Treatment options for advanced pancreatic neuroendocrine tumors: what is on the horizon?

2015 ◽  
Vol 2 (1) ◽  
pp. 39-55
Author(s):  
Edward M Wolin
Keyword(s):  
Neuroendocrine Tumors ◽  
Treatment Options ◽  
Pancreatic Neuroendocrine Tumors

scholarly journals Treatment Options for Pancreatic Neuroendocrine Tumors

Cancers ◽  
2019 ◽  
Vol 11 (6) ◽  
pp. 828 ◽  
Author(s):  
Amit Akirov ◽  
Vincent Larouche ◽  
Sameerah Alshehri ◽  
Sylvia L. Asa ◽  
Shereen Ezzat
Keyword(s):  
Neuroendocrine Tumors ◽  
Treatment Options ◽  
Distant Metastases ◽  
Mass Effect ◽  
Tumor Burden ◽  
Management Approach ◽  
Pancreatic Neuroendocrine Tumors ◽  
Genetic Syndromes ◽  
Stratification System ◽  
Specific Symptoms

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.

scholarly journals Pancreatic Neuroendocrine Tumors: Molecular Mechanisms and Therapeutic Targets

Cancers ◽  
2021 ◽  
Vol 13 (20) ◽  
pp. 5117
Author(s):  
Chandra K. Maharjan ◽  
Po Hien Ear ◽  
Catherine G. Tran ◽  
James R. Howe ◽  
Chandrikha Chandrasekharan ◽  
...  
Keyword(s):  
Drug Resistance ◽  
Neuroendocrine Tumors ◽  
Molecular Mechanisms ◽  
Treatment Options ◽  
Somatostatin Receptor ◽  
Therapeutic Targets ◽  
Model Systems ◽  
Pancreatic Neuroendocrine Tumors ◽  
Acquired Drug Resistance ◽  
Molecular Alterations

Pancreatic neuroendocrine tumors (pNETs) are unique, slow-growing malignancies whose molecular pathogenesis is incompletely understood. With rising incidence of pNETs over the last four decades, larger and more comprehensive ‘omic’ analyses of patient tumors have led to a clearer picture of the pNET genomic landscape and transcriptional profiles for both primary and metastatic lesions. In pNET patients with advanced disease, those insights have guided the use of targeted therapies that inhibit activated mTOR and receptor tyrosine kinase (RTK) pathways or stimulate somatostatin receptor signaling. Such treatments have significantly benefited patients, but intrinsic or acquired drug resistance in the tumors remains a major problem that leaves few to no effective treatment options for advanced cases. This demands a better understanding of essential molecular and biological events underlying pNET growth, metastasis, and drug resistance. This review examines the known molecular alterations associated with pNET pathogenesis, identifying which changes may be drivers of the disease and, as such, relevant therapeutic targets. We also highlight areas that warrant further investigation at the biological level and discuss available model systems for pNET research. The paucity of pNET models has hampered research efforts over the years, although recently developed cell line, animal, patient-derived xenograft, and patient-derived organoid models have significantly expanded the available platforms for pNET investigations. Advancements in pNET research and understanding are expected to guide improved patient treatments.

Understanding the Management and Treatment of Well-Differentiated Pancreatic Neuroendocrine Tumors: A Clinician’s Guide to a Complex Illness

2020 ◽  
Vol 16 (11) ◽  
pp. 720-728
Author(s):  
Daneng Li ◽  
Adam Rock ◽  
Jonathan Kessler ◽  
Richard Ballena ◽  
Shadman Hyder ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Treatment Options ◽  
Arterial Embolization ◽  
Standard Of Care ◽  
Neuroendocrine Cells ◽  
Pancreatic Neuroendocrine Tumors ◽  
Current Standard ◽  
Systemic Treatments ◽  
Individualized Approach ◽  
Diagnostic Modalities

Pancreatic neuroendocrine tumors (PanNETs) are rare neoplasms that arise in the neuroendocrine cells of the pancreas. Although their clinical presentations differ depending on cell type, most are indolent, whereas others cause noteworthy hormone-related symptoms. The increasing incidence of PanNETs, attributed to improved diagnostic modalities, demonstrates advances in current standard of care. However, given the heterogeneity of these tumors, treatment decisions can become complex and an individualized approach is often required. Surgical intervention has remained the mainstay for localized tumors, whereas systemic therapies remain viable options for patients with unresectable or metastatic disease. Liver-directed therapies such as radiofrequency ablation and hepatic arterial embolization have also become available adjunct therapies for patients with liver-predominant metastases. Despite the increase in the armamentarium of treatment options for patients with PanNETs, data regarding the ideal sequence of treatment, especially systemic treatments, are currently lacking. Ongoing clinical trials are aimed at addressing this knowledge gap in addition to developing the next generation of novel therapeutics.

Proposal of a two-step dynamic prognostic stratification for stage IV sporadic pancreatic neuroendocrine tumors

2016 ◽  
Author(s):  
Vincenzo Marotta ◽  
Thomas Walter ◽  
Cao Christine Do ◽  
Salvatore Tafuto ◽  
Vincenzo Montesarchio ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Stage Iv ◽  
Pancreatic Neuroendocrine Tumors ◽  
Prognostic Stratification

Pre-operative Diagnosis of Pancreatic Neuroendocrine Tumors with Endoscopic Ultrasonography and Computed Tomography in a Large Series

2016 ◽  
Vol 25 (3) ◽  
pp. 317-321 ◽  
Author(s):  
Raffaele Manta ◽  
Elisabetta Nardi ◽  
Nico Pagano ◽  
Claudio Ricci ◽  
Mariano Sica ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Neuroendocrine Tumors ◽  
Endoscopic Ultrasonography ◽  
Fine Needle Aspiration ◽  
Chromogranin A ◽  
Needle Aspiration ◽  
Large Series ◽  
Tumor Diameter ◽  
Pancreatic Neuroendocrine Tumors ◽  
Fine Needle

Background & Aims: Diagnosis of pancreatic neuroendocrine tumors (p-NETs) is frequently challenging. We describe a large series of patients with p-NETs in whom both pre-operative Computed Tomography (CT) and Endoscopic Ultrasonography (EUS) were performed. Methods: This was a retrospective analysis of prospectively collected sporadic p-NET cases. All patients underwent both standard multidetector CT study and EUS with fine-needle aspiration (FNA). The final histological diagnosis was achieved on a post-surgical specimen. Chromogranin A (CgA) levels were measured. Results: A total of 80 patients (mean age: 58 ± 14.2 years; males: 42) were enrolled. The diameter of functioning was significantly lower than that of non-functioning p-NETs (11.2 ± 8.5 mm vs 19.8 ± 12.2 mm; P = 0.0004). The CgA levels were more frequently elevated in non-functioning than functioning pNET patients (71.4% vs 46.9%; P = 0.049). Overall, the CT study detected the lesion in 51 (63.7%) cases, being negative in 26 (68.4%) patients with a tumor ≤10 mm, and in a further 3 (15%) cases with a tumor diameter ≤20 mm. CT overlooked the pancreatic lesion more frequently in patients with functioning than non-functioning p-NETs (46.5% vs 24.3%; P = 0.002). EUS allowed a more precise pre-operative tumor measurement, with an overall incorrect dimension in only 9 (11.2%) patients. Of note, the EUS-guided FNA suspected the neuroendocrine nature of tumor in all cases. Conclusions: Data of this large case series would suggest that the EUS should be included in the diagnostic work-up in all patients with a suspected p-NET, even when the CT study was negative for a primary lesion in the pancreas.– . Abbrevations: CgA: chromogranin A; EUS: Endoscopic Ultrasonography; FNA: fine-needle aspiration; p-NETs: pancreatic neuroendocrine tumors.

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