Diagnosis and Treatment of Gastroenteropancreatic Neuroendocrine Neoplasms

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Cancer Causes, Screening and Diagnosis".

Deadline for manuscript submissions: closed (31 October 2023) | Viewed by 7681

Special Issue Editor

Department of Surgery, Uppsala University Hospital, Uppsala University, Uppsala, Sweden
Interests: neuroendocrine tumors: diagnosis and treatment
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

This Special Issue focuses on the diagnosis and treatment of gastroenteropancreatic tumors, with world-leading experts in the field providing the readers with contemporary treatment and diagnosis algorithms, as well as a glimpse into novel and evolving treatments.

Gastrointestinal pancreatic neuroendocrine neoplasms (GEP-Nets) present many diagnostic and therapeutic challenges due to their relative rarity and complexity. Therefore, the latest findings on biomarkers, peptide receptor-based therapy or imaging therapy, etc., for the treatment of gastroenteropancreatic tumors are exciting. We would like to invite an array of scientific readers to contribute their research to this Special Issue of Cancers, dedicated to the generic topic of the diagnosis and treatment of gastroenteropancreatic tumors, such as gastric neuroendocrine tumors, pancreatic neuroendocrine tumors and small intestinal neuroendocrine tumors.

We would like to thank you in advance for your enthusiastic participation in this exciting Special Issue of Cancers.

Prof. Dr. Peter Stålberg
Guest Editor

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Keywords

  • biomarkers
  • imaging
  • gastric NETs
  • pancreatic NETs
  • small intestinal NETs
  • receptor-based therapy

Published Papers (5 papers)

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Research

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16 pages, 813 KiB  
Article
Pancreatic Neuroendocrine Tumors in MEN1 Patients: Difference in Post-Operative Complications and Tumor Progression between Major and Minimal Pancreatic Surgeries
by Francesco Tonelli, Francesca Marini, Francesca Giusti, Teresa Iantomasi, Francesco Giudici and Maria Luisa Brandi
Cancers 2023, 15(20), 4919; https://doi.org/10.3390/cancers15204919 - 10 Oct 2023
Viewed by 673
Abstract
Pancreatic neuroendocrine neoplasms (PNENs) affect over 80% of patients with multiple endocrine neoplasia type 1 (MEN1). Surgery is usually the therapy of choice, but the real immediate and long-term therapeutic benefit of a partial extensive pancreatic resection remains controversial. We analyzed, in 43 [...] Read more.
Pancreatic neuroendocrine neoplasms (PNENs) affect over 80% of patients with multiple endocrine neoplasia type 1 (MEN1). Surgery is usually the therapy of choice, but the real immediate and long-term therapeutic benefit of a partial extensive pancreatic resection remains controversial. We analyzed, in 43 PNEN MEN1 patients who underwent 19 pancreaticoduodenectomies (PD), 19 distal pancreatectomies (DP), and 5 minimal pancreatectomies, the prevalence of surgery-derived early complications and post-operative pancreatic sequelae, and the PNEN relapse-free survival time after surgery, comparing major (PD+DP) and minimal pancreatic surgeries. No post-operative mortality was observed. Metastatic cancers were found in 12 cases, prevalently from duodenal gastrinoma. Long-term cure of endocrine syndromes, by the 38 major pancreatic resections, was obtained in 78.9% of gastrinomas and 92.9% of insulinomas. In only one patient, hepatic metastases, due to gastrinoma, progressed to death. Out of the 38 major surgeries, only one patient was reoperated for the growth of a new PNEN in the remnant pancreas. No functioning PNEN persistence was reported in the five minimal pancreatic surgeries, PNEN relapse occurred in 60% of patients, and 40% of cases needed further pancreatic resection for tumor recurrence. No significant difference in PNEN relapse-free survival time after surgery was found between major and minimal pancreatic surgeries. Full article
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14 pages, 1069 KiB  
Article
Survival Trends in Patients with Small Intestinal Neuroendocrine Tumours—A Cohort Study in Central Norway
by Oddry Folkestad, Øyvind Hauso, Patricia Mjønes, Reidun Fougner, Hans H. Wasmuth and Reidar Fossmark
Cancers 2023, 15(13), 3272; https://doi.org/10.3390/cancers15133272 - 21 Jun 2023
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Abstract
Improved surgical resection and oncological treatment, or an earlier diagnosis may increase survival in small intestinal neuroendocrine tumours (SI-NETs), but only few studies have examined survival trends. We aimed to examine the trend in overall survival and associated factors in SI-NET patients. All [...] Read more.
Improved surgical resection and oncological treatment, or an earlier diagnosis may increase survival in small intestinal neuroendocrine tumours (SI-NETs), but only few studies have examined survival trends. We aimed to examine the trend in overall survival and associated factors in SI-NET patients. All patients with SI-NETs at a regional hospital from June 2005 to December 2021 (n = 242) were identified, and the cohort was divided in half, constituting a first period (until November 2012) and a second period (from November 2012). Disease and treatment characteristics, including European Neuroendocrine Tumour Society (ENETS) stage, surgery, oncological treatment and survival, were recorded. The majority (n = 205 (84.7%)) were treated surgically and surgery was considered curative in 137 (66.8%) patients. Median survival was longer in the second period (9.0 years 95% CI 6.4–11.7 in the first period vs. median not reached in the second period, p = 0.014), with 5-year survival rates of 63.5% and 83.5%, respectively. ENETS stage and oncological treatment did not differ between the periods, but factors associated with surgical quality, such as lymph node harvest and resection of multiple SI-NETs, were significantly higher in the second period. Age, ENETS stage, time period and tumour resection were independently associated with survival in a multivariate analysis. Full article
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14 pages, 902 KiB  
Article
Surgical Approach to Liver Metastases in GEP-NET in a Tertiary Reference Center
by Frederike Butz, Agata Dukaczewska, Henning Jann, Eva Maria Dobrindt, Lisa Reinhard, Georg Lurje, Johann Pratschke, Peter E. Goretzki, Wenzel Schöning and Martina T. Mogl
Cancers 2023, 15(7), 2048; https://doi.org/10.3390/cancers15072048 - 29 Mar 2023
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Abstract
Indications for liver resection in patients with gastroenteropancreatic neuroendocrine tumors (GEP-NET) vary from liver resection with curative intent to tumor debulking or tissue sampling for histopathological characterization. With increasing expertise, the number of minimally invasive liver surgeries (MILS) in GEP-NET patients has increased. [...] Read more.
Indications for liver resection in patients with gastroenteropancreatic neuroendocrine tumors (GEP-NET) vary from liver resection with curative intent to tumor debulking or tissue sampling for histopathological characterization. With increasing expertise, the number of minimally invasive liver surgeries (MILS) in GEP-NET patients has increased. However, the influence on the oncological outcome has hardly been described. The clinicopathological data of patients who underwent liver resection for hepatic metastases of GEP-NET at the Department of Surgery, Charité—Universitätsmedizin Berlin, were analyzed. Propensity score matching (PSM) was performed to compare MILS with open liver surgery (OLS). In total, 22 patients underwent liver surgery with curative intent, and 30 debulking surgeries were analyzed. Disease-free survival (DFS) was longer than progression-free survival (PFS) (10 vs. 24 months), whereas overall survival (OS) did not differ significantly (p = 0.588). Thirty-nine (75%) liver resections were performed as OLS, and thirteen (25%) as MILS. After PSM, a shorter length of hospital stay was found for the MILS group (14 vs. 10 d, p = 0.034), while neither DFS/PFS nor OS differed significantly. Both curative intended and cytoreductive resection of hepatic GEP-NET metastases achieved excellent outcomes. MILS led to a reduced length of hospital, while preserving a good oncological outcome. Full article
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14 pages, 601 KiB  
Article
Prospective Multicentric Assessment of 68Ga-DOTANOC PET/CT in Grade 1-2 GEP-NET
by Alexandre Lugat, Éric Frampas, Yann Touchefeu, Éric Mirallié, Maëlle Le Bras, Hélène Senellart, Aurore Rauscher, Vincent Fleury, Loïc Campion, Vincent Rohmer, Olivier-François Couturier, Rachida Lebtahi, François Rouzet, Philippe Ruszniewski, Françoise Kraeber-Bodéré, Mickaël Bourgeois and Catherine Ansquer
Cancers 2023, 15(2), 513; https://doi.org/10.3390/cancers15020513 - 14 Jan 2023
Cited by 2 | Viewed by 1908
Abstract
The aim of this multicentric study was to prospectively compare 68Ga-DOTANOC PET/CT versus somatostatin receptor scintigraphy (SRS) with SPECT/CT, combined with multiphasic CT scan and MRI in patients with grade 1 or 2 gastroenteropancreatic neuroendocrine tumors (GEP-NET). Patients with histologically proven grade [...] Read more.
The aim of this multicentric study was to prospectively compare 68Ga-DOTANOC PET/CT versus somatostatin receptor scintigraphy (SRS) with SPECT/CT, combined with multiphasic CT scan and MRI in patients with grade 1 or 2 gastroenteropancreatic neuroendocrine tumors (GEP-NET). Patients with histologically proven grade 1 or 2 GEP-NET with suspicion of recurrence or progression, or with typical aspects of GEP-NET on morphological imaging, were explored with conventional imaging (CI): SRS with SPECT/CT, multiphasic CT scan and/or liver MRI followed by 68Ga-DOTANOC PET/CT. The gold standard was based on histology and imaging follow-up. The data of 105 patients (45 woman and 60 men; median age) were analyzed. 68Ga-DOTANOC PET/CT sensitivity was significantly higher than CI sensitivity in per-patient (98.9% vs. 88.6%, p = 0.016) and per-region (97.6% vs. 75.6%, p < 0.001) analyses, in the detection of the primary (97.9% vs. 78.7%; p = 0.016), peritoneal carcinomatosis (95% vs. 30%, p < 0.001), and bone metastases (100% vs. 33.3%, p = 0.041). 68Ga-DOTANOC PET/CT had an impact on the therapeutic management of 41.9% (44/105) patients compared to decisions based on CI explorations. Our data confirm the superiority of 68Ga-DOTANOC PET/CT over CI in the detection of peritoneal carcinomatosis and bone metastasis, as well as its strong therapeutic impact on the management of patients with grade 1-2 GEP-NETs. Full article
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Review

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22 pages, 8313 KiB  
Review
Surgical Management of Pancreatic Neuroendocrine Tumors
by Megan L. Sulciner and Thomas E. Clancy
Cancers 2023, 15(7), 2006; https://doi.org/10.3390/cancers15072006 - 28 Mar 2023
Cited by 2 | Viewed by 2596
Abstract
Pancreatic neuroendocrine tumors (PNETs) are relatively uncommon malignancies, characterized as either functional or nonfunctional secondary to their secretion of biologically active hormones. A wide range of clinical behavior can be seen, with the primary prognostic indicator being tumor grade as defined by the [...] Read more.
Pancreatic neuroendocrine tumors (PNETs) are relatively uncommon malignancies, characterized as either functional or nonfunctional secondary to their secretion of biologically active hormones. A wide range of clinical behavior can be seen, with the primary prognostic indicator being tumor grade as defined by the Ki67 proliferation index and mitotic index. Surgery is the primary treatment modality for PNETs. While functional PNETs should undergo resection for symptom control as well as potential curative intent, nonfunctional PNETs are increasingly managed nonoperatively. There is increasing data to suggest small, nonfunctional PNETs (less than 2 cm) are appropriate follow with nonoperative active surveillance. Evidence supports surgical management of metastatic disease if possible, and occasionally even surgical management of the primary tumor in the setting of widespread metastases. In this review, we highlight the evolving surgical management of local and metastatic PNETs. Full article
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