patients with a proven tumour suggests that such possibilities exist, and the use of this biochemical marker in the future might reduce the age at diagnosis and thus improve the likelihood of cure and survival of patients with endocrine pancreatic tumours.