Consensus Guidelines for the Management and Treatment of Neuroendocrine Tumors

Abstract

Neuroendocrine tumors (NETs) are a heterogeneous group of tumors originating in various anatomic locations. The management of this disease poses a significant challenge because of the heterogeneous clinical presentations and varying degree of aggressiveness. The recent completion of several phase III trials, including those evaluating octreotide, sunitinib, and everolimus, demonstrate that rigorous evaluation of novel agents in this disease is possible and can lead to practice-changing outcomes. Nevertheless, there are many aspects to the treatment of NETs that remain unclear and controversial. The North American Neuroendocrine Tumor Society (NANETS) published a set of consensus guidelines in 2010 which provided an overview for the treatment of patients with these malignancies. Here, we present a set of consensus tables intended to complement these guidelines and serve as a quick, accessible reference for the practicing physician.

Role of targeted agents in neuroendocrine tumors: Results from a meta-analysis

“…NETs are a heterogeneous disease arising from various primary sites such as the small intestine or other sites of the gastrointestinal tract, and the lung, 30,31 therefore their management is complicated by a different clinical presentations, clinical disease course, symptoms and degree of aggressiveness. 3 In our analysis, target agents improved the PFS and OS of advanced NETs patients compared with control group. Therefore, we may confirm the important role of targeted agents in treating the advanced NETs and in this setting, the completion of several randomized phase III studies has brought to the approval of 2 new sunitinib, and everolimus.…”

“…1,2 The prognosis of NETs may vary widely, depending on stage, grade or primary tumor site. 3 Surgical resection of the primary tumors usually offer the only change of a long curative treatment; however, only a low percentage of patients are candidate to surgery as more than 50% of patients with NET have regional or distant metastatic disease at diagnosis. 3 In advanced diseases, the therapeutic options may include: a close surveillance for slowly progressive tumors; a liver-directed treatments such as transarterial embolization; systemic treatment with cytotoxic chemotherapy or radionuclide.…”

Hindgut Neuroendocrine Neoplasia

“…Hindgut WDNETs rarely secrete serotonin and as such assessment of Urine 5-HIAA can be considered but is not necessary. [11] …”

“…Systemic cytotoxic chemotherapy has not shown to be of any benefit in metastatic WDNET outside of pancreatic WDNETs. [11] Peptide receptor radionuclide therapy using radiolabeled somatostatin analogs has shown some benefit in metastatic unresectible WDNET however is not currently offered in the US outside of clinical trials. [20] Prognosis & Follow up…”

“…Checking chromogranin A levels can be considered particularly if they were elevated pre-operatively. [11] The prognosis for patients presenting with metastatic hindgut WDNETs is better than that for metastatic hindgut adenocarcinoma. Although rarely curable WDNET are slow growing and as such patients often live with the disease for some time.…”