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Current Updates and Future Directions in Neuroendocrine Neoplams (NENs): Understanding...
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Current Updates and Future Directions in Neuroendocrine Neoplams (NENs): Understanding Biology, Diagnosis, Management and Research Efforts in NENs

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Cancer Causes, Screening and Diagnosis".

Deadline for manuscript submissions: 20 March 2025 | Viewed by 7029

Special Issue Editors

Dr. Jaydira del Rivero

E-Mail Website
Guest Editor
National Cancer Institute (NCI), Bethesda, MD, USA
Interests: neuroendocrine neoplasms; neuroendocrine tumors; neuroendocrine carcinomas; pheochromocytoma/paraganglioma; adrenocortical carcinoma
Dr. Aman Chauhan

E-Mail Website
Co-Guest Editor
Sylvester Comprehensive Cancer Center, University of Miami, Coral Gables, FL, USA
Interests: neuroendocrine cancer; clinical care; neuroendocrine neoplasms; neuroendocrine tumors; neuroendocrine carcinomas

Special Issue Information

Dear Colleagues,

Neuroendocrine neoplasms (NENs) are categorized as neuroendocrine tumors (NETs) or neuroendocrine carcinomas (NECs). These are rare malignancies which occur, for example, in the gastrointestinal tract, islets of the pancreas, lung, adrenal medulla, thyroid C-cells, etc., and are heterogeneous groups of neoplasms with unique tumor biology, natural history, and clinical management issues. Most NETs are sporadic, but they can be part of familial cancer syndromes such as multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2A/B neurofibromatosis type 1 (NF1) or Von Hippel–Lindau (VHL) syndrome. The standard treatment for localized NETs is surgical resection; however, a variety of therapeutic options are available for patients with advanced NETs. When to apply a given option, what combination therapeutic approach should be used, how long treatment should be continued remain unclear and controversial topics. Moreover, patients with these endocrine cancers seek expert advice in the management of their care. This Special Issue will is a collaboration of research which will help us to further understand the biology of endocrine cancers.

Dr. Jaydira del Rivero
Guest Editor

Dr. Aman Chauhan
Co-Guest Editor

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Keywords

  • neuroendocrine neoplasms
  • NENs
  • neuroendocrine carcinomas
  • tumor biology
  • clinical management
  • therapeutic

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Published Papers (4 papers)

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Research

9 pages, 508 KiB  
Article
Radiation Exposure from GEP NET Surveillance
by Jordan Iannuzzi, Caitlin T. Yeo, Vicky Parkins, Dean Ruether, Errol Stewart, Denise Chan, Janice Pasieka and Kirstie Lithgow
Cancers 2024, 16(2), 427; https://doi.org/10.3390/cancers16020427 - 19 Jan 2024
Viewed by 1124
Abstract
Background: Neuroendocrine tumors (NET) are neoplasms that secrete peptides and neuroamines. For gastroenteropancreatic (GEP) NET, surgical resection represents the only curative option. Ten-year imaging surveillance programs are recommended due to long time-to-recurrence following resection. We performed retrospective chart review evaluating radiation exposure and [...] Read more.
Background: Neuroendocrine tumors (NET) are neoplasms that secrete peptides and neuroamines. For gastroenteropancreatic (GEP) NET, surgical resection represents the only curative option. Ten-year imaging surveillance programs are recommended due to long time-to-recurrence following resection. We performed retrospective chart review evaluating radiation exposure and practice patterns from surveillance of completely resected GEP NET. Methods: We performed a retrospective cohort study of cases with well-differentiated GEP NET from January 2005 to July 2020. Location of primary, modality of imaging, and duration of follow-up were collected. Dosimetry data was collected to calculate effective dose. Results: 62 cases were included with 422 surveillance scans performed. Cross-sectional imaging was used in 82% and functional imaging was used in 18% of scans. Mean number of scans per year was 1.25 (0.42–3). Mean total effective dose was 56.05 mSv (SD 45.56; 0 to 198 mSv) while mean total effective dose per year was 10.62 mSv (SD 9.35; 0 to 45 mSv). Over the recommended ten years of surveillance the estimated total effective dose was 106 mSv. Conclusions: Surveillance of completely resected GEP NET results in cumulative radiation doses in the range associated with secondary malignancy development. Strategies to minimize radiation exposure in surveillance should be considered in future guideline development. Full article
(This article belongs to the Special Issue Current Updates and Future Directions in Neuroendocrine Neoplams (NENs): Understanding Biology, Diagnosis, Management and Research Efforts in NENs)
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16 pages, 2040 KiB  
Article
Outlook for 615 Small Intestinal Neuroendocrine Tumor Patients: Recurrence Risk after Surgery and Disease-Specific Survival in Advanced Disease
by Cecilie Slott, Seppo W. Langer, Stine Møller, Jesper Krogh, Marianne Klose, Carsten Palnæs Hansen, Andreas Kjaer, Pernille Holmager, Rajendra Singh Garbyal, Ulrich Knigge and Mikkel Andreassen
Cancers 2024, 16(1), 204; https://doi.org/10.3390/cancers16010204 - 1 Jan 2024
Cited by 2 | Viewed by 2379
Abstract
Background: Small intestinal neuroendocrine tumors (siNET) are one of the most common neuroendocrine neoplasms. Radical surgery is the only curative treatment. Method: We utilized a single-center study including consecutive patients diagnosed from 2000 to 2020 and followed them until death or the end [...] Read more.
Background: Small intestinal neuroendocrine tumors (siNET) are one of the most common neuroendocrine neoplasms. Radical surgery is the only curative treatment. Method: We utilized a single-center study including consecutive patients diagnosed from 2000 to 2020 and followed them until death or the end of study. Disease-specific survival and recurrence-free survival (RFS) were investigated by Cox regression analyses with the inclusion of prognostic factors. Aims/primary outcomes: We identified three groups: (1) disease specific-survival in the total cohort (group1), (2) RFS and disease-specific survival after intended radical surgery (group2), (3) disease specific-survival in patients with unresectable disease or residual tumor after primary resection (group3). Results: In total, 615 patients, with a mean age (SD) 65 ± 11 years were included. Median (IQR) Ki-67 index was 4 (2–7)%. Median disease-specific survival in group1 was 130 months. Median RFS in group2 was 138 months with 5- and 10-year RFS rates of 72% and 59% with age, plasma chromogranin A (p-CgA) and Ki-67 index as prognostic factors. The ten year disease-specific survival rate in group2 was 86%. The median disease-specific survival in group3 was 85 months with age, Ki-67 index, p-CgA and primary tumor resection as prognostic factors. When proliferation was expressed by WHO grade, no difference was observed between G1 vs. G2 for any of the primary outcomes. Conclusions: Recurrence rates remained high 5–10 years after surgery (group2) supporting long-term follow-up. Median disease-specific survival in patient with unresectable disease (group3) was 7 years, with a favorable impact of primary tumor resection. Our data does not support the current grading system since no significant prognostic information was detected in G1 vs. G2 tumors. Full article
(This article belongs to the Special Issue Current Updates and Future Directions in Neuroendocrine Neoplams (NENs): Understanding Biology, Diagnosis, Management and Research Efforts in NENs)
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14 pages, 1591 KiB  
Article
Recurrence-Free Survival and Disease-Specific Survival in Patients with Pancreatic Neuroendocrine Neoplasms: A Single-Center Retrospective Study of 413 Patients
by Stine Møller, Seppo W. Langer, Cecilie Slott, Jesper Krogh, Carsten Palnæs Hansen, Andreas Kjaer, Pernille Holmager, Marianne Klose, Rajendra Singh Garbyal, Ulrich Knigge and Mikkel Andreassen
Cancers 2024, 16(1), 100; https://doi.org/10.3390/cancers16010100 - 24 Dec 2023
Cited by 3 | Viewed by 1362
Abstract
Introduction: The prognosis and impact of different prognostic factors in pancreatic neuroendocrine neoplasms (pNEN) remain controversial. Aim: To investigate prognostic factors for recurrence-free survival and disease-specific survival in patients with pNEN, divided into three groups: patients undergoing surveillance (tumor size < 2 cm, [...] Read more.
Introduction: The prognosis and impact of different prognostic factors in pancreatic neuroendocrine neoplasms (pNEN) remain controversial. Aim: To investigate prognostic factors for recurrence-free survival and disease-specific survival in patients with pNEN, divided into three groups: patients undergoing surveillance (tumor size < 2 cm, group 1), patients followed after curative-intended surgery (group 2), and patients with unresectable disease or residual tumors after resection (group 3). Method: A single-center retrospective study including consecutive patients over a 20-year period. Multivariate Cox regression analyses were performed to identify risk factors. Results: 413 patients were included, with a mean (SD) age of 62 ± 14 years. In group 1 (n = 51), median (IQR) follow-up was 29 (21–34) months, and tumor size was 1.0 (0.8–1.4) cm. One progressed and had a tumor resection. In group 2 (n = 165), follow-up 59 (31–102) months, median tumor size 2 (1.2–3.4) cm, median Ki-67 index 5 (3–10)%, the 5-year recurrence rate was 21%. Tumor size (p < 0.001), Ki-67 index (p = 0.02), and location in the pancreatic head (p < 0.001) were independent risk factors. In group 3 (n = 197), follow-up 19 (6–46) months, median tumor size 4.2 (2.6–7.0) cm, Ki-67 index 17 (9–64)%, the median disease-specific survival was 22 (6–75) months—99 in NET G1; 54 in NET G2; 14 in NET G3; and 6 months in neuroendocrine carcinomas (NEC). Age (p = 0.029), plasma chromogranin A (p = 0.014), and proliferation, expressed by grade (p = 0.001) and Ki-67 index (p < 0.001), were risk factors. Conclusion: Growth in pNET < 2 cm requiring surgery was observed in 1/51. Tumor size, Ki-67 index, and location in the head were prognostic factors for disease recurrence, while age, plasma chromogranin A, and proliferation predicted mortality in patients with unresectable disease or residual tumors after resection. Full article
(This article belongs to the Special Issue Current Updates and Future Directions in Neuroendocrine Neoplams (NENs): Understanding Biology, Diagnosis, Management and Research Efforts in NENs)
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21 pages, 1404 KiB  
Article
Assessment of the Role of Leptin and Adiponectinas Biomarkers in Pancreatic Neuroendocrine Neoplasms
by Agnes Bocian-Jastrzębska, Anna Malczewska-Herman, Violetta Rosiek and Beata Kos-Kudła
Cancers 2023, 15(13), 3517; https://doi.org/10.3390/cancers15133517 - 6 Jul 2023
Cited by 2 | Viewed by 1341
Abstract
Data on the possible connection between circulating adipokines and PanNENs are limited. This novel study aimed to assess the serum levels of leptin and adiponectin and their ratio in patients with PanNENs and to evaluate the possible relationship between them and PanNEN’s grade [...] Read more.
Data on the possible connection between circulating adipokines and PanNENs are limited. This novel study aimed to assess the serum levels of leptin and adiponectin and their ratio in patients with PanNENs and to evaluate the possible relationship between them and PanNEN’s grade or stage, including the presence of metastases. The study group consisted of PanNENs (n = 83), and healthy controls (n = 39). Leptin and adiponectin measurement by an ELISA assay was undertaken in the entire cohort. The serum concentration of adiponectin was significantly higher in the control group compared to the study group (p < 0.001). The concentration of leptin and adiponectin was significantly higher in females than in males (p < 0.01). Anincreased leptin–adiponectin ratio was observed in well-differentiated PanNENs (G1) vs. moderatelydifferentiated PanNENs (G2) (p < 0.05). An increased leptin–adiponectin ratio was found in PanNENs with Ki-67 < 3% vs. Ki-67 ≥ 3% (p < 0.05). PanNENs with distal disease presented lower leptin levels (p < 0.001) and a decreased leptin–adiponectin ratio (p < 0.01) compared with the localized disease group. Leptin, adiponectin, and the leptin–adiponectin ratio may serve as potential diagnostic, prognostic, and predictive biomarkers for PanNENs. Leptin levels and the leptin–adiponectin ratio may play an important role as predictors of malignancy and metastasis in PanNENs. Full article
(This article belongs to the Special Issue Current Updates and Future Directions in Neuroendocrine Neoplams (NENs): Understanding Biology, Diagnosis, Management and Research Efforts in NENs)
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