Multiple endocrine neoplasia type 1: a review of current diagnostic and treatment approaches

Pancreatic Neuroendocrine Neoplasms in Multiple Endocrine Neoplasia Type 1

“…Somatostatin is a hormone able to inhibit the release of other hormones, cell proliferation and angiogenesis. As their sporadic counterpart, MEN1 pNETs overexpress somatostatin receptors (SSTRs), mainly the subtypes 2 and 5 (SSTR2 and SSTR5); gastrinomas, glucagonomas, VIP-secreting tumors and NF-pNETs overexpress SSTR2 in 80–100% of cases, and insulinomas in about 50–70% of cases [ 38 ]. This molecular characteristic, which distinguishes pNETs from healthy pancreas, enables the use of SSAs for both the control of hormone hypersecretion in functioning pNETs and the reduction of cancer growth both for secreting and non-secreting tumors.…”

“…This molecular characteristic, which distinguishes pNETs from healthy pancreas, enables the use of SSAs for both the control of hormone hypersecretion in functioning pNETs and the reduction of cancer growth both for secreting and non-secreting tumors. Long-lasting synthetic SSAs (octreotide and lanreotide) have high affinity for SSTR2, and they have shown long-term safety and effect in reducing pancreatic hormone hyper-secretion (glucagon, gastrin, and VIP), controlling tumor growth in low-grade pNETs (Ki-67 < 5%) [ 39 ], and reducing both size and number of type 2 gastric neuroendocrine tumors after six months of treatment, leading to their complete disappearance after one year [ 40 ], while they failed to control insulin over-production in a great majority of MEN1 insulinomas [ 38 ].…”

“…mTOR signaling commonly regulates cell proliferation; somatic mutations in genes involved in this regulative pathway have been identified in about 15% of pNETs. The mTOR oral inhibitor everolimus increased PFS from 6 to 11 months in patients with advanced neuroendocrine tumors, including pNETs [ 38 ]. However, pNETs from hereditary syndromes, such as MEN1, were under-represented in pivotal phase III trials on everolimus.…”

Rare cancers, the continued agenda for progress – editor’s special foreword

“…MEN1 tumor suppressor gene. Marini et al comprehensively review the current diagnostic and treatment approaches for this complex multiple tumor syndrome, providing up-to-date insight (clinical practice guidelines were published in 2012) [7]. The authors note that one of the main challenges for clinicians is to reach a compromise between the risk of exposure to repeated doses of ionizing radiation and the benefit of tumor diagnosis at an early stage.…”