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Current Status of Neuroendocrine Tumors with a Special Focus on...
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Current Status of Neuroendocrine Tumors with a Special Focus on Diagnosis and Novel Treatments—2nd Edition

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

Deadline for manuscript submissions: 31 January 2025 | Viewed by 3068

Special Issue Editor

Dr. Dan Granberg

E-Mail Website
Guest Editor
1. Department of Molecular Medicine and Surgery, Karolinska Institute, Karolinska University Hospital, Solna, Stockholm, Sweden
2. Department of Endocrine Tumors and Sarcomas, Karolinska University Hospital, Solna, Stockholm, Sweden
Interests: neuroendocrine tumors; bronchial carcinoids; peptide receptor radionuclide therapy
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Neuroendocrine tumors are a growing group of tumors, the majority of which malignant, with local or distant metastases at diagnosis. Tumors are divided according to their proliferative rate into grade 1, grade 2, and grade 3 (G1, G2, and G3, respectively). Most tumors are G1 and G2 and hence slow-growing, and patients suffering from such tumors may live for several years. Some tumors, however, are highly proliferative, and patients affected by them have a short survival and poor prognosis. Most G1 and G2 tumors, and some G3 tumors, express somatostatin receptors and may be detected by somatostatin receptor imaging. Diagnostic sensitivity has improved with the introduction of 68Gallium-DOTATOC PET CT. Somatostatin analogues are the main treatment for patients with low proliferative tumors. Other treatments include chemotherapy, PRRT with 177Lutetium-DOTATATE, and targeted therapies, such as Everolimus and Sunitinib, which have led to an improvement in prognosis. The need for newer therapeutic agents is, however, urgent.

This Special Issue will address current knowledge at the epidemiologic, diagnostic, molecular, and therapeutic level, focusing on new diagnostic and therapeutic measures.

Dr. Dan Granberg
Guest Editor

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 tumors
  • somatostatin analogues
  • PRRT
  • chemotherapy
  • 68Gallium-DOTATOC
  • PET
  • CT
  • targeted therapies
  • mTOR
  • inhibitors
  • angiogenesis

Published Papers (2 papers)

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Research

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16 pages, 2071 KiB  
Article
Metronomic Temozolomide (mTMZ) and Bevacizumab—The Safe and Effective Frontier for Treating Metastatic Neuroendocrine Tumors (NETs): A Single-Center Experience
by Çağlar Ünal and Sezer Sağlam
Cancers 2023, 15(23), 5688; https://doi.org/10.3390/cancers15235688 - 1 Dec 2023
Viewed by 938
Abstract
Addressing the persistent challenges in treating metastatic neuroendocrine tumors (NETs) demands ongoing refinement and innovation in therapeutic strategies. This study investigates the potential advantages of combining metronomic temozolomide (mTMZ) with bevacizumab for patients diagnosed with metastatic NETs, particularly focusing on those with a [...] Read more.
Addressing the persistent challenges in treating metastatic neuroendocrine tumors (NETs) demands ongoing refinement and innovation in therapeutic strategies. This study investigates the potential advantages of combining metronomic temozolomide (mTMZ) with bevacizumab for patients diagnosed with metastatic NETs, particularly focusing on those with a Ki-67 index under 55%. Data from 30 patients were analyzed, using key performance indicators such as progression-free survival (PFS), overall survival (OS), and response rates to therapy, to gauge the treatment’s efficacy. The results were encouraging: the median PFS recorded was 16.3 months, and the OS was 25.9 months. The disease control rate (DCR) reached an impressive 86.7%, and the objective response rate (ORR) stood at 63.3%. The treatment regimen was well-tolerated, with no reported instances of grade 4 toxicities. Such a safety profile indicates that this regimen may be particularly advantageous for older, fragile patients who might struggle with conventional dosage levels. These initial findings suggest that the mTMZ and bevacizumab combination could potentially rival the conventional temozolomide–capecitabine therapy in managing metastatic NETs. We aimed to meticulously assess the efficacy of the mTMZ and bevacizumab combination in treating metastatic NETs. Given the initial promising results, a more conclusive understanding of its efficacy will require further research through larger, multicenter prospective clinical trials. Full article
(This article belongs to the Special Issue Current Status of Neuroendocrine Tumors with a Special Focus on Diagnosis and Novel Treatments—2nd Edition)
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Review

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18 pages, 2590 KiB  
Review
Lung Carcinoids: A Comprehensive Review for Clinicians
by Dan Granberg, Carl Christofer Juhlin, Henrik Falhammar and Elham Hedayati
Cancers 2023, 15(22), 5440; https://doi.org/10.3390/cancers15225440 - 16 Nov 2023
Cited by 2 | Viewed by 1841
Abstract
Lung carcinoids are neuroendocrine tumors, categorized as typical or atypical carcinoids based on their histological appearance. While most of these tumors are slow-growing neoplasms, they still possess malignant potential. Many patients are diagnosed incidentally on chest X-rays or CT scans. Presenting symptoms include [...] Read more.
Lung carcinoids are neuroendocrine tumors, categorized as typical or atypical carcinoids based on their histological appearance. While most of these tumors are slow-growing neoplasms, they still possess malignant potential. Many patients are diagnosed incidentally on chest X-rays or CT scans. Presenting symptoms include cough, hemoptysis, wheezing, dyspnea, and recurrent pneumonia. Endocrine symptoms, such as carcinoid syndrome or ectopic Cushing’s syndrome, are rare. Surgery is the primary treatment and should be considered in all patients with localized disease, even when thoracic lymph node metastases are present. Patients with distant metastases may be treated with somatostatin analogues, chemotherapy, preferably temozolomide-based, mTOR inhibitors, or peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE. Most patients have an excellent prognosis. Poor prognostic factors include atypical histology and lymph node metastases at diagnosis. Long-term follow-up is mandatory since metastases may occur late. Full article
(This article belongs to the Special Issue Current Status of Neuroendocrine Tumors with a Special Focus on Diagnosis and Novel Treatments—2nd Edition)
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Planned Papers

The below list represents only planned manuscripts. Some of these manuscripts have not been received by the Editorial Office yet. Papers submitted to MDPI journals are subject to peer-review.

Title: Pathological Features Associated With Lymph Node Disease in Patients with Appendiceal Neuroendocrine Tumors
Authors: Salvador Rodriguez Franco; Sumaya Ghaffar; Ying Jin; Reed Weiss; Mona Hamermesh; Madeline Thomas; Andrii Khomiak; Lia Roque Assumpção; Toshitaka Sugawara; Oskar Franklin; Richard D Schulick; Steven Ahrendt; Marco Del Chiaro; Martin McCarter; Ana L Gleisner
Affiliation: University of Colorado, USA
Abstract: This study aimed to evaluate the role of pathological features, beyond tumor size, in the risk of lymph node metastasis in appendiceal neuroendocrine tumors. Analyzing data from the national cancer database, we found that among 5,353 cases, 18.8% had lymph node metastasis. Focusing on tumors smaller than 2cm, a subject of considerable debate in treatment strategies, we identified lymphovascular invasion as one of the strongest predictors of lymph node disease. Interestingly, extension into the subserosa and beyond, a current factor in the staging system, was not a strong predictor. These findings suggest that careful interpretation of pathological features is needed when selecting therapeutic approaches using current staging systems.

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