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Topic: Neuroendocrine Tumors

Neuroendocrine tumors: a multidisciplinary approach for a complex
disease

Rossana Berardi
Medical Oncology Unit, Università Politecnica delle Marche, Azienda Ospedaliero-Universitaria Ospedali Riuniti Umberto I, GM Lancisi,
G Salesi di Ancona, Via Conca 71, 60126 Ancona, Italy.
Correspondence to: Dr. Rossana Berardi, Medical Oncology Unit, Università Politecnica delle Marche, Azienda Ospedaliero-Universitaria
Ospedali Riuniti Umberto I, GM Lancisi, G Salesi di Ancona, Via Conca 71, 60126 Ancona, Italy. E-mail: r.berardi@univpm.it

Neuroendocrine neoplasms include a heterogeneous of neuroendocrine tumors, where somatostatin analogues
group of neoplasms, representing a spectrum of rare (DOTATOC, DOTATATE) are radiolabeled with Lu, Y,
177
90
neoplasms arising in different organism sites with different or In for pre-therapeutic and therapeutic purposes. [5]
111
malignant potential and behavior. They typically occur in
gastrointestinal and bronchopulmonary tracts. There are many cumulative evidences about the
effectiveness and tolerability of this therapeutic approach,
The incidence and prevalence of these neoplasms showed especially in gastro-entero-pancreatic neuroendocrine
a significant increase in the last four decades leading to a tumors.
rising interest in these tumours with remarkable progresses
in their both treatment and management. Nevertheless, Neuroendocrine neoplasms therapy also includes cytotoxic
they are still considered rare diseases with a global clinical agents, expecially in symptomatic patients, in progressive
incidence of 3.65 cases/100,000 per year according disease, in case of moderate or poor differentiation and
to the National Cancer Institute SEER (Surveillance more aggressive features. Chemotherapy schedules used
Epidemiology and End Results) registry. [1] in this setting include alkylating agents (streptozotocin,
dacarbazine, and temozolomide), antimetabolites
Surgery still remains the primary treatment approach (5-fluorouracil, capecitabine), etoposide and platinum
mainly depending on tumour size, stage and patients derivatives (including cisplatin and oxaliplatin). [6]
performance status. However in loco-regional unresectable
and/or metastatic disease, curative surgery is generally not The availability of new targeted agents, such as everolimus
possible, therefore medical therapy is usually primarily and sunitinib, which are effective in advanced and
considered. Several treatment options are available and metastatic pancreatic neuroendocrine tumors, has provided
to date the management of neuroendocrine tumors within new treatment opportunities.
clinical practice is based on a multimodal therapeutic
strategy including surgery and other loco-regional Despite comprehensive and interesting medical progress,
therapies, somatostatin analogs (SSAs), peptide receptor the current available therapeutic options are still
radionuclide therapy (PRRT), cytotoxic agents, biological inadequate for gastrointestinal and lung neuroendocrine
agents (including angiogenesis inhibitors such as sunitinib tumors, mainly due to the lack of in-depth knowledge of
and inhibitors of mammalian target of rapamycin as molecular mechanisms and predictive factors.
everolimus) with a multidisciplinary approach. [2]
Prognostic evaluation is mainly based on their morphologic
SSAs, including octreotide and lanreotide, represent features and proliferation index, according to WHO
effective options in the presence of carcinoid syndrome, classification. [7]
but they also have an antiproliferative effect in secreting
and nonsecreting neuroendocrine tumors. [3,4] Due to the usually long life-expectancy of these patients,
many different lines of therapy are performed according to
PRRT is an emerging treatment modality for advanced
neuroendocrine tumors. It is performed in the treatment This is an open access article distributed under the terms of the Creative
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How to cite this article: Berardi R. Neuroendocrine tumors: a
multidisciplinary approach for a complex disease. J Cancer Metasta
DOI: Treat 2016;2:277-8.
10.20517/2394-4722.2016.50
Received: 07-08-2016; Accepted: 08-08-2016.

©2016 Journal of Cancer Metastasis and Treatment ¦ Published by OAE Publishing Inc. 277