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Pharmaceuticals
Special Issues
Neuroendocrine Neoplasms and Endocrine Glands Tumors: Pharmacological and Clinical Aspects
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Neuroendocrine Neoplasms and Endocrine Glands Tumors: Pharmacological and Clinical Aspects

A special issue of Pharmaceuticals (ISSN 1424-8247). This special issue belongs to the section "Pharmacology".

Deadline for manuscript submissions: closed (15 March 2022) | Viewed by 13973

Special Issue Editors

Dr. Alberto Bongiovanni

E-Mail Website
Guest Editor
1. Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori, Meldola, Italy
2. IRCCS - Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST), Meldola, Italy
Interests: neuroendocrine tumors; neuroendocrine carcinoma; thyroid carcinoma; paraganglioma and pheochromocytoma; adrenal gland carcinoma pharmacology; chemotherapy; network gene expression profiling; immunotherapy; tumor microenvironment; immune contexture; stromal cells; epidemiology; toxicity
Dr. Jaume Capdevila

E-Mail Website
Guest Editor
Department of Medical Oncology, Vall d'Hebron University Hospital, Vall d'Hebron Institute of Oncology (VHIO), CIBERONC, Barcelona, Spain
Interests: neuroendocrine tumors; neuroendocrine carcinoma; thyroid carcinoma; paraganglioma and pheochromocytoma; adrenal gland carcinoma pharmacology; chemotherapy; network gene expression profiling; immunotherapy; tumor microenvironment; immune contexture; stromal cells; epidemiology; toxicity
Dr. Marcella Tazzari

E-Mail Website
Guest Editor
Unit of Immunotherapy - Cell Therapy and Biobank, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori, Meldola, Italy
Interests: tumor microenvironment; immune suppression; cancer immunotherapy

Special Issue Information

Dear Colleagues,

Neuroendocrine neoplasias (NENs) represent a heterogeneous group of malignancies with different clinical behaviors. The incidence is estimated at less than 6 in 100,000 people per year. Despite their low incidence, NENs represent tumors with high prevalence.

Often, NEN patient outcomes are affected by several factors. The rarity of these tumors can cause difficulties or delays in diagnosis, limited access to centers with clinical expertise, less effective standard treatments, and poor resources for preclinical and clinical research programs. Hence, NENs are a significant public health issue, and an urgent need exists to better understand the natural history, explore prognostic and predictive biomarkers, develop novel therapies, and facilitate clinical trials. Furthermore, innovative treatments are often not easily available for rare cancers in general and specifically for neuroendocrine and endocrine gland tumors, even though some of them have been paradigmatic models, like peptide radionuclide receptor therapy in well-differentiated NET.

Endocrine gland tumors are also a rare form of cancer, and they can spread throughout the body. Endocrine glands produce hormones that regulate many important functions including mood, metabolism, growth, and sleep. Some of the major glands that make up our endocrine system include:

  • Thyroid gland
  • Parathyroid glands
  • Adrenal glands
  • Pituitary glands

In order to answer these unmet scientific and clinical needs, a strong collaboration between clinicians and laboratory researchers grouped in multidisciplinary cancer teams is required.

This Special Issue aims to give a comprehensive overview of key areas of NEN and endocrine gland tumor research while focusing on preclinical, translational, and clinical studies. Topics of interests include but are not limited to the following:

  1. Gene expression profiling and preclinical pharmacology in the field of NENs and endocrine gland (EG) tumors;
  2. Experimental studies on NEN/EG cell lines and/or primary cultures in vitro and in vivo;
  3. Translational investigation into the mechanism of action of innovative drugs or innovative chemotherapy regimens for the treatment of NENs and endocrine gland tumors;
  4. Optimization of diagnostic pathways in NENs and endocrine gland tumors;
  5. Advanced clinical practice: challenges and therapeutic opportunities;
  6. New diagnostic tools for radionuclide imaging;
  7. New drugs and new side effects: cancer immunotherapy, PRRT, and targeted therapies;
  8. The role of cancer immunotherapy in NENs and endocrine gland neoplasms;
  9. NEN and endocrine gland tumor environment studies, including the role of the tumor microenvironment and interactions with other cell types;
  10. Clinical epidemiology: understanding the natural history of NENs and endocrine gland tumors and associated hereditary syndromes.

We welcome original research, review, meta-analysis, clinical trial, case report, data report, opinion, and study protocol articles.

Dr. Alberto Bongiovanni
Dr. Jaume Capdevila
Dr. Marcella Tazzari
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. Pharmaceuticals is an international peer-reviewed open access monthly 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 tumors
  • neuroendocrine carcinoma
  • thyroid carcinoma
  • paraganglioma and pheochromocytoma
  • adrenal gland carcinoma
  • pharmacology
  • chemotherapy
  • network gene expression profiling
  • immunotherapy
  • tumor microenvironment
  • immune contexture
  • stromal cells
  • epidemiology
  • toxicity

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Published Papers (4 papers)

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Review

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19 pages, 374 KiB  
Review
Carcinoid Syndrome and Hyperinsulinemic Hypoglycemia Associated with Neuroendocrine Neoplasms: A Critical Review on Clinical and Pharmacological Management
by Francesca Spada, Roberta E. Rossi, Elda Kara, Alice Laffi, Sara Massironi, Manila Rubino, Franco Grimaldi, Sherrie Bhoori and Nicola Fazio
Pharmaceuticals 2021, 14(6), 539; https://doi.org/10.3390/ph14060539 - 4 Jun 2021
Cited by 7 | Viewed by 3807
Abstract
The carcinoid syndrome (CS) and hyperinsulinemic hypoglycemia (HH) represent two of the most common clinical syndromes associated with neuroendocrine neoplasms (NENs). The former is mainly related to the serotonin secretion by a small bowel NEN, whereas the latter depends on an insulin hypersecretion [...] Read more.
The carcinoid syndrome (CS) and hyperinsulinemic hypoglycemia (HH) represent two of the most common clinical syndromes associated with neuroendocrine neoplasms (NENs). The former is mainly related to the serotonin secretion by a small bowel NEN, whereas the latter depends on an insulin hypersecretion by a pancreatic insulinoma. Both syndromes/conditions can affect prognosis and quality of life of patients with NENs. They are often diagnosed late when patients become strongly symptomatic. Therefore, their early detection and management are a critical step in the clinical management of NEN patients. A dedicated and experienced multidisciplinary team with appropriate therapeutic strategies is needed and should be encouraged to optimize clinical outcomes. This review aims to critically analyze clinical features, evidence and treatment options of CS and HH and therefore to improve their management. Full article
(This article belongs to the Special Issue Neuroendocrine Neoplasms and Endocrine Glands Tumors: Pharmacological and Clinical Aspects)
14 pages, 5373 KiB  
Review
Activity and Safety of Immune Checkpoint Inhibitors in Neuroendocrine Neoplasms: A Systematic Review and Meta-Analysis
by Alberto Bongiovanni, Brigida Anna Maiorano, Irene Azzali, Chiara Liverani, Martine Bocchini, Valentina Fausti, Giandomenico Di Menna, Ilaria Grassi, Maddalena Sansovini, Nada Riva and Toni Ibrahim
Pharmaceuticals 2021, 14(5), 476; https://doi.org/10.3390/ph14050476 - 17 May 2021
Cited by 17 | Viewed by 3409
Abstract
Immune-checkpoint inhibitors (ICIs) have widened the therapeutic scenario of different cancer types. Phase I/II trials have been designed to evaluate the role of ICIs both as single agents and in combination in neuroendocrine neoplasms (NENs), but as yet no randomized controlled phase III [...] Read more.
Immune-checkpoint inhibitors (ICIs) have widened the therapeutic scenario of different cancer types. Phase I/II trials have been designed to evaluate the role of ICIs both as single agents and in combination in neuroendocrine neoplasms (NENs), but as yet no randomized controlled phase III trials have been carried out. A systematic review and meta-analysis of studies published could help to reduce the biases of single-phase II trials. Efficacy data were obtained on 636 patients. Pooled percentages of the overall response rate (ORR) and disease control rate (DCR) were 10% (95% CI: 6–15%, I2 = 67%, p < 0.1) and 42% (95% CI: 28–56%, I2 = 93%, p < 0.1), respectively. Median progression-free survival (mPFS) was 4.1 months (95% CI 2.6–5.4; I2 = 96%, p < 0.1) and median overall survival (mOS) was 11 months (95% CI 4.8–21.1; I2 = 98%, p < 0.1). Among the ICIs used as single agents, the anti-PD1 toripalimab achieved the highest ORR. Combination regimens were superior to monotherapy, e.g., the ICI combination nivolumab + ipilimumab, and the ICI + anti-angiogenetic combination atezolizumab + bevacizumab, both of which warrant further investigation. Promising efficacy and a good safety profile of ICIs represent a valid opportunity for expanding the therapeutic landscape of NENs. Predictive biomarkers are needed to identify the most suitable candidates for these regimens. Full article
(This article belongs to the Special Issue Neuroendocrine Neoplasms and Endocrine Glands Tumors: Pharmacological and Clinical Aspects)
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24 pages, 1378 KiB  
Review
An Overview on Molecular Characterization of Thymic Tumors: Old and New Targets for Clinical Advances
by Valentina Tateo, Lisa Manuzzi, Claudia Parisi, Andrea De Giglio, Davide Campana, Maria Abbondanza Pantaleo and Giuseppe Lamberti
Pharmaceuticals 2021, 14(4), 316; https://doi.org/10.3390/ph14040316 - 1 Apr 2021
Cited by 16 | Viewed by 3110
Abstract
Thymic tumors are a group of rare mediastinal malignancies that include three different histological subtypes with completely different clinical behavior: the thymic carcinomas, the thymomas, and the rarest thymic neuroendocrine tumors. Nowadays, few therapeutic options are available for relapsed and refractory thymic tumors [...] Read more.
Thymic tumors are a group of rare mediastinal malignancies that include three different histological subtypes with completely different clinical behavior: the thymic carcinomas, the thymomas, and the rarest thymic neuroendocrine tumors. Nowadays, few therapeutic options are available for relapsed and refractory thymic tumors after a first-line platinum-based chemotherapy. In the last years, the deepening of knowledge on thymus’ biological characterization has opened possibilities for new treatment options. Several clinical trials have been conducted, the majority with disappointing results mainly due to inaccurate patient selection, but recently some encouraging results have been presented. In this review, we summarize the molecular alterations observed in thymic tumors, underlying the great biological differences among the different histology, and the promising targeted therapies for the future. Full article
(This article belongs to the Special Issue Neuroendocrine Neoplasms and Endocrine Glands Tumors: Pharmacological and Clinical Aspects)
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Other

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12 pages, 1149 KiB  
Systematic Review
Somatostatin Analogue Therapy in MEN1-Related Pancreatic Neuroendocrine Tumors from Evidence to Clinical Practice: A Systematic Review
by Anna La Salvia, Franz Sesti, Chiara Grinzato, Rossella Mazzilli, Maria Grazia Tarsitano, Elisa Giannetta and Antongiulio Faggiano
Pharmaceuticals 2021, 14(10), 1039; https://doi.org/10.3390/ph14101039 - 12 Oct 2021
Cited by 7 | Viewed by 2900
Abstract
Neuroendocrine neoplasms (NENs) are relatively rare and complex tumors that can be sporadic or hereditary, as in the context of multiple endocrine neoplasia type 1 (MEN1) where patients display a 70% lifelong risk of developing a pancreatic NENs (pNENs). To date, specific personalized [...] Read more.
Neuroendocrine neoplasms (NENs) are relatively rare and complex tumors that can be sporadic or hereditary, as in the context of multiple endocrine neoplasia type 1 (MEN1) where patients display a 70% lifelong risk of developing a pancreatic NENs (pNENs). To date, specific personalized treatment for pNENs in patients with MEN1 are lacking. The aim of this study was to systematically analyze the efficacy and safety of somatostatin analogue (SSA) treatment in patients affected by MEN1-related pNENs. We performed a systematic review of the literature, searching for peer-reviewed articles on SSA (octreotide or lanreotide) treatment in MEN1 associated with pNENs. We selected 20 studies with a pooled population of 105 MEN1 patients with pNENs. Females were 58.5%, median age was 44 years (18–73). TNM stage at diagnosis was stage I–II in 84.8% and stage IV in 15.2%. The overall response rate (SD+PR+CR) was achieved in 88.3% of cases, with stable disease in 75.6% and objective response in 12.7% of patients. The safety profile was favorable with both SSA agents. SSAs appear to be an effective and safe treatment option for MEN1-related pNEN, either at localized or advanced stages. Full article
(This article belongs to the Special Issue Neuroendocrine Neoplasms and Endocrine Glands Tumors: Pharmacological and Clinical Aspects)
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