Introduction: Neuroendocrine neoplasms (NENs) are relatively rare and complex tumors that can be sporadic or hereditary, as in the context of multiple endocrine neoplasia type 1 (MEN1) where patients display a 70% lifelong risk of developing a pancreatic NENs (pNENs). To date, specific personalized treatment for pNENs in patients with MEN1 are lacking. The aim of this study was to systematically analyze the efficacy and safety of somatostatin analogue (SSA) treatment in patients affected by MEN1-related pNENs. Materials and Methods: We performed a systematic review of the literature, searching for peer-reviewed articles on SSA (octreotide or lanreotide) treatment in MEN1 associated with pNENs. Results: We selected 20 studies with a pooled population of 105 MEN1 patients with pNENs. Females were 58.5%, median age was 44 years (18–73). TNM stage at diagnosis was stage I-II in 84.8% and stage IV in 15.2%. The overall response rate (SD+PR+CR) was achieved in 88.3% of cases, with stable disease in 75.6% and objective response in 12.7% of patients. The safety profile was favorable with both SSA agents. Conclusions: SSAs appear to be an effective and safe treatment option for MEN1-related pNEN, either at localized or advanced stages.

SommarioLe neoplasie neuroendocrine (NEN) sono un gruppo eterogeneo di tumori, a insorgenza sporadica o ereditaria nel contesto di sindromi neoplastiche genetiche. Gli analoghi della somatostatina (SSA) sono considerati il trattamento di prima linea per le forme ben differenziate, non resecabili chirurgicamente o metastatiche. In letteratura esistono pochissimi studi dedicati al trattamento con SSA nelle NEN ereditarie e la maggior parte dei dati deriva da casistiche miste con prevalenza di forme sporadiche e da piccole serie o casi clinici. La maggior parte dei dati riguardano i tumori neuroendocrini pancreatici associati a sindrome neoplastica multiendocrina di tipo 1 (MEN1). Questi tumori sembrano essere particolarmente sensibili agli SSA, data l’elevatissima espressione dei recettori somatostatinergici e lo stadio tumorale localizzato nella maggior parte dei casi. Alcuni studi su casistiche dedicate sembrano suggerire un utilizzo precoce degli SSA nella MEN1 come agenti anti-tumorali e anti-secretori.