On July 2000, 127 gastrinomas (31.1%) were studied by the Endocrine Tumour Group (GTE) using a 408-patient cohort of Multiple Endocrine Neoplasia Type I patients. The aim of this study was to assess clinical, biological, surgical data as well as their trends over three periods (<1980-1980/1989->1990). A Zollinger-Ellison syndrome (SZE) was present in 96% of the cases. Mean age at the onset of the disease was 39.4 years. There were 55.9% of men. Synchronous liver metastasis was present in 7.1%. Taken independently, the positivity of the four main diagnosis tests decreased over the time. The diagnosis of oesophagitis increased (4.5-29.7%), as well as the size of the resected tumours (9.9-16.8 mm). There was an increase in the familial background diagnosis (73.1-80%), an increasing use of Octreoscan scintigraphy and transduodenal ultrasound with positive detection of metastasis and tumours in 81.3% and 92.3%, respectively after 1991. Patients were operated on less frequently (96-52.5%), less frequently from the pancreas (87.5-37.5%), and from the gastro-intestinal tract (70.8-30%). The relative percentage of major pancreatic resections increased (with at least removal of the duodenum and the pancreatic head) (10-26.7%). The operative mortality disappeared. Six out of the seven patients (85.7%) who benefited from major pancreatic resections normalized their gastrine level postoperatively versus 15% in less radical techniques. Overall 5 years survival was 90 +/- 4.4%. Survival increased after 1985 (85 +/- 4.8% versus 95 +/- 3.6, P = 0.1).Conclusion. - SZE in NEM1 were diagnosed at an earlier stage and were less frequently operated on. Nevertheless, the incidence of synchronous metastasis did not change significantly. Patients were mainly operated on for gastric emergencies and pancreatic tumours in order to prevent metastasis without mortality after 1991. (C) 2004 Elsevier SAS. Tous droits reserves.

Multiple endocrine neoplasia type 1 (MEN 1) is a rare but misleading disease. The diagnosis is evocated when two main lesions are present (parathyroid, endocrine,pancreas, pitituary gland) but also when a family-tree shows recurrent lesions. Other lesions must be taken into account (adrenal glands, neuroendocrine thymic or bronchic lesions, cutaneous lesions, lipomas, nervous central system tumors). Any surgical cure without knowing the MEN1 background leads to failure. Specific treatment of each lesion is reviewed. Genetic diagnosis is possible but the mutation is not found in all cases. Nevertheless, when the mutation is known in a family, a negative genetic test allows to exclude the disease. Prognosis is related to hepatic metastases and to thymic neuroendocrine tumors which are rare (2.1%) but aggressive. As a general,rule, any apparently isolated endocrine lesion such hyperparathyroidism must prompt the surgeon to look for another endocrine lesion and to look for an abnormal family tree with reccurent monoglandular or pluriglandular lesions. (C) 2002 Editions scientifiques et medicales Elsevier SAS. All rights reserved.

Multiple endocrine neoplasia type 1 (MEN1) is a rare but misleading disease. The diagnosis is evocated when two main lesions are present (parathyroid, endocrine pancreas, pituary gland) but also when a family tree shows recurrent lesions. Other lesions must be taken into account (adrenal glands, neuroendocrine thymic or bronchic lesions, cutaneous lesions, lipomas, nervous central system tumors). Any surgical cure without knowing the MEN1 background leads to failure. Specific treatment of each lesion is reviewed. Genetic diagnosis is possible but the mutation is not found in all cases. Nevertheless, when the mutation is known in a family, a negative genetic test allows to exclude the disease. Prognosis is related to hepatic metastases and to thymic neuroendocrine tumors which are rare (2.1%) but aggressive. As a general rule, any apparently isolated endocrine lesion such hyperparathyroidism must prompt the surgeon to look for another endocrine lesion and to look for an abnormal family tree with recurent monoglandular or pluriglandular lesions.

Study aim: The aim of this retrospective study conducted by the much less than Groups d'etude des neoplasies endocriniennes multiples type 1 much greater than (GENEM) was to report a series of insulinomas associated with multiple endocrine neoplasias type 1 (NEM 1) and to determine the most appropriate strategy for the topographical exploration and surgical management.Patients and methods: From 1960 to 1996, 44 patients were included, 16 men and 28 women (mean age: 36,4 +/- 13,9 years). This study concerned morphological investigations, tumoral features, modalities and results of the treatment.Results: Insulinomas were associated with other functional islet tumors in 15 patients: ZE syndrome (n = 8), glucagonoma (n = 6), vipoma (n = 1). Malignant lesions were present in 6 patients: isolated insulinomas (n = 2), insulinomas associated with ZE syndrome (n = 2) and vipoma (n = 1). The sensivity of the preoperative imaging procedures was less than 70 %. Fourty-one patients were operated on: subtotal pancreatectomy (n = 26 including cephalic enucleations in 8), enucleations (n = 8), total pancreatectomy (n = 3), pancreaticoduodenectomy with caudal enucleations (n = 1), and for 3 patients, no details were available. There was no postoperative mortality. Hyperinsulinism disappeared in 27 patients (including 2 after reoperation) with a mean follow-up of 9 years. Among 6 patients with malignant lesions, 3 were alive with a 3, 4 and 10 year-followup.Conclusion: Subtotal pancreatectomy with splenic conservation and enucleation of cephalic lesions is suggested as the procedure of choice in this group of patients. This procedure allows simplification of preoperative imaging investigations because lesions of the pancreatic head have only to be detected. Preoperative endoscopic ultrasonography and intraoperative ultrasonography are the best investigations. (C) 2000 Editions scientifiques et medicates Elsevier SAS.

Medullary thyroid carcinoma (MTC) is a calcitonin (CT)-secreting endocrine tumor. Although plasma CT level is a specific and sensitive marker of MTC, its preoperative usefulness in predicting tumor size and postoperative CT normalization has not been documented. From a nationwide database set up by the French CT Tumor Study Group, 226 MTC patients were selected according to the following criteria: preoperative CT level determination by an immunoradiometric assay (normal value, < 10 pg/mL) within the 6 months prior to surgery, total thyroidectomy and diagnosis of MTC ascertained by histological report including tumor size. Patients were 129 females and 97 males (female/male ratio, 1.3). One hundred and twelve patients (49.6%) had the sporadic variety of the disease, 74 (32.7%) had multiple endocrine neoplasia 2A, three (1.3%) had multiple endocrine neoplasia 2B, and 37 (16.4%) had familial MTC. Median age at diagnosis was 44.8 yr (range, 4.9-80.1 yr). Complete neck dissection was performed in 159 patients (70.4%). Postoperative CT normalization was ascertained by negative response of CT to pentagastrin stimulation (< 10 pg/mL) in 94 patients. Seventy-one patients were considered as not cured because of residual tumor tissue and/or elevated CT levels. Median tumor size was 11.0 mm (range, 0.2-80.0 mm), significantly larger in females (15.0 vs. 8.0 mm, P < 0.05), and in sporadic forms (15.0 vs. 7.0 mm, P < 0.05). Tumor size was significantly correlated (r2 = 0.52, P < 0.01) with preoperative CT levels, the relationship being more straight in familial (r2 = 0.71) than in sporadic (r2 = 0.36) forms. Furthermore, preoperative CT levels under 50 pg/mL appeared to be predictive of postoperative CT normalization (44 of 45 patients). However, higher CT levels did not mean absence of postoperative CT normalization (50 of 120 patients). We conclude that low preoperative CT levels are predictive of tumor size and postoperative CT normalization.

STUDY AIM:The aim of this retrospective study conducted by the "Groupe d'étude des néoplasies endocriniennes multiples type 1" (GENEM) was to report a series of insulinomas associated with multiple endocrine neoplasias type 1 (NEM 1) and to determine the most appropriate strategy for the topographical exploration and surgical management.PATIENTS AND METHODS:From 1960 to 1996, 44 patients were included, 16 men and 28 women (mean age: 36.4 +/- 13.9 years). This study concerned morphological investigations, tumoral features, modalities and results of the treatment.RESULTS:Insulinomas were associated with other functional islet tumors in 15 patients: ZE syndrome (n = 8), glucagonoma (n = 6), vipoma (n = 1). Malignant lesions were present in 6 patients: isolated insulinomas (n = 2), insulinomas associated with ZE syndrome (n = 2) and vipoma (n = 1). The sensitivity of the preoperative imaging procedures was less than 70%. Fourty-one patients were operated on: subtotal pancreatectomy (n = 26 including cephalic enucleations in 8), enucleations (n = 8), total pancreatectomy (n = 3), pancreaticoduodenectomy with caudal enucleations (n = 1), and for 3 patients, no details were available. There was no postoperative mortality. Hyperinsulinism disappeared in 27 patients (including 2 after reoperation) with a mean follow-up of 9 years. Among 6 patients with malignant lesions, 3 were alive with a 3, 4 and 10 year-follow-up.CONCLUSION:Subtotal pancreatectomy with splenic conservation and enucleation of cephalic lesions is suggested as the procedure of choice in this group of patients. This procedure allows simplification of preoperative imaging investigations because lesions of the pancreatic head have only to be detected. Preoperative endoscopic ultrasonography and intraoperative ultrasonography are the best investigations.

Primary hyperparathyroidism (PHPT) in multiple endocrine neoplasia (MEN) type IIa is rare, occurring in 20% to 30% of the patients. The aim of this study was to evaluate clinical findings, surgical therapy, and outcome for 56 patients affected by PHPT among 249 MEN-IIa patients collected from 84 families assembled by the Groupe d'Etude des Tumeurs á Calcitonine (GETC, French Calcitonin Tumors Study Group). This retrospective study was based on cases registered by the GETC (20 participating centers) from 1969 to 1994. Characteristics of PHPT in 56 patients (31 women, 25 men) with MEN-IIa were reviewed. All but two underwent cervicotomy. The median age at diagnosis was 37.6 years. PHPT was found concomitantly with medullary thyroid carcinoma (MTC) or pheochromocytoma in 43 patients (77%). PHPT was asymptomatic in 68% of the patients. Serum calcium levels ranged from 2.20 to 3.70 mmol/L (median 2.82 mmol/L; normal 2. 10-2.60 mmol/L). The number of parathyroid glands removed at surgery was 0 (n = 2), 1 (n = 24), 2 (n = 5), > 2 (n = 12), 4 (n = 11). Pathology (initial surgery) consisted of 24 adenomas, 4 double adenomas, and 25 hyperplasia. Cure after initial surgery was obtained in 89%, including a 22% incidence of hypoparathyroidism. There were 6 cases (11%) with persistent PHPT. With a mean follow-up of 6.4 years, five patients (9%) had recurrent PHPT. The results indicate that MEN-IIa-related PHPT is generally associated with mild, often asymptomatic hypercalcemia. Despite recurrences encountered 5 to 15 years after the first cervicotomy, resection of only macroscopically enlarged glands generally appears sufficient. Subtotal or total parathyroidectomy with autotransplantation is associated with a high rate of hypoparathyroidism.

Le Registre National Francais des NEM 1 (en partie etendu a la Belgique) a collige entre 1989 et 1992, 103 patients atteints d'hyperparathyroidie (HPT). Il s'agissait de 71 femmes et 32 hommes d'âge moyen de 40,8 ans ± 13,5 ans (âges extremes de 7 et 77 ans). L'HPT etait associee a une autre atteinte glandulaire 91 fois (atteinte pancreatique 74 %, hypophysaire 56 %, surrenalienne 14 %, tumeur carcinoide 6,5 %) et elle etait isolee, fruit d'un depistage familial 12 fois. 26 fois l'HPT etait revelatrice de la polyendocrinopathie. Elle etait symptomatique dans 39,8 % des cas (lithiase urinaire 28 %, douleurs osteoarticulaires 21 %, asthenie et faiblesse musculaire 40 %, troubles psychiques 12,6 %, syndrome polyuropolydipsique 15 %, HTA 14 %, hypercalcemie aigue 5 %)