MEN1 Bone Complications

Multiple endocrine neoplasia type 1 (MEN1) is a rare congenital tumor syndrome affecting multiple endocrine glands and, thus, being responsible for the development of a variety of endocrine syndromes that can secondarily alter the correct metabolism and function of other organs not directly affected by MEN1-related tumors, such as the skeleton and the kidneys. In the last decades, a few studies have shown that patients affected by MEN1 have a lower bone mass and a reduced bone mineral density (BMD) when compared with the general population of the same age and gender. It has been hypothesized that MEN1-associated functioning tumors, secreting an excess of hormones, and the derived endocrine syndromes may have a direct role in altering the correct bone metabolism, starting from the childhood, and continuing during adolescence and early adulthood, thus, significantly influencing the achievement of correct peak bone mass and the physiological bone modeling and remodeling, making the skeleton of MEN1 patients more prone to develop an early-onset osteopenia/osteoporosis and at increased risk of pathological fractures. Therefore, a regular evaluation of bone metabolism and bone mass should be included as part of the routine clinical monitoring of MEN1 patients starting from early adolescence, to monitor skeletal health and decide for a possible early therapeutic intervention to prevent the occurrence of fragility fracture. Here, we report the case of a young male MEN1 patients with a prolactin-secreting macroadenoma, which was not controlled by pharmacological therapy, who develop a severe osteoporosis at a very young age as a consequence of a long-lasting secondary hypogonadism.