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Cancers
Special Issues
Management of Neuroendocrine Neoplasms
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Management of Neuroendocrine Neoplasms

A special issue of Cancers (ISSN 2072-6694).

Deadline for manuscript submissions: closed (31 December 2019) | Viewed by 40539

Special Issue Editor

Prof. Dr. Sylvia L. Asa

grade E-Mail Website
Guest Editor
Department of Pathology, Case Western Reserve University and University Hospitals Cleveland, Cleveland, OH 44106, USA
Interests: endocrine pathology; endocrine oncology; neuroendocrine tumors; pathophysiology; molecular pathology
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Neuroendocrine neoplasms are increasing in incidence. The complexity of these tumors is manifested in their dual impact on patients: Not only do they grow and metastasize like other cancers, but they also make hormones that result in clinical syndromes, causing signs and symptoms that impact quality of life. Because they tend not to be rapidly proliferative, the usual approach to these tumors with chemotherapeutic agents has not been successful. However, recent advances in understanding the genetic and epigenetic alterations that underlie neuroendocrine tumors provide novel approaches to their treatment, while also clarifying the role of genetic screening for early detection. In this Special Issue, experts in this field will review the current approaches to the management of patients with the spectrum of neuroendocrine neoplasia.

Prof. Dr. Sylvia L. Asa
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 tumor
  • Pituitary
  • Medullary thyroid carcinoma
  • Parathyroid
  • Pancreas
  • Small bowel
  • Lung
  • Pheochromcytoma
  • Paraganglioma

Published Papers (5 papers)

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Review

17 pages, 1642 KiB  
Review
Parathyroid Cancer: A Review
by Nikita N Machado and Scott M Wilhelm
Cancers 2019, 11(11), 1676; https://doi.org/10.3390/cancers11111676 - 28 Oct 2019
Cited by 42 | Viewed by 5680
Abstract
Parathyroid cancer is one of the rarest causes of primary hyperparathyroidism and tends to present with more severe symptoms than its more benign counterparts. This article details various aspects of the disease process, including epidemiology, clinical presentation, and a step-wise diagnostic process for [...] Read more.
Parathyroid cancer is one of the rarest causes of primary hyperparathyroidism and tends to present with more severe symptoms than its more benign counterparts. This article details various aspects of the disease process, including epidemiology, clinical presentation, and a step-wise diagnostic process for parathyroid cancer. This includes laboratory assessments as well as a proposed staging system. The en bloc principle of surgical intervention is detailed, as well as the current role of adjuvant treatments. A general guide to surveillance and the natural history of the disease is also outlined. Full article
(This article belongs to the Special Issue Management of Neuroendocrine Neoplasms)
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13 pages, 1600 KiB  
Review
Orthopedia Homeobox (OTP) in Pulmonary Neuroendocrine Tumors: The Diagnostic Value and Possible Molecular Interactions
by Laura Moonen, Jules Derks, Anne-Marie Dingemans and Ernst-Jan Speel
Cancers 2019, 11(10), 1508; https://doi.org/10.3390/cancers11101508 - 08 Oct 2019
Cited by 22 | Viewed by 3639
Abstract
Generally, patients with stage I-IIIa (TNM) pulmonary carcinoid disease have a favourable prognosis after curative resection. Yet, distant recurrence of disease after curative surgery occurs in approximately 1–6% of patients with typical carcinoid and 14–29% in patients with atypical carcinoid disease, respectively. Known [...] Read more.
Generally, patients with stage I-IIIa (TNM) pulmonary carcinoid disease have a favourable prognosis after curative resection. Yet, distant recurrence of disease after curative surgery occurs in approximately 1–6% of patients with typical carcinoid and 14–29% in patients with atypical carcinoid disease, respectively. Known predictors of distant recurrence of disease are atypical carcinoid, lymphatic involvement, and incomplete resection status. However, none of them can be reliably used, alone or in combination, to exclude patients from long-term follow-up (advised 15 years). By genomic profiling, Orthopedia homeobox (OTP) has been identified as a promising prognostic marker for pulmonary carcinoid with a favourable prognosis and low risk of distant disease recurrence. Moreover, OTP is a highly specific marker for carcinoids of pulmonary origin and recent genome wide analysis has identified OTP as a crucial predictor of aggressive tumor behaviour. OTP in combination with CD44, a stem cell marker and cell-surface protein, enables the identification of patients with surgical resected carcinoid disease that could potentially be excluded from long-term follow-up. In future clinical practice OTP may enable clinicians to reduce the diagnostic burden and related distress and reduce costs of long-term radiological assessments in patients with a pulmonary carcinoid. This review addresses the current clinical value of OTP and the possible molecular mechanisms regulating OTP expression and function in pulmonary carcinoids. Full article
(This article belongs to the Special Issue Management of Neuroendocrine Neoplasms)
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27 pages, 1407 KiB  
Review
Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine
by Svenja Nölting, Martin Ullrich, Jens Pietzsch, Christian G. Ziegler, Graeme Eisenhofer, Ashley Grossman and Karel Pacak
Cancers 2019, 11(10), 1505; https://doi.org/10.3390/cancers11101505 - 08 Oct 2019
Cited by 103 | Viewed by 12699
Abstract
Pheochromocytomas and paragangliomas (PCC/PGLs) are rare, mostly catecholamine-producing neuroendocrine tumors of the adrenal gland (PCCs) or the extra-adrenal paraganglia (PGL). They can be separated into three different molecular clusters depending on their underlying gene mutations in any of the at least 20 known [...] Read more.
Pheochromocytomas and paragangliomas (PCC/PGLs) are rare, mostly catecholamine-producing neuroendocrine tumors of the adrenal gland (PCCs) or the extra-adrenal paraganglia (PGL). They can be separated into three different molecular clusters depending on their underlying gene mutations in any of the at least 20 known susceptibility genes: The pseudohypoxia-associated cluster 1, the kinase signaling-associated cluster 2, and the Wnt signaling-associated cluster 3. In addition to tumor size, location (adrenal vs. extra-adrenal), multiplicity, age of first diagnosis, and presence of metastatic disease (including tumor burden), other decisive factors for best clinical management of PCC/PGL include the underlying germline mutation. The above factors can impact the choice of different biomarkers and imaging modalities for PCC/PGL diagnosis, as well as screening for other neoplasms, staging, follow-up, and therapy options. This review provides a guide for practicing clinicians summarizing current management of PCC/PGL according to tumor size, location, age of first diagnosis, presence of metastases, and especially underlying mutations in the era of precision medicine. Full article
(This article belongs to the Special Issue Management of Neuroendocrine Neoplasms)
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21 pages, 1286 KiB  
Review
Management of Small Bowel Neuroendocrine Tumors
by Vincent Larouche, Amit Akirov, Sameerah Alshehri and Shereen Ezzat
Cancers 2019, 11(9), 1395; https://doi.org/10.3390/cancers11091395 - 18 Sep 2019
Cited by 22 | Viewed by 5389
Abstract
Several important landmark trials have reshaped the landscape of non-surgical management of small bowel neuroendocrine tumors over the last few years, with the confirmation of the antitumor effect of somatostatin analogue therapy in PROMID and CLARINET trials as well as the advent of [...] Read more.
Several important landmark trials have reshaped the landscape of non-surgical management of small bowel neuroendocrine tumors over the last few years, with the confirmation of the antitumor effect of somatostatin analogue therapy in PROMID and CLARINET trials as well as the advent of therapies with significant potential such as mammalian target of rapamycin inhibitor (mTor) everolimus (RADIANT trials) and peptide receptor radionuclide therapy (PRRT) with 177-Lutetium (NETTER-1 trial). This narrative summarizes the recommended management strategies of small bowel neuroendocrine tumors. We review the main evidence behind each recommendation as well as compare and contrast four major guidelines, namely the 2016 Canadian Consensus guidelines, the 2017 North American Neuroendocrine Tumor Society guidelines, the 2018 National Comprehensive Cancer Network guidelines, and the 2016 European Neuroendocrine Tumor Society guidelines. Different clinical situations will be addressed, from loco-regional therapy to metastatic unresectable disease. Carcinoid syndrome, which is mostly managed by somatostatin analogue therapy and the serotonin antagonist telotristat etiprate for refractory diarrhea, as well as neuroendocrine carcinoma will be reviewed. However, several questions remain unanswered, such as the optimal management of neuroendocrine carcinomas or the effect of combining and sequencing of the aforementioned modalities where more randomized controlled trials are needed. Full article
(This article belongs to the Special Issue Management of Neuroendocrine Neoplasms)
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13 pages, 536 KiB  
Review
Treatment Options for Pancreatic Neuroendocrine Tumors
by Amit Akirov, Vincent Larouche, Sameerah Alshehri, Sylvia L. Asa and Shereen Ezzat
Cancers 2019, 11(6), 828; https://doi.org/10.3390/cancers11060828 - 14 Jun 2019
Cited by 52 | Viewed by 12236
Abstract
The management of pancreatic neuroendocrine tumors (PanNETs) involves classification into non-functional or functional PanNET, and as localized or metastatic PanNET. In addition, while most PanNETs are sporadic, these endocrine neoplasms can also be manifestations of genetic syndromes. All these factors may assist in [...] Read more.
The management of pancreatic neuroendocrine tumors (PanNETs) involves classification into non-functional or functional PanNET, and as localized or metastatic PanNET. In addition, while most PanNETs are sporadic, these endocrine neoplasms can also be manifestations of genetic syndromes. All these factors may assist in forming a risk stratification system permitting a tailored management approach. Most PanNETs are classified as non-functional because they are not associated with clinical sequelae of hormone excess. They are characterized by non-specific symptoms, such as abdominal pain or weight loss, resulting from mass effect related to the pancreatic tumor or secondary to distant metastases. Accurate staging of the disease is essential for determining the appropriate approach to therapy. As cure is only potentially possible with surgical resection of the tumor, it is recommended to remove all localized and limited metastatic disease. However, many patients present with metastatic and/or advanced local disease. In such instances, the goal of therapy is to control tumor growth and/or decrease tumor burden, lengthen survival, and palliate local symptoms and those of hormone excess. This typically requires a multimodal approach, including surgery, liver-directed treatment, and systemic medical therapy. Full article
(This article belongs to the Special Issue Management of Neuroendocrine Neoplasms)
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