Population-based study of the treatment and prognosis of carcinoma of the rectum.

International audienceFew population-based studies address the issue of treatment of carcinoma of the rectum (15 cm or less from the anal verge) both from surgical and epidemiological aspects

Pituitary disease in MEN type 1 (MEN1): Data from the France-Belgium MEN1 multicenter study

To date, data on pituitary adenomas in MEN type 1 (MEN1) still have to be evaluated. We analyzed the data of a large series of 324 MEN1 patients from a French and Belgian multicenter study. Data on pituitary disease were compared with those from 110 non-MEN1 patients with pituitary adenomas, matched for age, year of diagnosis, and follow-up period. Genetic analysis of the MEN1 gene was performed in 197 of the MEN1 patients. In our MEN1 series, pituitary disease occurred in 136 of 324 (42%), less frequently than hyperparathyroidism (95%, P < 0.001) and endocrine enteropancreatic tumors (54%, P < 0.01). Mean age of onset of pituitary tumors was 38.0 +/- 15.3 yr (range, 12-83 yr). Pituitary disease was associated with hyperparathyroidism in 90%. of cases, with enteropancreatic tumors in 47%, with adrenal tumors in 16%, and with thoracic neuroendocrine tumors in 4%. Pituitary disease was the initial lesion of MEN1 in 17% of all MEN1 patients. MEN1 pituitary adenomas were significantly more frequent in women than in men (50% vs. 31%,P < 0.001). Among the 136 pituitary adenomas, there were 85 prolactinomas and 12 GH-secreting, 6 ACTH-secreting, 13 cosecreting, and 20 nonsecreting tumors. Eighty-five percent of MEN1-related pituitary lesions were macroadenomas (vs. 42% in non-MEN1 patients, P < 0.001), including 32% of invasive cases. Among secreting adenomas, hormonal hypersecretion was normalized, after treatment, in only 42% (vs. 90% in non-MEN1 patients, P < 0.001), with a median follow-up of 11.4 yr. No correlation was found between the type of MEN1 germ-line mutation and the presence or absence of pituitary adenoma. Our study, based on a large group of MEN1 patients, shows that pituitary adenomas occur in 42% of the cases and are characterized by a larger size and a more aggressive presentation than without MEN1

Screening of multiple endocrine neoplasias type 1. Reflexions of the Study Group on Multiple Endocrine Neoplasias type 1

The "Groupe d'Etude des Neoplasies Endocriniennes Multiples de type 1 (GENEM 1)" is a French group involved in a comprehensive multicentre study of Multiple Endocrine Neoplasia type 1 syndrome (NEM 1). The objectives of this group are to define diagnostic and therapeutic protocols and to carry out genetic research on NEM1. The first aim of physicians is to recognize the syndrome and to determine the appropriate screening especially into two circumstances: 1 degree In case of isolated and sporadic glandular disease -i-e-parathyroid glands, endocrine pancreas, antehypophysis, adrenal glands and neuroendocrine tumors? 2 degrees In case of very high probability of NEM 1 syndrome? This paper answers these two questions, based on the analysis of the first 150 cases collected by the GENEM 1.Les néoplasies endocriniennes multiples de type 1 (NEM1) font l'objet d'une étude française multidisciplinaire et multicentrique coordonnée par le Groupe d'Étude des Néoplasies Endocriniennes de type 1 (GENEM 1). Les objectifs de ce groupe sont d'établir des protocoles diagnostiques et thérapeutiques et de parfaire la connaissance génétique de cette affection. La première étape de cette démarche consiste à reconnaître le syndrome et à déterminer le bilan à pratiquer, en particulier dans deux circonstances: I) devant une pathologie glandulaire isolée d'allure sporadique atteignant les glandes parathyroïdes, le pancréas endocrine, l'antéhypophyse, les surrénales ou encore devant une tumeur neuroendocrine; 2) devant une forte suspicion de NEM 1. Le but des auteurs est de répondre à ces deux questions en se référant préalablement à l'analyse des 150 premiers cas collectés par le GENEM 1