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Review
. 2011 May;108(18):305-12.
doi: 10.3238/arztebl.2011.0305. Epub 2011 May 6.

Neuroendocrine neoplasms of the gastrointestinal tract

Matthias Schott  1 , Günter Klöppel, Andreas Raffel, Andreas Saleh, Wolfram Trudo Knoefel, Werner A Scherbaum
Affiliations
Review

Neuroendocrine neoplasms of the gastrointestinal tract

Matthias Schott et al. Dtsch Arztebl Int. 2011 May.

Abstract

Background: Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are complex tumors whose incidence is rising and whose treatment requires precise classification and risk stratification.

Method: Selective review of the relevant literature, including recently published guidelines.

Results: GEP-NENs are initially classified by their degree of histological differentiation and their graded cell proliferation (Ki-67 index). In addition, there are GEP-NEN specific TNM staging protocols. The laboratory assessment includes the measurement of general tumor markers (synaptophysin, chromogranin A) as well as specific ones (hormones). The most important imaging technique for diagnosis is octreotide scintigraphy. The surgical treatment of GEP-NEN is based on oncological resection criteria whose aim is to achieve locally radical resection while preserving as much organ function as possible. Metastases, too, may be amenable to resection. The treatment options for unresectable metastases include radiofrequency ablation and chemoembolization, both of which are palliative methods of reducing tumor volume and hormone production. Other chemotherapeutic and nuclear-medical treatments can be applied depending on the extent of metastatic spread, the proliferation index, and the degree of hormone production by the tumor.

Conclusion: The accurate diagnosis and appropriate treatment of GEP-NET currently gives most patients with this tumor a good prognosis, as long as it is discovered early. Early GEP-NETs have a favorable prognosis. Further advances in the diagnosis and treatment of this disease may result from structural changes in patient care, including the establishment of NET centers.

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Figures

Figure 1
Figure 1
Well-differentiated neuroendocrine tumor in the gastric body extending into the mucosa and submucosa and with a greatest diameter less than 1 cm. This tumor can be endoscopically resected. (With kind permission of Springer Science and Business Media)
Figure 2
Figure 2
Well-differentiated neuroendocrine tumor of the stomach with infiltration of the mucosa, submucosa, and muscularis propria and with a greatest diameter of 2 cm. This tumor needs to be surgically resected. (With kind permission of Springer Science and Business Media)
Figure 3
Figure 3
Liver metastases from a malignant insulinoma. Arterial-phase abdominal CT shows an arterially hypervascularized hotspot (a, arrow) which pools contrast further during the portal-venous phase (b, arrow). Catheter angiography with selective imaging of the right hepatic artery in the late arterial phase (c) shows two arterial tumor blushes; the one marked with the arrow correlates to the CT slices in (a) and (b). A further metastasis is marked with an arrowhead. In the parenchymal phase (d), both metastases (arrow and arrowhead) are visible as persisting blush. After chemoembolization (e), tumor blush is no longer demonstrated
Figure 4
Figure 4
Insulinoma in the pancreatic tail. Abdominal CT in the arterial phase (a) shows an 8-mm spherical space-occupying mass in the ventral part of the pancreatic tail, which appears arterially hyperdense (arrow). The maximum intensity projection (b) allows better anatomic characterization of this finding
See this image and copyright information in PMC

Comment in

  • Correspondence (letter to the editor): Incomplete picture.
    Starke AA, Goretzki PE, Saddig C. Starke AA, et al. Dtsch Arztebl Int. 2012 Jan;109(4):66; author reply 66-7. doi: 10.3238/arztebl.2012.0066a. Epub 2012 Jan 27. Dtsch Arztebl Int. 2012. PMID: 22334829 Free PMC article. No abstract available.

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