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Cancers
Special Issues
Updates on Diagnostic and Therapeutic Management of Neuroendocrine Neoplasms
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Updates on Diagnostic and Therapeutic Management of Neuroendocrine Neoplasms

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Cancer Therapy".

Deadline for manuscript submissions: closed (30 April 2022) | Viewed by 24186

Special Issue Editors

Dr. Anja Rinke

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Guest Editor
Department of Gastroenterology University Hospital Marburg Baldingerstrasse 1, DE-35043 Marburg, Germany
Interests: neuroendocrine tumors; cancer; treatment
Dr. Jörg Schrader

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Guest Editor
Universitätsklinikum Hamburg-Eppendorf und Medizinische Fakultät, Hamburg, Germany
Interests: gastroenterology and hepatology; pancreatic neuroendocrine tumors
Prof. Dr. Sebastian Krug

E-Mail Website
Guest Editor
Department of Internal Medicine I, Martin Luther University Halle-Wittenberg, 06120 Halle, Germany
Interests: neuroendocrine neoplasms; epigenetics; tumor-associated macrophages; in vitro models; translational studies; RIP1Tag2 mouse model
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Neuroendocrine neoplasms (NENs) are heterogeneous epithelial tumors defined by their neuroendocrine phenotype, which is verified by immunohistochemical staining for synaptophysin and chromogranin A. They can arise almost anywhere in the body but most commonly originate in the gastroenteropancreatic system and lungs. The WHO classification was updated in 2017 (pancreas) and 2019 (gastrointestinal tract), taking differentiation as well as the proliferative activity measured using Ki67 into consideration. The spectrum of clinical presentations as well as of biological behavior and prognosis is extremely broad. Many subgroups are relatively rare, and patients are often diagnosed with advanced disease. Therefore, treatment is often challenging and requires a multidisciplinary approach. In recent years, the understanding of the biology has improved and new therapeutic options including molecular targeted treatments as well as peptide receptor radionuclide therapy (PRRT) have become broadly available. However, there are numerous unmet needs including reliable predictive markers and data on the best sequencing and therapeutic options for rare subgroups.

This Special Issue will focus on the advances in the diagnosis and treatment of gastroenteropancreatic and pulmonary neuroendocrine neoplasms including new treatment approaches and clinical trials.

Dr. Anja Rinke
Dr. Jörg Schrader
Dr. Sebastian Krug
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Cancers is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2900 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • neuroendocrine neoplasm
  • neuroendocrine tumor
  • neuroendocrine carcinoma
  • Ki67
  • carcinoid syndrome
  • gastrinoma
  • insulinoma
  • chemotherapy
  • PRRT
  • locoregional treatment
  • molecular targeted treatment
  • prognosis

Published Papers (10 papers)

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16 pages, 3171 KiB  
Article
Prognostic and Predictive Role of Body Composition in Metastatic Neuroendocrine Tumor Patients Treated with Everolimus: A Real-World Data Analysis
by Nicoletta Ranallo, Andrea Prochoswski Iamurri, Flavia Foca, Chiara Liverani, Alessandro De Vita, Laura Mercatali, Chiara Calabrese, Chiara Spadazzi, Carlo Fabbri, Davide Cavaliere, Riccardo Galassi, Stefano Severi, Maddalena Sansovini, Andreas Tartaglia, Federica Pieri, Laura Crudi, David Bianchini, Domenico Barone, Giovanni Martinelli, Giovanni Luca Frassineti, Toni Ibrahim, Luana Calabrò, Rossana Berardi and Alberto Bongiovanniadd Show full author list remove Hide full author list
Cancers 2022, 14(13), 3231; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers14133231 - 30 Jun 2022
Cited by 5 | Viewed by 2019
Abstract
Neuroendocrine tumors (NETs) are rare neoplasms frequently characterized by an upregulation of the mammalian rapamycin targeting (mTOR) pathway resulting in uncontrolled cell proliferation. The mTOR pathway is also involved in skeletal muscle protein synthesis and in adipose tissue metabolism. Everolimus inhibits the mTOR [...] Read more.
Neuroendocrine tumors (NETs) are rare neoplasms frequently characterized by an upregulation of the mammalian rapamycin targeting (mTOR) pathway resulting in uncontrolled cell proliferation. The mTOR pathway is also involved in skeletal muscle protein synthesis and in adipose tissue metabolism. Everolimus inhibits the mTOR pathway, resulting in blockade of cell growth and tumor progression. The aim of this study is to investigate the role of body composition indexes in patients with metastatic NETs treated with everolimus. The study population included 30 patients with well-differentiated (G1-G2), metastatic NETs treated with everolimus at the IRCCS Romagnolo Institute for the Study of Tumors (IRST) “Dino Amadori”, Meldola (FC), Italy. The body composition indexes (skeletal muscle index [SMI] and adipose tissue indexes) were assessed by measuring on a computed tomography (CT) scan the cross-sectional area at L3 at baseline and at the first radiological assessment after the start of treatment. The body mass index (BMI) was assessed at baseline. The median progression-free survival (PFS) was 8.9 months (95% confidence interval [CI]: 3.4–13.7 months). The PFS stratified by tertiles was 3.2 months (95% CI: 0.9–10.1 months) in patients with low SMI (tertile 1), 14.2 months (95% CI: 2.3 months-not estimable [NE]) in patients with intermediate SMI (tertile 2), and 9.1 months (95% CI: 2.7 months-NE) in patients with high SMI (tertile 3) (p = 0.039). Similarly, the other body composition indexes also showed a statistically significant difference in the three groups on the basis of tertiles. The median PFS was 3.2 months (95% CI: 0.9–6.7 months) in underweight patients (BMI ≤ 18.49 kg/m2) and 10.1 months (95% CI: 3.7–28.4 months) in normal-weight patients (p = 0.011). There were no significant differences in terms of overall survival. The study showed a correlation between PFS and the body composition indexes in patients with NETs treated with everolimus, underlining the role of adipose and muscle tissue in these patients. Full article
(This article belongs to the Special Issue Updates on Diagnostic and Therapeutic Management of Neuroendocrine Neoplasms)
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21 pages, 2191 KiB  
Article
Treatment Approaches and Outcome of Patients with Neuroendocrine Neoplasia Grade 3 in German Real-World Clinical Practice
by Simone Luecke, Christian Fottner, Harald Lahner, Henning Jann, Dominik Zolnowski, Detlef Quietzsch, Patricia Grabowski, Birgit Cremer, Sebastian Maasberg, Ulrich-Frank Pape, Hans-Helge Mueller, Thomas Matthias Gress, Anja Rinke and the members of the German NET Registry
Cancers 2022, 14(11), 2718; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers14112718 - 31 May 2022
Cited by 2 | Viewed by 1869
Abstract
Background: Neuroendocrine neoplasia grade 3 (NEN G3) represents a rare and heterogeneous cancer type with a poor prognosis. The aim of our study was to analyze real-world data from the German NET Registry with a focus on therapeutic and prognostic aspects. Methods: NEN [...] Read more.
Background: Neuroendocrine neoplasia grade 3 (NEN G3) represents a rare and heterogeneous cancer type with a poor prognosis. The aim of our study was to analyze real-world data from the German NET Registry with a focus on therapeutic and prognostic aspects. Methods: NEN G3 patients were identified within the German NET Registry. Demographic data and data on treatments and outcomes were retrieved. Univariate analyses were performed using the Kaplan–Meier-method. Multivariate analysis was performed using a Cox proportional hazard model. Results: Of 445 included patients, 318 (71.5%) were diagnosed at stage IV. Well-differentiated morphology (NET G3) was described in 31.7%, 60% of cases were classified as neuroendocrine carcinoma (NEC), and the median Ki67 value was 50%. First-line treatment comprised chemotherapy in 43.8%, with differences in the choice of regimen with regard to NET or NEC, and surgery in 41.6% of patients. Median overall survival for the entire cohort was 31 months. Stage, performance status and Ki67 were significant prognostic factors in multivariate analysis. Conclusions: The survival data of our national registry compare favorably to population-based data, probably mainly because of a relatively low median Ki67 of 50%. Nevertheless, the best first- and second-line approaches for specific subgroups remain unclear, and an international effort to fill these gaps is needed. Full article
(This article belongs to the Special Issue Updates on Diagnostic and Therapeutic Management of Neuroendocrine Neoplasms)
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12 pages, 565 KiB  
Article
Enucleation Is a Feasible Procedure for Well-Differentiated pNEN—A Matched Pair Analysis
by Anna Nießen, Fabiola A. Bechtiger, Ulf Hinz, Magdalena Lewosinska, Franck Billmann, Thilo Hackert, Markus W. Büchler and Simon Schimmack
Cancers 2022, 14(10), 2570; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers14102570 - 23 May 2022
Cited by 3 | Viewed by 1870
Abstract
The extent of surgical resection in the treatment of pancreatic neuroendocrine neoplasms (pNEN) is still controversial. This study aimed to evaluate the outcomes of enucleation for well-differentiated non-functional (nf) pNEN. Patients undergoing enucleation (2001–2020) were analyzed. Clinicopathological parameters, perioperative outcomes and survival were [...] Read more.
The extent of surgical resection in the treatment of pancreatic neuroendocrine neoplasms (pNEN) is still controversial. This study aimed to evaluate the outcomes of enucleation for well-differentiated non-functional (nf) pNEN. Patients undergoing enucleation (2001–2020) were analyzed. Clinicopathological parameters, perioperative outcomes and survival were assessed. The analysis was performed as a nested case-control study and matched-pair analysis with formal resection. Sixty-one patients undergoing enucleation were identified. Compared to patients undergoing formal resection, enucleation was associated with a significantly shorter median length of operative time (128 (IQR 95–170) versus 263 (172–337) minutes, p < 0.0001) and a significantly lower rate of postoperative diabetes (2% versus 21%, p = 0.0020). There was no significant difference in postoperative pancreatic fistula rate (18% versus 16% type B/C, p = 1.0), Clavien−Dindo ≥ III complications (20% versus 26%, p = 0.5189), readmission rate (12% versus 15%, p = 0.6022) or length of hospital stay (8 (7–11) versus 10 (8–17) days, p = 0.0652). There was no 30-day mortality after enucleation compared to 1.6% (n = 1) after formal resection. 10-year overall survival (OS) and disease-free survival (DFS) was similar between the two groups (OS: 89% versus 77%, p = 0.2756; DFS: 98% versus 91%, p = 0.0873). Enucleation presents a safe surgical approach for well-differentiated nf-pNEN with good long-term outcomes for selected patients. Full article
(This article belongs to the Special Issue Updates on Diagnostic and Therapeutic Management of Neuroendocrine Neoplasms)
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15 pages, 591 KiB  
Article
Pancreaticoduodenectomy Is the Best Surgical Procedure for Zollinger–Ellison Syndrome Associated with Multiple Endocrine Neoplasia Type 1
by Weihua Kong, Max Benjamin Albers, Jerena Manoharan, Joachim Nils Goebel, Peter Herbert Kann, Moritz Jesinghaus and Detlef Klaus Bartsch
Cancers 2022, 14(8), 1928; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers14081928 - 11 Apr 2022
Cited by 9 | Viewed by 1649
Abstract
Aim: The aim of this research was to evaluate the long-term outcome of pancreaticoduodenectomy (PD) versus other duodenopancreatic resections (non-PD) for the surgical treatment of the Zollinger–Ellison syndrome (ZES) in patients with multiple endocrine neoplasia type 1 (MEN1). Methods: Prospectively recorded patients with [...] Read more.
Aim: The aim of this research was to evaluate the long-term outcome of pancreaticoduodenectomy (PD) versus other duodenopancreatic resections (non-PD) for the surgical treatment of the Zollinger–Ellison syndrome (ZES) in patients with multiple endocrine neoplasia type 1 (MEN1). Methods: Prospectively recorded patients with biochemically confirmed MEN1-ZES who underwent duodenopancreatic surgery were retrospectively analyzed in terms of clinical characteristics, complications, cure rate, and long-term morbidity, including quality of life assessment (EORTC QLQ-C30). Results: 35 patients (16 female, 19 male) with MEN1-ZES due to duodenopancreatic gastrinomas with a median age of 42 (range 30–74) years were included. At the time of diagnosis, 28 (80%) gastrinomas were malignant, but distant metastases were only present in one (3%) patient. Eleven patients (31.4%) underwent pancreatoduodenectomy (PD) as the initial procedure, whereas 24 patients underwent non-PD resections involving duodenotomy with gastrinoma excision, enucleation of the pNEN from the head of the pancreas, and peripancreatic lymphadenectomy, either with or without distal pancreatectomy (i.e., either Thompson procedure, n = 12, or DUODX, n = 12). There was no significant difference in perioperative morbidity and mortality between the two groups (p ≥ 0.05). One (9%) patient of the PD group required reoperation for recurrent or metastatic ZES compared to eight (22.8%) patients of the non-PD resection groups. After a median follow-up time of 134 months (range 6–480) nine of 11 (82%) patients in the PD group, two of 12 (16%) patients in the Thompson procedure group, and three of 12 (25%) patients in the DUODX group had normal serum gastrin levels. In addition, the global health QoLScore was better in the PD group (76.9) compared to the Thompson procedure (57.4) and DUODX (59.5) groups. Conclusions: Initial PD seems to be the superior surgical procedure for MEN1-ZES, resulting in a long-term cure rate of about 80%, fewer duodenopancreatic reoperations, and an acceptable quality of life. Full article
(This article belongs to the Special Issue Updates on Diagnostic and Therapeutic Management of Neuroendocrine Neoplasms)
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20 pages, 3929 KiB  
Article
Second MAFA Variant Causing a Phosphorylation Defect in the Transactivation Domain and Familial Insulinomatosis
by Christian Fottner, Stefanie Sollfrank, Mursal Ghiasi, Anke Adenaeuer, Thomas Musholt, Arno Schad, Matthias Miederer, Simin Schadmand-Fischer, Matthias M. Weber, Karl J. Lackner and Heidi Rossmann
Cancers 2022, 14(7), 1798; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers14071798 - 01 Apr 2022
Cited by 5 | Viewed by 2129
Abstract
Adult-onset familial insulinomatosis is a rare disorder with recurrent, severe hypoglycemia caused by multiple insulin-secreting pancreatic tumors. The etiology was unclear until the variant p.Ser64Phe in the transcription factor MAFA, a key coordinator of β-cell insulin secretion, was defined as the cause in [...] Read more.
Adult-onset familial insulinomatosis is a rare disorder with recurrent, severe hypoglycemia caused by multiple insulin-secreting pancreatic tumors. The etiology was unclear until the variant p.Ser64Phe in the transcription factor MAFA, a key coordinator of β-cell insulin secretion, was defined as the cause in two families. We here describe detailed genetic, clinical, and family analyses of two sisters with insulinomatosis, aiming to identify further disease causes. Using exome sequencing, we detected a novel, heterozygous missense variant, p.Thr57Arg, in MAFA’s highly conserved transactivation domain. The impact of the affected region is so crucial that in vitro expression studies replacing Thr57 have already been performed, demonstrating a phosphorylation defect with the impairment of transactivation activity and degradation. However, prior to our study, the link to human disease was missing. Furthermore, mild hyperglycemia was observed in six additional, heterozygote family members, indicating that not only insulinomatosis but also MODY-like symptoms co-segregate with p.Thr57Arg. The pre-described MAFA variant, p.Ser64Phe, is located in the same domain, impairs the same phosphorylation cascade, and results in the same symptoms. We confirm MAFA phosphorylation defects are important causes of a characteristic syndrome, thus complementing the pathophysiological and diagnostic disease concept. Additionally, we verify the high penetrance and autosomal dominant inheritance pattern. Full article
(This article belongs to the Special Issue Updates on Diagnostic and Therapeutic Management of Neuroendocrine Neoplasms)
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11 pages, 1129 KiB  
Article
Prognostic Value of the Largest Lesion Size for Progression-Free Survival in Patients with NET Undergoing Salvage PRRT with [177Lu]Lu-DOTATOC
by Markus Galler, Julian M. M. Rogasch, Kai Huang, Henning Jann, Kristina Plehm, Christoph Wetz and Holger Amthauer
Cancers 2022, 14(7), 1768; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers14071768 - 31 Mar 2022
Cited by 2 | Viewed by 1897
Abstract
(1) Background: retreatment with radionuclide-labeled somatostatin analogues following disease progression after initial treatment cycles is often referred to as salvage peptide receptor radionuclide therapy (salvage PRRT). Salvage PRRT is shown to have a favorable safety profile in patients with metastatic neuroendocrine tumors (NETs), [...] Read more.
(1) Background: retreatment with radionuclide-labeled somatostatin analogues following disease progression after initial treatment cycles is often referred to as salvage peptide receptor radionuclide therapy (salvage PRRT). Salvage PRRT is shown to have a favorable safety profile in patients with metastatic neuroendocrine tumors (NETs), but numerous questions about the efficacy and prognostic or predictive factors remain to be answered. The purpose of this study was to evaluate two parameters that have shown prognostic significance in progression-free survival (PFS) in initial PRRT treatment, namely the size of the largest lesion (LLS) and the De Ritis ratio (aspartate aminotransferase (AST)/alanine aminotransferase (ALT)), as prognostic factors in the context of salvage PRRT. In addition, the PFS after initial PRRT was evaluated as a predictor of the PFS following salvage PRRT. (2) Methods: retrospective, monocentric analysis in 32 patients with NETs (gastroenteropancreatic, 23; unknown primary, 7; kidney, 1; lung, 1) and progression after initial PRRT undergoing retreatment with [177Lu]Lu-DOTATOC. The prognostic values of LLS, the De Ritis ratio, and PFS after initial treatment cycles regarding PFS following salvage PRRT were evaluated with univariable and multivariable Cox regression. PFS was defined as the time from treatment start until tumor progression according to RECIST 1.1 criteria, death from any cause or start of a new treatment due to progression of cancer-related symptoms (namely carcinoid syndrome). (3) Results: progression after salvage PRRT was observed in 29 of 32 patients with median PFS of 10.8 months (95% confidence interval (CI), 8.0–15.9 months). A higher LLS (hazard ratio (HR): 1.03; p = 0.002) and a higher De Ritis ratio (HR: 2.64; p = 0.047) were associated with shorter PFS after salvage PRRT in univariable Cox regression. PFS after initial PRRT was not associated with PFS following salvage PRRT. In multivariable Cox regression, only LLS remained a significant predictor. (4) Conclusions: the size of the largest lesion is easy to obtain and might help identify patients at risk of early disease progression after salvage PRRT. Validation is required. Full article
(This article belongs to the Special Issue Updates on Diagnostic and Therapeutic Management of Neuroendocrine Neoplasms)
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17 pages, 2199 KiB  
Article
Quantitative Analysis of Plasma Cell-Free DNA and Its DNA Integrity and Hypomethylation Status as Biomarkers for Tumor Burden and Disease Progression in Patients with Metastatic Neuroendocrine Neoplasias
by Esther Mettler, Christian Fottner, Neda Bakhshandeh, Anja Trenkler, Robert Kuchen and Matthias M. Weber
Cancers 2022, 14(4), 1025; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers14041025 - 17 Feb 2022
Cited by 11 | Viewed by 2459
Abstract
Background: Neuroendocrine neoplasia (NEN) encompasses a diverse group of malignancies marked by histological heterogeneity and highly variable clinical outcomes. Apart from Chromogranin A, specific biomarkers predicting residual tumor disease, tumor burden, and disease progression in NEN are scant. Thus, there is a strong [...] Read more.
Background: Neuroendocrine neoplasia (NEN) encompasses a diverse group of malignancies marked by histological heterogeneity and highly variable clinical outcomes. Apart from Chromogranin A, specific biomarkers predicting residual tumor disease, tumor burden, and disease progression in NEN are scant. Thus, there is a strong clinical need for new and minimally invasive biomarkers that allow for an evaluation of the prognosis, clinical course, and response to treatment of NEN patients, thereby helping implement individualized treatment decisions in this heterogeneous group of patients. In the current prospective study, we evaluated the role of plasma cell-free DNA concentration and its global hypomethylation and fragmentation as possible diagnostic and prognostic biomarkers in patients with neuroendocrine neoplasias. Methods: The plasma cfDNA concentration, cfDNA Alu hypomethylation, and LINE-1 cfDNA integrity were evaluated prospectively in 63 NEN patients with presumably cured or advanced metastatic disease. The cfDNA characteristics in NEN patients were compared to the results of a group of 29 healthy controls and correlated with clinical and histopathological data of the patients. Results: Patients with advanced NEN showed a significantly higher cfDNA concentration and percentage of Alu hypomethylation and a reduced LINE-1 cfDNA integrity as compared to the surgically cured NET patients and the healthy control group. The increased hypomethylation and concentration of cfDNA and the reduced cfDNA integrity in NEN patients were strongly associated with tumor burden and poor prognosis, while no correlation with tumor grading, differentiation, localization, or hormonal activity could be found. Multiparametric ROC analysis of plasma cfDNA characteristics was able to distinguish NEN patients with metastatic disease from the control group and the cured NEN patients with AUC values of 0.694 and 0.908, respectively. This was significant even for the group with only a low tumor burden. Conclusions: The present study, for the first time, demonstrates that the combination of plasma cfDNA concentration, global hypomethylation, and fragment length pattern has the potential to serve as a potent and sensitive prognostic and therapeutic “liquid biopsy” biomarker for tumor burden and disease progression in patients with neuroendocrine neoplasias. Full article
(This article belongs to the Special Issue Updates on Diagnostic and Therapeutic Management of Neuroendocrine Neoplasms)
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15 pages, 2232 KiB  
Article
The Patient’s Point of View: COVID-19 and Neuroendocrine Tumor Disease
by Sebastian Krug, Maryam Khosravian, Julia Weissbach, Katharina George, Marko Damm, Jakob Garbe, Jens Walldorf, Philipp A. Reuken, Tania Amin, Alexander Siebenhüner, Jonas Rosendahl, Thomas M. Gress, Patrick Michl, Jörg Schrader and Anja Rinke
Cancers 2022, 14(3), 613; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers14030613 - 26 Jan 2022
Cited by 3 | Viewed by 2353
Abstract
The assessment of cancer patient care during the COVID-19 pandemic has been mainly reported from a physician’s perspective. Patients with rare tumor entities such as neuroendocrine tumors (NET), which require a complex and specialized care infrastructure, were highly affected by the COVID-19 crisis. [...] Read more.
The assessment of cancer patient care during the COVID-19 pandemic has been mainly reported from a physician’s perspective. Patients with rare tumor entities such as neuroendocrine tumors (NET), which require a complex and specialized care infrastructure, were highly affected by the COVID-19 crisis. Using a structured questionnaire consisting of a general section on the disease and a special COVID-19 section to record medical care, vaccination behavior as well as social and psycho-emotional parameters were collected from NET patients. The survey was distributed via direct medical contact and via the patient organization NETZWERK NeT. A total of 684 patients participated in the survey and 79.2% (n = 542) of the participants answered the questionnaire completely (54 questions). Patient characteristics were comparable to those in large NET registries. The majority of participants were patients with pancreatic and small bowel NET on somatostatin analogue (SSA) therapy. Medical care under COVID-19 was adequate and appointment cancellations and postponements were not common. Nevertheless, the majority of patients were worried about adequate treatment for their tumor disease during the crisis. Most of the participants considered themselves to be at risk of severe COVID-19 infection and were therefore very concerned. This was accompanied by an extremely high vaccination readiness rate of 90%. Increased distress in the social and psycho-emotional domains in the course of the crisis reflected a need for optimization in the medical care of NET patients, although the rate of COVID-19 positive participants was low (3.7%). Therefore, patient-reported measurements are required to identify and address all areas of medical care. Overall, our survey provides an essential contribution to the care of NET patients during the COVID-19 pandemic from the patient’s perspective. Full article
(This article belongs to the Special Issue Updates on Diagnostic and Therapeutic Management of Neuroendocrine Neoplasms)
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Review

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27 pages, 425 KiB  
Review
Advances in the Diagnosis and Therapeutic Management of Gastroenteropancreatic Neuroendocrine Neoplasms (GEP-NENs)
by Krzysztof Kaliszewski, Maksymilian Ludwig, Maria Greniuk, Agnieszka Mikuła, Karol Zagórski and Jerzy Rudnicki
Cancers 2022, 14(8), 2028; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers14082028 - 17 Apr 2022
Cited by 6 | Viewed by 2260
Abstract
Neuroendocrine neoplasms (NENs) are an increasingly common cause of neoplastic diseases. One of the largest groups of NENs are neoplasms localized to the gastroenteropancreatic system, which are known as gastroenteropancreatic NENs (GEP-NENs). Because of nonspecific clinical symptoms, GEP-NEN patient diagnosis and, consequently, their [...] Read more.
Neuroendocrine neoplasms (NENs) are an increasingly common cause of neoplastic diseases. One of the largest groups of NENs are neoplasms localized to the gastroenteropancreatic system, which are known as gastroenteropancreatic NENs (GEP-NENs). Because of nonspecific clinical symptoms, GEP-NEN patient diagnosis and, consequently, their treatment, might be difficult and delayed. This situation has forced researchers all over the world to continue progress in the diagnosis and treatment of patients with GEP-NENs. Our review is designed to present the latest reports on the laboratory diagnostic techniques, imaging tests and surgical and nonsurgical treatment strategies used for patients with these rare neoplasms. We paid particular attention to the nuclear approach, the use of which has been applied to GEP-NEN patient diagnosis, and to nonsurgical and radionuclide treatment strategies. Recent publications were reviewed in search of reports on new strategies for effective disease management. Attention was also paid to those studies still in progress, but with successful results. A total of 248 papers were analyzed, from which 141 papers most relevant to the aim of the study were selected. Using these papers, we highlight the progress in the development of diagnostic and treatment strategies for patients with GEP-NENs. Full article
(This article belongs to the Special Issue Updates on Diagnostic and Therapeutic Management of Neuroendocrine Neoplasms)
16 pages, 548 KiB  
Review
Multimodal Management of Grade 1 and 2 Pancreatic Neuroendocrine Tumors
by Ugo Marchese, Martin Gaillard, Anna Pellat, Stylianos Tzedakis, Einas Abou Ali, Anthony Dohan, Maxime Barat, Philippe Soyer, David Fuks and Romain Coriat
Cancers 2022, 14(2), 433; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers14020433 - 15 Jan 2022
Cited by 9 | Viewed by 4741
Abstract
Pancreatic neuroendocrine tumors (p-NETs) are rare tumors with a recent growing incidence. In the 2017 WHO classification, p-NETs are classified into well-differentiated (i.e., p-NETs grade 1 to 3) and poorly differentiated neuroendocrine carcinomas (i.e., p-NECs). P-NETs G1 and G2 are often non-functioning tumors, [...] Read more.
Pancreatic neuroendocrine tumors (p-NETs) are rare tumors with a recent growing incidence. In the 2017 WHO classification, p-NETs are classified into well-differentiated (i.e., p-NETs grade 1 to 3) and poorly differentiated neuroendocrine carcinomas (i.e., p-NECs). P-NETs G1 and G2 are often non-functioning tumors, of which the prognosis depends on the metastatic status. In the localized setting, p-NETs should be surgically managed, as no benefit for adjuvant chemotherapy has been demonstrated. Parenchymal sparing resection, including both duodenum and pancreas, are safe procedures in selected patients with reduced endocrine and exocrine long-term dysfunction. When the p-NET is benign or borderline malignant, this surgical option is associated with low rates of severe postoperative morbidity and in-hospital mortality. This narrative review offers comments, tips, and tricks from reviewing the available literature on these different options in order to clarify their indications. We also sum up the overall current data on p-NETs G1 and G2 management. Full article
(This article belongs to the Special Issue Updates on Diagnostic and Therapeutic Management of Neuroendocrine Neoplasms)
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