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Neuroendocrine tumor disease : An evolving landscape

Frilling, A. (author)
Åkerström, Göran (author)
Uppsala universitet,Endokrinkirurgi
Falconi, M. (author)
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Pavel, M. (author)
Ramos, J. (author)
Kidd, M. (author)
Modlin, I. M. (author)
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 (creator_code:org_t)
2012
2012
English.
In: Endocrine-Related Cancer. - 1351-0088 .- 1479-6821. ; 19:5, s. R163-R185
  • Research review (peer-reviewed)
Abstract Subject headings
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  • 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.

Keyword

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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