Are Patients with Multiple Endocrine Neoplasia Type I Prone to Premature Death?

Abstract

Multiple endocrine neoplasia type I (MEN-I) is an autosomal dominant disorder characterized by endocrinopathies involving the anterior pituitary gland, parathyroid glands, and pancreas. The long-term prognosis for patients affected with this disorder is uncertain. To better characterize this prognosis, we performed a retrospective review of all patients with MEN-I treated at a single institution during the period 1951-1997. A group of 233 patients served as the study population. Their records were analyzed for confirmation of diagnosis, treatments received, long-term survival, and cause of death. Altogether, 108 eight male patients (46%) and 125 female patients (54%) were identified. At the conclusion of the study, 164 (70%) were alive and 69 (30%) were deceased, with a median follow-up for patients alive at last contact of 13.4 years (range < 1 month to 54.3 years). The cause of death was reliably obtained in 60 patients. Of these patients, 17 (28%) died of causes related to MEN-I, most commonly metastatic islet cell tumors (10 patients). The remaining patients died of causes unrelated to MEN-I, most commonly coronary artery disease and nonendocrine malignancies (14% each). The overall 20-year survival of MEN-I patients was 64% (95% CI was 56-72%), and that of an age- and gender-matched upper Midwest population was 81% (p < 0.001). Patients with MEN-I appear to be at increased risk of premature death. Earlier diagnosis and treatment of potentially malignant pancreatic islet cell neoplasms may result in a decrease of this premature mortality.

Gender-related differences in MEN1 lesion occurrence and diagnosis: a cohort study of 734 cases from the Groupe d'étude des Tumeurs Endocrines

“…Although this interpretation was obviously not proved, the authors considered that this gender difference was due to a clinical bias. Recently, an overall female predominance was also observed in four MEN1 studies from the United States (nZ233), from the United Kingdom (nZ220), from Germany (nZ301), and from Finland (nZ82), showing proportions of women of 54, 57, 59, and 57%, thus very close to our figures of 58% (12,26,27,30). The 2007 study from Finland was particularly interesting because the cohort was constituted during a comparable period of time (1982)(1983)(1984)(1985)(1986)(1987)(1988)(1989)(1990)(1991)(1992)(1993)(1994)(1995)(1996)(1997)(1998)(1999)(2000)(2001) and was exclusively made of patients with ascertained MEN1 mutations.…”

“…These large cohorts comprised more than 200 MEN1 patients. period of MEN1 diagnosis), and in Germany (nZ306: 1980-2006 period of MEN1 diagnosis) (9,12,26,27). The multicenter aspect of this study reduces the risk of bias encountered in single-center cohorts, which may select patients through a specific aspect of the disease such as the age of the patient, or the organ involved (duodeno-pancreas, pituitary, parathyroid glands, etc.).…”

“…There are large variations between studies in the sex ratio, depending on the center, the population size, and the type of lesion studied. Several cohort studies that did not focus on specific lesions suggested an overall female predominance of about 52-56% of MEN1 patients (11,12). In contrast, studies that focus on specific lesions provided discordant sex ratios with a proportion of females ranging, for example, from 36 to 58% in MEN1 patients with Zollinger-Ellison syndrome (ZES) (13)(14)(15)(16).…”

Natural History of MEN1 GEP‐NET: Single‐Center Experience After a Long Follow‐Up

“…Although scientific progress occurred in both MEN1 GEP-NET diagnosis and treatment, the natural history of the disease remains poorly evaluated [10,[25][26][27][28].…”

Multiple endocrine neoplasia type 1: a case report and review of the literature

“…Of the mutation carriers 82-99% has at least one manifestation of the disease at the age of 50 [1,2,[8][9][10]. Patients with MEN1 have a shorter life expectancy than the general population [1,[11][12][13] with the most important causes of MEN1-related death is malignant pNETs and thymic NETs [1,[11][12][13][14].…”