Neuroendocrine tumor disease : An evolving landscape

• Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) represent a heterogenous group of tumors arising from a variety of neuroendocrine cell types. The incidence and prevalence of GEP-NENs have markedly increased over the last three decades. Symptoms are often absent in early disease, or vague and nonspecific even in advanced disease. Delayed diagnosis is thus common. Chromogranin A is the most commonly used biomarker but has limitations as does the proliferative marker Ki-67%, which is often used for tumor grading and determination of therapy. The development of amultidimensional prognostic nomogrammay be valuable in predicting tumor behavior and guiding therapy but requires validation. Identification of NENs that express somatostatin receptors (SSTR) allows for SSTR scintigraphy and positron emission tomography imaging using novel radiolabeled compounds. Complete surgical resection of limited disease or endoscopic ablation of small lesions localized in stomach or rectum can provide cure; however, the majority of GEP-NENs are metastatic (most frequently the liver and/or mesenteric lymph nodes) at diagnosis. Selected patients with metastatic diseasemay benefit from advanced surgical techniques including hepatic resection or liver transplantation. Somatostatin analogs are effective for symptomatic treatment and exhibit some degree of antiproliferative activity in small intestinal NENs. There is a place for streptozotocin, temozolomide, and capecitabine in the management of pancreatic NENs, while new agents targeting either mTOR (everolimus) or angiogenic (sunitinib) pathways have shown efficacy in these lesions.

Nyckelord

5 hydroxyindoleacetic acid

angiopeptin

bevacizumab

capecitabine

chromogranin A

cyclophosphamide

dalotuzumab

everolimus

gefitinib

imatinib

Ki 67 antigen

motesanib

octreotide

pasireotide

pazopanib

somatostatin derivative

somatostatin receptor

sorafenib

streptozocin

sunitinib

temozolomide

temsirolimus

thalidomide

vasculotropin antibody

antiproliferative activity

cancer combination chemotherapy

cancer diagnosis

cancer epidemiology

cancer grading

cancer incidence

cancer localization

cancer prognosis

cancer surgery

delayed diagnosis

drug efficacy

gastric neuroendocrine tumor

gastrinoma

gastroenteropancreatic neuroendocrine neoplasm

glucagonoma

human

insulinoma

liver metastasis

lymph node metastasis

midgut neuroendocrine tumor

molecularly targeted therapy

neuroendocrine tumor

pancreatic neuroendocrine tumor

positron emission tomography

protein expression

review

treatment response

tumor ablation

vipoma

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