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Neuroendocrine Neoplasms: Current Challenges and Advances in the Biological Aspects,...
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Neuroendocrine Neoplasms: Current Challenges and Advances in the Biological Aspects, Diagnostic and Therapeutic Management (Volume II)

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

Deadline for manuscript submissions: closed (20 October 2023) | Viewed by 3732

Special Issue Editors

Dr. Salvatore Tafuto

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Guest Editor
Sarcomas and Rare Tumors Unit, Istituto Nazionale Tumori, IRCCS—Fondazione “G. Pascale”, 80131 Naples, Italy
Interests: clinical oncology; rare tumors; neuroendocrine tumors; medical oncology; cancer metastasis
Special Issues, Collections and Topics in MDPI journals
Dr. Claudia von Arx

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Guest Editor
Department of Breast and Thoracic Oncology, Division of Breast Medical Oncology, Istituto Nazionale Tumori IRCCS, Fondazione “G. Pascale”, Naples, Italy
Interests: neuroendocrine tumors; genomic landscape; immune signature; tumor microenviroment; copy number signatures; Axl; immunotherapy; CAR-T cells; STR; PRRT
Special Issues, Collections and Topics in MDPI journals
Dr. Vincenzo Quagliariello

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Guest Editor
Division of Cardiology, Istituto Nazionale Tumori IRCCS, Fondazione “G. Pascale”, Naples, Italy
Interests: inflammation; cancer; cardiology; cardio-oncology; nutrition

Special Issue Information

Dear Colleagues,

This Special Issue is the second edition of the previous collection, "Neuroendocrine Neoplasms: Current Challenges and Advances in the Biological Aspects, Diagnostic and Therapeutic Management" (https://www.mdpi.com/journal/cancers/special_issues/NN).

Neuroendocrine neoplasms (NENs) are a heterogeneous group of tumors with increasing incidence and prevalence. NENs clinical management requires a multidisciplinary approach to positively impact patient survival.

NENs heterogeneity is linked to both biological and clinical characteristics. In recent years, a better understanding of NENs biology and the efforts of different specialists and researchers in investigating NENs treatment and diagnosis strategies have continually evolved NENs management. As a result, many preclinical studies and phase II and phase III clinical trials have been completed and have improved the treatment strategy and diagnostic tools for NENs. However, many other studies are ongoing and are expected to further improve our understanding of the biology of NENs as well as treatment and diagnosis.

This Special Issue aims to gather research on the biological, diagnostic, and therapeutic aspects that can offer real progress in managing NENs patients. Original research and review articles should present new data from basic or clinical research and/or analyze and discuss previous research innovatively and originally.

We look forward to receiving your contributions.

Dr. Salvatore Tafuto
Dr. Claudia von Arx
Dr. Vincenzo Quagliariello
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 neoplasms
  • carcinoid syndrome
  • multidisciplinary team in NENs
  • somatostatin analogs
  • targeted therapies
  • peptide receptor radionuclide therapy (PRRT)

Published Papers (3 papers)

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Research

10 pages, 2613 KiB  
Article
Prognostic Thresholds of Mitotic Count and Ki-67 Labeling Index for Recurrence and Survival in Lung Atypical Carcinoids
by Patrick Soldath, Daniel Bianchi, Beatrice Manfredini, Andreas Kjaer, Seppo W. Langer, Ulrich Knigge, Franca Melfi, Pier Luigi Filosso and René Horsleben Petersen
Cancers 2024, 16(3), 502; https://doi.org/10.3390/cancers16030502 - 24 Jan 2024
Viewed by 965
Abstract
Atypical carcinoid (AC) is a rare neuroendocrine neoplasm of the lung, which exhibits a varying malignant potential. In this study, we aimed to identify the prognostic thresholds of the mitotic count and Ki-67 labeling index for recurrence and survival in AC. We retrospectively [...] Read more.
Atypical carcinoid (AC) is a rare neuroendocrine neoplasm of the lung, which exhibits a varying malignant potential. In this study, we aimed to identify the prognostic thresholds of the mitotic count and Ki-67 labeling index for recurrence and survival in AC. We retrospectively reviewed 78 patients who had been radically resected for AC and calculated said thresholds using time-dependent receiver operating characteristic curves and the Youden index. We then dichotomized the patients into groups of above or below these thresholds and estimated the cumulative incidences of the groups using the Aalen–Johansen estimator. We compared the groups using univariable and multivariable Fine–Gray subdistribution hazard models. Our findings show that more patients recurred and died from this disease if their mitotic count exceeded three and four mitoses per 2 mm2, respectively, or if their Ki-67 labeling index exceeded 14% and 11%, respectively. Both thresholds independently predicted survival (p < 0.001 and p = 0.015, respectively). These thresholds may serve as a valuable tool for clinicians and researchers in making treatment plans and predicting outcomes for patients with AC. Full article
(This article belongs to the Special Issue Neuroendocrine Neoplasms: Current Challenges and Advances in the Biological Aspects, Diagnostic and Therapeutic Management (Volume II))
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24 pages, 2360 KiB  
Article
Diagnostic Approaches for Neuroendocrine Neoplasms of Unknown Primary (NEN-UPs) and Their Prognostic Relevance—A Retrospective, Long-Term Single-Center Experience
by Moritz Schmidt, Clemens Hinterleitner, Stephan Singer, Ulrich M. Lauer, Lars Zender and Martina Hinterleitner
Cancers 2023, 15(17), 4316; https://doi.org/10.3390/cancers15174316 - 29 Aug 2023
Cited by 1 | Viewed by 1062
Abstract
Neuroendocrine neoplasms (NENs) represent a rare and heterogenous group of tumors with predominantly gastroenteropancreatic or pulmonary origin. Despite numerous diagnostic efforts, the primary tumor site remains unknown in up to 20% of the patients diagnosed with NEN. In this subgroup of NEN patients, [...] Read more.
Neuroendocrine neoplasms (NENs) represent a rare and heterogenous group of tumors with predominantly gastroenteropancreatic or pulmonary origin. Despite numerous diagnostic efforts, the primary tumor site remains unknown in up to 20% of the patients diagnosed with NEN. In this subgroup of NEN patients, a standard diagnostic algorithm has not yet been integrated into clinical routine. Of note, an undetermined primary tumor site in NENs is associated with an impaired clinical outcome by at least “formally” limiting treatment options exclusively approved for NENs of a certain histological origin. In this retrospective study, a patient cohort of 113 patients initially diagnosed with NEN of unknown primary (NEN-UP) was analyzed. In 13 patients (11.5%) a primary tumor site could be identified subsequently, amongst others, by performing somatostatin receptor (SSTR)-PET-based imaging, which was irrespective of the initial clinical or demographic features. Diagnostic work-up and therapeutic regimens did not differ significantly between patients with an identified or unidentified primary tumor site; only a detailed immunohistochemical assessment providing additional information on the tumor origin proved to be significantly associated with the detection of a primary tumor site. Our study revealed that a profound diagnostic work-up, particularly including SSTR-PET-based imaging, leads to additional treatment options, finally resulting in significantly improved clinical outcomes for patients with NEN-UPs. Full article
(This article belongs to the Special Issue Neuroendocrine Neoplasms: Current Challenges and Advances in the Biological Aspects, Diagnostic and Therapeutic Management (Volume II))
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Figure 1

17 pages, 4112 KiB  
Article
Analysis of the Clinicopathological Characteristics, Prognosis, and Lymphocyte Infiltration of Esophageal Neuroendocrine Neoplasms: A Surgery-Based Cohort and Propensity-Score Matching Study
by Long Zhang, Boyao Yu, Zhichao Liu, Jinzhi Wei, Jie Pan, Chao Jiang and Zhigang Li
Cancers 2023, 15(6), 1732; https://doi.org/10.3390/cancers15061732 - 13 Mar 2023
Cited by 1 | Viewed by 1329
Abstract
Background: Esophageal neuroendocrine neoplasms (E-NENs) are a rare and poorly reported subtype of esophageal carcinoma. We analyzed the differences in clinicopathological features, prognosis, and tumor-infiltrating lymphocytes (TILs) between E-NENs and esophageal squamous cell carcinoma (ESCC). Methods: A total of 3620 patients who underwent [...] Read more.
Background: Esophageal neuroendocrine neoplasms (E-NENs) are a rare and poorly reported subtype of esophageal carcinoma. We analyzed the differences in clinicopathological features, prognosis, and tumor-infiltrating lymphocytes (TILs) between E-NENs and esophageal squamous cell carcinoma (ESCC). Methods: A total of 3620 patients who underwent esophagectomy were enrolled retrospectively. The study cohort was divided into two groups (E-NENs and ESCC) through propensity-score matching, and the prognosis and TILs were compared between the two groups. The TILs were assessed using tumor specimens (including six cases of ESCC, six cases of neuroendocrine carcinomas [NECs], and six cases of mixed neuroendocrine–non-neuroendocrine neoplasms [MiNENs]). Results: E-NENs accounted for 3.0% (107/3620) of cases, among which there were just 3 neuroendocrine tumor cases, 51 NEC cases, and 53 MiNENs cases. After matching, esophageal neuroendocrine carcinomas (E-NECs) showed both poorer 5-year overall survival (OS; 35.4% vs. 54.8%, p = 0.0019) and recurrence-free survival (RFS; 29.3% vs. 48.9%, p < 0.001) compared with ESCC. However, the differences were not prominent in the subgroup with stage I. No significant survival benefit was observed for E-NECs with multimodal therapy. Multivariate analysis demonstrated that E-NECs are an independent risk factor for OS and RFS. In the exploratory analysis, E-NECs were associated with less infiltration of immune cells compared with ESCC. Conclusion: E-NECs are significantly associated with a poorer prognosis than ESCC except for early-stage disease. The fewer TILs within the tumor microenvironment of E-NECs compared with ESCC results in weaker anti-tumor immunity and may lead to a poorer prognosis. Full article
(This article belongs to the Special Issue Neuroendocrine Neoplasms: Current Challenges and Advances in the Biological Aspects, Diagnostic and Therapeutic Management (Volume II))
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