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Pancreatic Neuroendocrine Neoplasms: 2020 Update on Pathologic and Imaging Findings and Classification
Lokesh Khanna1, Srinivasa R Prasad1, Abhijit Sunnapwar1
1From the Departments of Radiology (L.K., A.S., U.S., V.S.K.) and Pathology (V.S.T.), University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Dr, San Antonio, TX 78229; Department of Radiology, University of Texas M. D. Anderson Cancer Center, Houston, Tex (S.R.P.); Department of Molecular Biosciences, University of Texas at Austin, Austin, Tex (S.K.); Department of Radiology, University of Pittsburgh Medical Center, Pittsburgh, Pa (A.D.); and Department of Radiology, University of Texas Health Science Center at Houston, Houston, Tex (A.N.).
Pancreatic neuroendocrine neoplasms (panNENs) are diverse tumors. Updated classifications and imaging techniques improve diagnosis and treatment for these neuroendocrine tumors.
Area of Science:
- Oncology
- Radiology
- Pathology
Background:
- Pancreatic neuroendocrine neoplasms (panNENs) are heterogeneous tumors with varying clinical, histomorphologic, and prognostic features.
- Genetic alterations and distinct biologic behaviors characterize panNENs, with up to 10% linked to hereditary cancer syndromes.
- Classification distinguishes functioning tumors (early symptoms) from nonfunctioning tumors (late presentation due to mass effect).
Purpose of the Study:
- To review recent advancements in the pathogenesis, classification, and staging of panNENs.
- To highlight the importance of understanding updated imaging findings for optimal patient management.
- To differentiate between high-grade pancreatic neuroendocrine tumors (panNETs) and pancreatic neuroendocrine carcinomas (panNECs).
Main Methods:
- Review of updated World Health Organization (WHO) classification systems (2010 and 2017).
- Incorporation of the eighth edition of the American Joint Committee on Cancer staging system.
- Analysis of imaging modalities including multidetector CT, MRI, endoscopic US, somatostatin receptor scintigraphy, and 18F-FDG PET/CT.
Main Results:
- PanNENs are histopathologically classified as well-differentiated pancreatic neuroendocrine tumors (panNETs) or poorly differentiated pancreatic neuroendocrine carcinomas (panNECs).
- Recent molecular biology advances reveal heterogeneity in panNECs, leading to the introduction of a new subtype: well-differentiated, high-grade panNET.
- High-grade panNETs and panNECs are distinct entities with differing pathogenesis, clinical presentation, imaging, treatment, and prognosis.
Conclusions:
- Updated WHO and AJCC staging systems reflect substantial changes in panNEN classification and staging.
- Advanced imaging techniques are crucial for localizing primary tumors, assessing spread, and detecting metastases.
- Knowledge of current panNEN updates and imaging findings is essential for tailoring optimal patient treatment strategies.
