Neuroendocrine Tumors: From Diagnosis to Therapy

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

Deadline for manuscript submissions: 31 August 2024 | Viewed by 1387

Special Issue Editors


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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

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Guest Editor
Digestive and Lilver Disease Unit, Sant’Andrea University Hospital, ENETS Center of Excellence, Rome, Italy
Interests: neuroendocrine tumors; digestive oncology; pancreatic diseases; gut hormones
Special Issues, Collections and Topics in MDPI journals

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

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Guest Editor
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

Published Papers (2 papers)

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Research

11 pages, 2769 KiB  
Article
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 [...] Read more.
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  
Article
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
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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 [...] Read more.
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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