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Updated: Sep 18, 2025
Robotic Enucleation of an Intra-Pancreatic Insulinoma in the Pancreatic Head
Published on: January 3, 2020
Pancreatic neuroendocrine tumors: A case-based evidence review
Naveena Rikhraj1, Cornelius J Fernandez2, Vanishri Ganakumar3
1Department of Medicine, Lancashire Teaching Hospitals NHS Trust, Preston PR2 9HT, Lancashire, United Kingdom.
Abstract:
Pancreatic neuroendocrine tumors (pNETs) are rare, presenting significant challenges in timely diagnosis and subsequent treatment. The clinical and pathobiological behavior of these tumors varies significantly, making follow-up and therapeutic approaches challenging for clinicians. Although the majority of these neoplasms are hormonally inactive, some can be associated with endocrine dysfunction. Very rarely, a nonfunctional tumor can later become hormonally active, further complicating prognostication and management. Depending on the character of the disease, clinical picture and prognosis, different treatment modalities are instituted with varying effectivities. We recently came across a unique case of nonfunctioning malignant pNET at an advanced stage, metastatic disease upon diagnosis, managed medically with somatostatin analog therapy (Octreotide) and targeted therapy (Everolimus) with stable disease for 40 months that subsequently turned out to become functional (insulinoma). With the aid of this unique case, we update the current clinical, diagnostic and therapeutic approach to pNETs in this evidence-based review.
Insights
This case study highlights a rare malignant pancreatic neuroendocrine tumor (pNET) that remained nonfunctional for 40 months before becoming functional. It underscores challenges in pNET diagnosis and management.
Area of Science:
- Oncology
- Endocrinology
- Gastroenterology
Background:
- Pancreatic neuroendocrine tumors (pNETs) are rare neoplasms with variable clinical behavior.
- Timely diagnosis and tailored treatment for pNETs remain significant challenges for clinicians.
- While often hormonally inactive, pNETs can present with endocrine dysfunction or rarely transition from nonfunctional to functional states.
Purpose of the Study:
- To present a unique case of a nonfunctioning malignant pNET that later became functional (insulinoma).
- To review and update the current clinical, diagnostic, and therapeutic strategies for pNETs.
- To discuss the implications of tumor behavior changes on patient management and prognostication.
Main Methods:
- Case report of a patient with advanced-stage, metastatic nonfunctioning malignant pNET.
- Medical management with somatostatin analog (Octreotide) and targeted therapy (Everolimus).
- Review of current evidence-based literature on pNET diagnosis and treatment.
Main Results:
- The patient achieved stable disease for 40 months on somatostatin analog and targeted therapy.
- The previously nonfunctioning pNET subsequently became functional, presenting as an insulinoma.
- This case illustrates the complex and evolving nature of pNET behavior.
Conclusions:
- The management of pNETs requires a flexible and adaptive approach due to their unpredictable behavior.
- Early diagnosis and appropriate medical therapies can achieve prolonged stable disease in advanced pNETs.
- Understanding the potential for functional transition in pNETs is crucial for long-term patient care and monitoring.
