Objective: To analyze the penetrance and clinical course of isolated nonfunctioning tumors of the pancreas (NFTP) in MEN 1 patients, and to propose a strategy for managing them. Summary Background Data: Pancreaticoduodenal tumors develop in a majority of MEN 1 patients and are a major cause of death. The natural history of NFTP is poorly defined, and no clear-cut guidelines have been widely accepted regarding treatment. Methods: Data on 108 patients with isolated NFTP among 579 MEN 1 patients from the French Endocrine Tumor Study Group (GTE) were analyzed. Survival rates were calculated using the Kaplan-Meier method. Results: The penetrance of NFTP was 34% at age 50, making it the most frequent pancreaticoduodenal tumor in MEN 1 patients. Fortythree patients (40%) underwent surgery, 32 of them curatively. No patient died because of surgery. Average life expectancy for patients with NFTP was shorter than that for MEN 1 patients who did not have pancreaticoduodenal tumors. Thirteen patients died during follow-up, 10 due to NFTP. Tumor size was correlated with the risks of metastasis and death. These risks were low for patients with tumors Յ20 mm. Conclusions: NFTP are currently the most common tumors of the pancreaticoduodenal region in patients with MEN 1. Prevention of tumor spread by surgery should be balanced with potential operative mortality and morbidity. We do not recommend routine surgery for NFTP Յ20 mm. (Ann Surg 2006;243: 265-272) M ultiple endocrine neoplasia type 1 (MEN 1) is a rare autosomal dominant condition characterized by the development of endocrine parathyroid, pancreaticoduodenal, and pituitary tumors. In addition, MEN 1 patients are also prone to developing adrenal tumors, neuroendocrine tumors (in particular of the thymus or bronchus), dermal lesions, thyroid disease, and meningeal tumors. 1-7Pancreaticoduodenal tumors are often multiple, have been shown at autopsy to have developed in up to 80% of patients with MEN 1, and are a major cause of premature death in these patients. 8 -12 Most pancreaticoduodenal tumors are functioning tumors, the most frequent of which are gastrinomas and insulinomas, followed by the rare glucagonomas, VIPomas, GRFomas, and somatostatinomas. Surgical resection is the treatment of choice for functioning tumors of the pancreas, although controversy exists regarding the timing of surgery for gastrinomas. [13][14][15][16][17][18] In the absence of hormonal symptoms, nonfunctioning tumors of the pancreas (NFTP) have been recognized as a separate entity whose penetrance in the MEN 1 population is not well known. 19,20 In addition, because large surgical series of patients presenting with isolated NFTP are lacking and few clinical series have followed patients with NFTP, no clear-cut treatment guidelines have been widely accepted. Some authors have recommended a conservative approach for asymptomatic NFTP less than 1, 2, or 3 cm. [21][22][23][24] Others have recommended early surgical excision of all tumors as soon as they are found on imaging studies, or even ea...

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This prospective series shows a higher duodenal polyposis progression rate and cumulative risk of late-stage (stage IV) duodenal polyposis in FAP patients compared with previous series. These results suggest that current modalities for surveillance and management of these patients need revision.

As duodenal neuroendocrine tumors (NETs) are rare, their optimal management has not been clearly established. The aim of this study was to evaluate the feasibility and outcome of endoscopic treatment of duodenal NETs. We reviewed the files of all patients who underwent endoscopic resection of a sporadic duodenal or ampullary NET between 1996 and 2014 at two centers. A total of 29 patients with 32 uT1N0M0 NETs < 20 mm were included. Treatment consisted of endoscopic mucosal resection in 19 cases, and cap aspiration in 13 cases. Prior submucosal saline injection was used in 15 cases. Mortality was 3 % (one severe bleeding). Morbidity was 38 % (11/29). At post-resection analysis, mean tumor size was 8.9 mm (range 3 - 17 mm), 29 lesions were stage pT1, one was pT2, and 2 were pTx because of piecemeal resection. All NETs were well differentiated. A total of 27 lesions were classified as grade 1 and 5 were grade 2. The resection was R0, R1, and Rx for 16, 14, and 2 lesions, respectively. Three R1 patients underwent additional surgical treatment, with no residual tumor on the surgical specimen but with positive metastatic lymph nodes in two cases. One patient was lost to follow-up. Finally, 24 patients were included in the follow-up analysis. The median follow-up period was 56 months (range 6 - 175 months). Two patients presented a tumor recurrence during the follow-up period. Endoscopic treatment of small duodenal NETs was associated with significant morbidity, a difficulty in obtaining an R0 specimen, and the risk of lymph node metastasis. Nevertheless, it represents an interesting alternative in small grade 1 duodenal lesions and in patients at high surgical risk.

Lanreotide is safe and well tolerated in patients with carcinoid tumors. It seems to have both symptomatic and antitumoral effects in this setting.

Contrast- and water-enhanced multidetector CT enteroclysis had an overall accuracy of 84.7% for depiction of small-bowel neoplasms.

The effect of potential mediators of mucus secretion was investigated in the isolated vascularly perfused rat colon by using a sandwich enzyme-linked immunosorbent assay for rat colonic mucin and by histochemical analysis. Bethanechol (100–200 μM), bombesin (100 nM), and vasoactive intestinal peptide (VIP, 100 nM) provoked a dramatic mucin discharge (maximal response at 900, 900, and 600% of control loops, respectively). VIP-stimulated mucin secretion was abolished by tetrodotoxin, whereas atropine was without effect. In contrast, both tetrodotoxin and atropine significantly decreased mucin release induced by bombesin. Isoproterenol or calcitonin gene-related peptide was without effect. Serotonin (1–5 μM) and peptide YY (10 nM) evoked mucin discharge, whereas glucagon-like peptide-1 did not release mucin. Finally, bromolasalocid (20 μM), interleukin-1β (0.25 nM), sodium nitroprusside (1 mM), and dimethyl-PGE2 (2.5 μM) induced mucus discharge. The results demonstrated a good correlation between the immunological method and histological analysis. In conclusion, these findings suggest a role for the enteric nervous system, the enteroendocrine cells, and resident immune cells in mediation of colonic mucus release.