Neuroendocrine Tumors of the Pancreas: Diagnosis and Management: Neuroendocrine Tumors of the Pancreas

Leva Gorji; Asfar S. Azmi; Najeeb Al Hallak; Eliza W. Beal

Authors

Leva Gorji Kettering Health Dayton, Dayton, Ohio, United States
Asfar S. Azmi Barbara Ann Karmanos Cancer Institute, Wayne State University School of Medicine, Detroit, MI, United States
Najeeb Al Hallak Barbara Ann Karmanos Cancer Institute, Wayne State University School of Medicine, Detroit, MI, United States
Eliza W. Beal Barbara Ann Karmanos Cancer Institute, Wayne State University School of Medicine, Detroit, MI, United States

Keywords:

Pancreatic neuroendocrine tumor (pNET), Diagnosis, Classification, Management, Imaging, Surgical Intervention

Abstract

Pancreatic neuroendocrine tumors (pNETs) are rare, heterogeneous malignancies with varied presentation and management. PNETs are categorized into syndromic and non-syndromic based on clinical manifestations secondary to the secretion of bioactive peptides. Approximately 10% of pNETs are associated with an inherited syndrome with an established genetic predisposition to tumor development. Generic non-invasive biomarker testing can be utilized for monitoring disease progression and treatment response, but these are non-specific and may be falsely elevated due to various factors. Non-invasive imaging modalities are effective methods of localization, but angiography, endoscopic ultrasound, and intraoperative palpation with ultrasound are beneficial adjuncts for localization. Overall survival and prognosis vary considerably based on tumor pathology, differentiation, and proliferation index, but many malignant tumors are clinically silent and are recognized after locoregional or metastatic spread. Surgical resection with negative margins can be curative for tumors identified at an early stage. For unresectable or metastatic disease, locoregional therapy and systemic treatment are options. In this review, we discuss the clinical presentation, diagnosis, and potential management options of pNETs.

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Neuroendocrine Tumors of the Pancreas: Diagnosis and Management