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Pharmaceuticals (Basel). 2021 Oct 12;14(10). doi: 10.3390/ph14101039.

Somatostatin Analogue Therapy in MEN1-Related Pancreatic Neuroendocrine Tumors from Evidence to Clinical Practice: A Systematic Review.

Pharmaceuticals (Basel, Switzerland)

Anna La Salvia, Franz Sesti, Chiara Grinzato, Rossella Mazzilli, Maria Grazia Tarsitano, Elisa Giannetta, Antongiulio Faggiano

Affiliations

  1. Department of Oncology, 12 de Octubre University Hospital, 28045 Madrid, Spain.
  2. Department of Experimental Medicine, Sapienza University of Rome, 00161 Rome, Italy.
  3. Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, Sapienza University of Rome, 00189 Rome, Italy.

PMID: 34681263 PMCID: PMC8538402 DOI: 10.3390/ph14101039

Abstract

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. We performed a systematic review of the literature, searching for peer-reviewed articles on SSA (octreotide or lanreotide) treatment in MEN1 associated with pNENs. 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. SSAs appear to be an effective and safe treatment option for MEN1-related pNEN, either at localized or advanced stages.

Keywords: MEN-1; efficacy; pancreatic neuroendocrine tumors; personalized treatment; safety; somatostatin analogues

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