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Neuroendocrine Tumors: From Diagnosis to Therapy

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

Deadline for manuscript submissions: 20 April 2025 | Viewed by 5929

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Special Issue Editors

Dr. Damiano Caruso

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Radiology Unit, Department of Surgical and Medical Sciences and Translational Medicine, Sant’Andrea University Hospital, University of Rome Sapienza, Via di Grottarossa 1035, 00189 Rome, Italy
Interests: imaging; oncology; CT; MRI; artificial intelligence; radiomics; response to therapy
Special Issues, Collections and Topics in MDPI journals

Prof. Dr. Francesco Panzuto

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1. Digestive Disease Unit, Sant’Andrea University Hospital, ENETS Center of Excellence or Rome, 00189 Rome, Italy
2. Department of Medical, Surgical Sciences and Translational Medicine, Sapienza University of Rome, 00189 Rome, Italy
Interests: neuroendocrine tumors; gastrointestinal disorders; pancreatic diseases; malnutrition
Special Issues, Collections and Topics in MDPI journals

Dr. Daniela Prosperi

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Nuclear Medicine Unit, University Hospital Sant'Andrea, Via di Grottarossa 1035, 00189 Rome, Italy
Interests: nuclear medicine; PET/CT; response to therapy; PRRT

Dr. Michela Polici

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Radiology Unit, Department of Medical Surgical Sciences and Translational Medicine, Sapienza University of Rome-Sant’Andrea University Hospital, Via di Grottarossa, 1035-1039, 00189 Rome, Italy
Interests: imaging; oncology; CT; MRI; artificial intelligence; radiomics; response to therapy

Special Issue Information

Dear Colleagues,

Neuroendocrine neoplasms (NENs) are considered rare tumors with a wide spectrum of behaviors, manly indolent, but are highly dependent on the presence of high-risk clinical features (such as metastatic disease, grading, ki67, the expression of somatostatin receptors, and primary sites). Diagnoses, using a dual morphological and functional approach in NENs, are based on histology and imaging.

However, these conventional evaluations can have some intrinsic drawbacks, from the limited tissue sampling, in the case of tumor biopsy, to the bias linked to the subjective assessment of medical images. Furthermore, based on the assessment of response to therapy, the morphological RECIST 1.1 criteria could be reductive and not appropriate, especially in the case of target therapy.

In this context, quantitative imaging has acquired relevance as a supporting tool for clinicians in order to overcome the limits associated with the conventional approach, especially in prediction prognosis and in response to therapy. Thus, to outline a structured therapeutic path, one of the future landscapes of NEN management may be the integration of quantitative imaging with conventional evaluations.

This Special Issue will highlight the main strengths and limitations in the management of NENs, from the diagnosis to the response to therapy, and will investigate the emerging role of quantitative biomarkers.

Dr. Damiano Caruso
Prof. Dr. Francesco Panzuto
Dr. Daniela Prosperi
Dr. Michela Polici
Guest Editors

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Keywords

neuroendocrine neoplasms
CT
MR
PET/CT
target therapy
quantitative biomarkers

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Published Papers (6 papers)

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Research

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10 pages, 1192 KiB

Open AccessArticle

miRNA Expression Profiling in G1 and G2 Pancreatic Neuroendocrine Tumors

by Gábor Nyirő, Bálint Kende Szeredás, Ábel Decmann, Zoltan Herold, Bálint Vékony, Katalin Borka, Katalin Dezső, Attila Zalatnai, Ilona Kovalszky and Peter Igaz

Cancers 2024, 16(14), 2528; https://doi.org/10.3390/cancers16142528 - 13 Jul 2024

Viewed by 1140

Abstract

Pancreatic neuroendocrine neoplasms pose a growing clinical challenge due to their rising incidence and variable prognosis. The current study aims to investigate microRNAs (miRNA; miR) as potential biomarkers for distinguishing between grade 1 (G1) and grade 2 (G2) pancreatic neuroendocrine tumors (PanNET). A total of 33 formalin-fixed, paraffin-embedded samples were analyzed, comprising 17 G1 and 16 G2 tumors. Initially, literature-based miRNAs were validated via real-time quantitative reverse transcription polymerase chain reaction (RT-qPCR), confirming significant downregulation of miR-130b-3p and miR-106b in G2 samples. Through next-generation sequencing, we have identified and selected the top six miRNAs showing the highest difference between G1 and G2 tumors, which were further validated. RT-qPCR validation confirmed the downregulation of miR-30d-5p in G2 tumors. miRNA combinations were created to distinguish between the two PanNET grades. The highest diagnostic performance in distinguishing between G1 and G2 PanNETs by a machine learning algorithm was achieved when using the combination miR-106b + miR-130b-3p + miR-127-3p + miR-129-5p + miR-30d-5p. The ROC analysis resulted in a sensitivity of 83.33% and a specificity of 87.5%. The findings underscore the potential use of miRNAs as biomarkers for stratifying PanNET grades, though further research is warranted to enhance diagnostic accuracy and clinical utility. Full article

(This article belongs to the Special Issue Neuroendocrine Tumors: From Diagnosis to Therapy)

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13 pages, 1175 KiB

Open AccessArticle

The Clinical Utility of the NETest in Patients with Small Intestinal Neuroendocrine Neoplasms (Si-NENs): A “Real-Life” Study

by Julian Gertner, Marina Tsoli, Aimee R. Hayes, Luke Furtado O’Mahony, Faidon-Marios Laskaratos, Thomas Glover, Priyesh Karia, Mohsin F. Butt, Oliver Eastwood, Dalvinder Mandair, Martyn Caplin and Christos Toumpanakis

Cancers 2024, 16(14), 2506; https://doi.org/10.3390/cancers16142506 - 10 Jul 2024

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Abstract

Current biomarkers do not adequately predict the behaviour of neuroendocrine neoplasms (NENs). This study assessed the NETest, a multianalyte blood biomarker, in patients with small intestinal NENs (Si-NENs). We studied two patient groups: Group 1: metastatic Si-NENs (n = 102) and Group 2: post-operatively disease-free according to 68Ga-DOTATATE PET (n = 16). NETest scores were ≤20% (normal), 21–40% (low), 41–79% (intermediate), or ≥80% (high). Overall survival (OS) and progression-free survival (PFS) were assessed using the Kaplan–Meier method. Univariate and multivariate analyses were performed using the Cox proportional hazards model. In Group 1, the median NETest score was 40% (IQR: 33.3–46.7%). The NETest value (HR: 1.032, 95% CI: 1.003–1.062, p = 0.033) and high-risk NETest category (HR: 10.5, 95% CI: 1.35–81.7, p = 0.025) were independent predictors of PFS, along with presence of lung metastases, CgA levels > 10 × ULN, and tumour growth rate (TGR). Independent predictors of OS were the NETest value (HR: 1.035, 95% CI: 1.005–1.066, p = 0.024) and high-risk NETest category (HR: 15.2, 95% CI: 1.52–151, p = 0.02), along with presence of lung metastases and CgA levels > 10 × ULN. In Group 2, ROC analysis identified an AUC of 0.909 (95% CI: 0.75–0.100) for prediction of local or metastatic recurrence. Blood NETest scores were associated with PFS and OS in patients with metastatic Si-NENs, along with TGR, CgA > 10 × ULN, and presence of lung metastases. Full article

(This article belongs to the Special Issue Neuroendocrine Tumors: From Diagnosis to Therapy)

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11 pages, 2769 KiB

Open AccessArticle

Effects of Peptide Receptor Radiotherapy in Patients with Advanced Paraganglioma and Pheochromocytoma: A Nation-Wide Cohort Study

by Linda Skibsted Kornerup, Mikkel Andreassen, Ulrich Knigge, Anne Kirstine Arveschoug, Per Løgstup Poulsen, Andreas Kjær, Peter Sandor Oturai, Henning Grønbæk and Gitte Dam

Cancers 2024, 16(7), 1349; https://doi.org/10.3390/cancers16071349 - 29 Mar 2024

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Abstract

Introduction: Pheochromocytomas and paragangliomas are rare neuroendocrine tumours that originate from chromaffin cells within the adrenal medulla or extra-adrenal sympathetic ganglia. Management of disseminated or metastatic pheochromocytomas and paragangliomas continues to pose challenges and relies on limited evidence. Method: In this study, we report retrospective data on median overall survival (OS) and median progression-free survival (PFS) for all Danish patients treated with peptide receptor radionuclide therapy (PRRT) with ¹⁷⁷Lu-Dotatate or ⁹⁰Y-Dotatate over the past 15 years. One standard treatment of PRRT consisted of 4 consecutive cycles with 8–14-week intervals. Results: We included 28 patients; 10 were diagnosed with pheochromocytoma and 18 with paraganglioma. Median age at first PRRT was 47 (IQR 15–76) years. The median follow-up time was 31 (IQR 17–37) months. Eight patients died during follow-up. Median OS was 72 months, and 5-year survival was 65% with no difference between pheochromocytoma and paraganglioma. Patients with germline mutations had better survival than patients without mutations (p = 0.041). Median PFS after the first cycle of PRRT was 30 months. For patients who previously received systemic treatment, the median PFS was 19 months, compared with 32 months for patients with no previous systemic treatment (p = 0.083). Conclusions: The median OS of around 6 years and median PFS of around 2.5 years found in this study are comparable to those reported in previous studies employing PRRT. Based on historical data, the efficacy of PRRT may be superior to ¹³¹I-MIBG therapy, and targeted therapy with sunitinib and PRRT might therefore be considered as first-line treatment in this patient group. Full article

(This article belongs to the Special Issue Neuroendocrine Tumors: From Diagnosis to Therapy)

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16 pages, 3230 KiB

Open AccessArticle

Responses to Medical Treatment in 192 Patients with Pancreatic Neuroendocrine Neoplasms Referred to the Copenhagen Neuroendocrine Tumour Centre in 2000–2020

by Sofie Skovlund Petersen, Stine Møller, Cecilie Slott, Jesper Krogh, Carsten Palnæs Hansen, Andreas Kjaer, Pernille Holmager, Peter Oturai, Rajendra Singh Garbyal, Seppo W. Langer, Ulrich Knigge and Mikkel Andreassen

Cancers 2024, 16(6), 1190; https://doi.org/10.3390/cancers16061190 - 18 Mar 2024

Cited by 1 | Viewed by 1154

Abstract

Background: Given the rarity and heterogeneity of pancreatic neuroendocrine neoplasms (pNEN), treatment algorithms and sequencing are primarily guided by expert opinions with limited evidence. Aim: To investigate overall survival (OS), median progression-free survival (mPFS), and prognostic factors associated with the most common medical treatments for pNEN. Methods: Retrospective single-center study encompassing patients diagnosed and monitored between 2000 and 2020 (n = 192). Results: Median OS was 36 (95% CI: 26–46) months (99 months for grade (G) 1, 62 for G2, 14 for G3, and 10 for neuroendocrine carcinomas). Patients treated with somatostatin analogues (SSA) (n = 59, median Ki-67 9%) had an mPFS of 28 months. Treatment line (HR (first line as reference) 4.1, 95% CI: 1.9–9.1, p ≤ 0.001) emerged as an independent risk factor for time to progression. Patients with a Ki-67 index ≥10% (n = 28) had an mPFS of 27 months. Patients treated with streptozocin/5-fluorouracil (STZ/5FU) (n = 70, first-line treatment n = 68, median Ki-67 10%) had an mPFS of 20 months, with WHO grade serving as an independent risk factor (HR (G1 (n = 8) vs. G2 (n = 57)) 2.8, 95% CI: 1.1–7.2, p-value = 0.031). Median PFS was 21 months for peptide receptor radionuclide therapy (PRRT) (n = 41, first line n = 2, second line n = 29, median Ki-67 8%), 5 months for carboplatin and etoposide (n = 66, first-line treatment n = 60, median Ki-67 80%), and 3 months for temozolomide-based therapy (n = 56, first-line treatment n = 17, median Ki-67 30%). Conclusion: (1) Overall survival was, as expected, highly dependent on grade; (2) median PFS for SSA was around 2.5 years without difference between tumors with Ki-67 above or below 10%; (3) STZ/5FU as first-line treatment exhibited a superior mPFS of 20 months compared to what has historically been reported for targeted treatments; (4) PRRT in G2 pNEN achieved an mPFS similar to first-line chemotherapy; and (5) limited treatment efficacy was observed in high-grade tumors when treated with carboplatin and etoposide or temozolomide. Full article

(This article belongs to the Special Issue Neuroendocrine Tumors: From Diagnosis to Therapy)

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Review

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15 pages, 1217 KiB

Open AccessReview

Therapeutical Usefulness of PD-1/PD-L1 Inhibitors in Aggressive or Metastatic Pituitary Tumours

by Mariana Lopes-Pinto, Ema Lacerda-Nobre, Ana Luísa Silva and Pedro Marques

Cancers 2024, 16(17), 3033; https://doi.org/10.3390/cancers16173033 - 30 Aug 2024

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Abstract

Therapeutic options for pituitary neuroendocrine tumours (PitNETs) refractory to temozolomide are scarce. Immune checkpoint inhibitors (ICIs), particularly inhibitors of the programmed cell death-1 (PD-1) pathway and its ligand (PD-L1), have been experimentally used in aggressive or metastatic PitNETs. We aimed to study the therapeutic usefulness of anti-PD-1 drugs in patients with aggressive or metastatic PitNETs. Published cases and case series involving patients with PitNETs treated with PD-1/PD-L1 inhibitors were reviewed. Demographic data, clinical–pathological features, previous therapies, drug dosage and posology, and the best radiological and biochemical responses, as well as survival data, were evaluated. We identified 29 cases of aggressive (n = 13) or metastatic (n = 16) PitNETs treated with PD-1/PD-L1 inhibitors. The hypersecretion of adrenocorticotropic hormone (ACTH) was documented in eighteen cases (62.1%), seven were prolactinomas (24.1%), and four were non-functioning PitNETs. All patients underwent various therapies prior to using ICIs. Overall, a positive radiological response (i.e., partial/complete radiological response and stable disease) was observed in eighteen of twenty-nine cases (62.1%), of which ten and four were ACTH- and prolactin-secreting PitNETs, respectively. Hormonal levels reduced or stabilised after using ICIs in 11 of the 17 functioning PitNET cases with available data (64.7%). The median survival of patients treated with ICIs was 13 months, with a maximum of 42 months in two ACTH-secreting tumours. Among 29 patients with PitNETs treated with PD-1/PD-L1 inhibitors, the positive radiological and biochemical response rates were 62.1% and 64.7%, respectively. Altogether, these data suggest a promising role of ICIs in patients with aggressive or metastatic PitNETs refractory to other treatment modalities. Full article

(This article belongs to the Special Issue Neuroendocrine Tumors: From Diagnosis to Therapy)

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15 pages, 292 KiB

Open AccessReview

Immunotherapy in Neuroendocrine Neoplasms: A Diamond to Cut

by Esmeralda García-Torralba, Esther Garcia-Lorenzo, Bernard Doger, Francesca Spada and Angela Lamarca

Cancers 2024, 16(14), 2530; https://doi.org/10.3390/cancers16142530 - 13 Jul 2024

Viewed by 903

Abstract

A raise in the incidence of NENs is expected. Therefore, the identification of new therapeutic strategies, such as immunotherapy, remains crucial. To date, immune checkpoint inhibitors as monotherapy have shown modest activity in unselected NENs. Although immunotherapy combos (plus another immune agents or chemotherapy, among others) are potentially more active than single agents, this has not been uniformly confirmed, even in high-grade NENs. Other immunotherapeutic strategies under development include bispecific antibodies, targeting specific tumor antigens like DLL3, and cell therapy. Currently, no predictive immune biomarkers are available to guide clinical decisions. A comprehensive tumor molecular profiling approach needs to be developed for the selection of patients with NEN who could potentially benefit from immunotherapy. Ideally, clinical trials should incorporate this tumor molecular profiling to identify predictive biomarkers and improve efficacy. Achieving this goal requires an international collaborative effort. Full article

(This article belongs to the Special Issue Neuroendocrine Tumors: From Diagnosis to Therapy)

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