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Topic: Neuroendocrine Tumors

Controversies in the treatment of digestive neuroendocrine tumors

Maria Rinzivillo, Francesco Panzuto, Gianfranco Delle Fave
Department of Digestive and Liver Disease, Digestive Neuroendocrine Unit, Sant’Andrea Hospital, Sapienza University of Rome, 00189 Rome, Italy.
Correspondence to: Prof. Gianfranco Delle Fave, Department of Digestive and Liver Disease, Digestive Neuroendocrine Unit, Sant’Andrea
Hospital, Sapienza University of Rome, Via di Grottarossa 1035-39, 00189 Rome, Italy. E-mail: gianfranco.dellefave@uniroma1.it

A B S T R AC T
Gastroenteropancreatic neuroendocrine tumors (NETs) have an incidence of 2.39 per 100,000 inhabitants per year, and a prevalence
of 35 cases per 100,000 inhabitants; the gap between these rates is due to the relatively long survival time of these tumors, which
can be thus considered as chronic oncological diseases. Recently, more therapeutic options have become available, but criteria
for defining timing, priority and sequence of different therapeutic options are still debated. This review offers an overview of
pancreatic and small bowel NETs, critically underlining the issues that still need to be clarified and some controversial issues on
the therapeutic approach for NET patients.
Key words: Neuroendocrine tumors; therapeutic strategy; surgical treatment; medical therapies

INTRODUCTION Histological diagnosis: conventional immunohistochemistry
evaluation and Ki67 assessment are needed to classify
Gastroenteropancreatic neuroendocrine tumors (NETs) the disease according with WHO classification, as well as
are a heterogeneous group of neoplasms derived from the define tumor grading;
diffuse endocrine system in the gastrointestinal tract and
pancreas. The WHO classification classifies these tumors Disease staging: conventional contrast enhanced
into three principal categories with different malignant computed tomography (or magnetic resonance
behavior: NETs with Ki67 ≤ 2% (G1 NETs), NETs with imaging) should be performed together with functional
imaging (68 Ga-PET or Octreoscan) to stage the disease
Ki67 3-20% (G2 NETs) and neuroendocrine carcinomas according with the ENETS staging system.
(NECs) with Ki67 > 20% (NECs G3). [1]
(b) Patient’s clinical status:
In the last few decades, the increasing incidence of Performance status;
these diseases has aroused much interest resulting in
improvements in available therapeutic options and new Presence of symptoms resulting from tumor-related
clinical trials. In fact, treatment options for NETs have secretion of active substances, in the case of a
increased in number and this is definitely an advantage for “functioning tumor”;
patients. However, criteria for defining timing, priority and
sequence of different therapeutic options are still debated. Prior treatments and comorbidity, which may reduce
therapeutic options.
The optimal therapeutic sequence should be based on the
evaluation of at least three major issues: (c) Defining the objectives of care:
The only curative option is represented by radical surgery;
(a) Tumor characterization:
Primary site: pancreatic and small bowel NETs should In most patients, since curative surgery is not feasible,
medical treatment is needed to treat advanced
be considered different diseases in terms of both risk
of tumor progression and overall survival; This is an open access article distributed under the terms of the Creative
Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows
others to remix, tweak, and build upon the work non-commercially, as long as
Access this article online the author is credited and the new creations are licensed under the identical
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How to cite this article: Rinzivillo M, Panzuto F, Delle Fave G.
Controversies in the treatment of digestive neuroendocrine tumors. J
DOI: Cancer Metasta Treat 2016;2:304-9.
10.20517/2394-4722.2016.40
Received: 20-06-2016; Accepted: 26-07-2016.
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©2016 Journal of Cancer Metastasis and Treatment ¦ Published by OAE Publishing Inc.