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...

This study suggests that surgery may not be beneficial for MEN1 patients with NFPET < or = 2 cm.

Systematic subtotal parathyroidectomy associated with thymectomy is effective in treating most renal transplant recipients with tertiary HPT and also minimizes the recurrence of HPT in patients with declining renal function.

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Background and aims Intra-operative parathyroid hormone (IOPTH) results are not known in the setting of tertiary hyperparathyroidism (HPT) after renal transplantation. Materials and methods A retrospective analysis of 35 tertiary HPT patients who all underwent subtotal parathyroidectomy and IOPTH monitoring was conducted.Results The mean follow-up time was 2.2±1.4 years. Thirty-four patients were cured; one patient (2.8%) had a persistent disease and was cured after reoperation. Median parathyroid hormone (PTH) (median percent decrease from highest) at baseline and at 5, 10, 20, and 30 min were 244, 78 (69%), 63 (75%), 53 (79%), and 49 pg/ml (83%), respectively. Four patients who were cured had a decrease of <50% at 5 min and two of them had a decrease of <50% at 10 min. The patient with persistent disease had a decrease of >50% at 10 min. The sensitivity of the test was 94% at 10 min using the Miami criteria. Conclusion This study shows that IOPTH in tertiary hyperparathyroidism has a high sensitivity. However, because of the low risk of persistent hyperparathyroidism when a subtotal parathyroidectomy is performed, its potential impact on the overall success rate is very small. We therefore do not recommend the routine use of IOPTH in tertiary hyperparathyroidism.

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