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Current Treatment Options in Oncology
Published in: Current Treatment Options in Oncology 6/2022

01-06-2022 | Neuroendocrine Tumor | Neuroendocrine Cancers (M Cives, Section Editor)

Surgery in Patients with Gastro-Entero-Pancreatic Neuroendocrine Carcinomas, Neuroendocrine Tumors G3 and High Grade Mixed Neuroendocrine-Non-Neuroendocrine Neoplasms

Authors: Pernille Holmager, MD, PhD, Seppo W. Langer, MD, PhD, Andreas Kjaer, MD, DMSci, Lene Ringholm, MD, PhD, Rajendra Singh Garbyal, MD, Hans-Christian Pommergaard, MD, PhD, Carsten Palnæs Hansen, MD, DMSci, Birgitte Federspiel, MD, Mikkel Andreassen, MD, PhD, Ulrich Knigge, MD, DMSci

Published in: Current Treatment Options in Oncology | Issue 6/2022

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Opinion statement

In the 2019 WHO guidelines, the classification of gastro-entero-pancreatic neuroendocrine neoplasms (GEP NEN) has changed from one being based on Ki-67 proliferation index alone to one that also includes tumor differentiation. Consequently, GEP NENs are now classified as well-differentiated neuroendocrine tumor (NET), NET G1 (Ki-67 <3%), NET G2 (Ki-67 3–20%) and NET G3 (Ki-67 >20%), and poorly differentiated neuroendocrine carcinoma (NEC) (Ki-67 >20%). It has been suggested that NET G3 should be treated as NET G2 with respect to surgery, while surgical management of NEC should be expanded from local disease to also include patients with advanced disease where curative surgery is possible. High grade mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) have a neuroendocrine and a non-neuroendocrine component mostly with a poor prognosis. All studies evaluating the effect of surgery in NEC and MiNEN are observational and hold a risk of selection bias, which may overestimate the beneficial effect of surgery. Further, only a few studies on the effect of surgery in MiNEN exist. This review aims to summarize the data on the outcome of surgery in patients with GEP NET G3, GEP NEC and high grade MiNEN. The current evidence suggests that patients with NEN G3 and localized disease and NEN G3 patients with metastatic disease where curative surgery can be achieved may benefit from surgery. In patients with MiNEN, it is currently not possible to evaluate on the potential beneficial effect of surgery due to the low number of studies.
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Metadata
Title
Surgery in Patients with Gastro-Entero-Pancreatic Neuroendocrine Carcinomas, Neuroendocrine Tumors G3 and High Grade Mixed Neuroendocrine-Non-Neuroendocrine Neoplasms
Authors
Pernille Holmager, MD, PhD
Seppo W. Langer, MD, PhD
Andreas Kjaer, MD, DMSci
Lene Ringholm, MD, PhD
Rajendra Singh Garbyal, MD
Hans-Christian Pommergaard, MD, PhD
Carsten Palnæs Hansen, MD, DMSci
Birgitte Federspiel, MD
Mikkel Andreassen, MD, PhD
Ulrich Knigge, MD, DMSci
Publication date
01-06-2022
Publisher
Springer US
Published in
Current Treatment Options in Oncology / Issue 6/2022
Print ISSN: 1527-2729
Electronic ISSN: 1534-6277
DOI
https://doi.org/10.1007/s11864-022-00969-x

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