Advances and Current Concepts in the Medical Management of Gastroenteropancreatic Neuroendocrine Neoplasms

الملخص EN

Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are rare and heterogeneous group of tumors presenting as localised or metastatic disease and in a subset with distinct clinical syndromes.

Treatment is aimed at controlling the functional syndrome, eradicating the tumor, and/or preventing further tumor growth.

Surgery is the treatment of choice in removing the primary tumor and/or reducing tumor burden but cannot be applied to all patients.

Somatostatin analogs (SS-analogs) obtain control of functional syndromes in the majority of GEP-neuroendocrine tumors (NETs); phase III trials have shown that SS-analogs can be used as first-line antiproliferative treatment in patients with slow-growing GEP-NETs.

The role of the recently approved serotonin inhibitor, telotristat ethyl, and gastrin receptor antagonist, netazepide, is evolving.

Streptozotocin-based chemotherapy has been used for inoperable or progressing pancreatic NENs but the orally administered combination of capecitabine/temozolomide is becoming more popular due to its better tolerability and potential effect in other GEP-NENs.

Phase III trials have shown efficacy of molecular targeted therapies in GEP-NETs and of radionuclide treatment in patients with midgut carcinoid tumors expressing somatostatin receptors.

Most patients will develop disease progression necessitating further therapeutic options.

A combination of currently available treatments along with the molecular signature of each tumor will guide future treatment.