A Rare Case of Primary Well-Differentiated Neuroendocrine Tumor (NET) Grade 1 of the Pancreas: Diagnostic and Therapeutic Insights

• DOI: https://doi.org/10.22533/at.ed.1595302518096

• Palavras-chave: Relato de caso, glucagonoma, tumores neuroendócrinos, eritema necrolítico migratório (ENM), hepatectomia

• Keywords: Case report, glucagonoma, neuroendocrine tumors, necrolytic migratory erythema (NME), hepatectomy

• Abstract: Abstract: Neuroendocrine Tumors (NETs), are a group of rare neoplasms that arise from specialized cells in the neuroendocrine system, affecting approximately 6 in 100,000 people worldwide. Their most frequent locations are the gastrointestinal tract (GI), pancreas and lungs1. Those originating from the pancreas are known as Glucagonomas, which arise from the alpha cells of the pancreas and are in their majority malignant 2.  Goal: To report an atypical case of a patient with a stage 1 well-differentiated neuroendocrine tumor (NET) in the pancreas. Case report: We report the case of a 49-year-old female diagnosed with a well-differentiated neuroendocrine tumor (NET) Grade 1 (Ki-67 < 3%) of the pancreas. The patient presented with clinical features consistent with glucagonoma, erythema necrolyticum migrans, and a history of bariatric surgery. She underwent multimodal imaging, including PET-CT with 18F-NOTA-Octreotide and PET-CT with Gallium-68 DOTATATE. The patient received Lutetium-177 therapy with four cycles between 28/11/2023 and 09/07/2024, followed by surgical resection of the tumors and subsequent follow-up imaging. This report highlights the importance of precise imaging and histopathological evaluation for accurate diagnosis and effective management.  Discussion / Final Considerations: This case highlights the diagnostic complexity of well-differentiated NETs, where clinical symptoms overlap with metabolic syndromes. This case underscores the importance of multidisciplinary management for optimal outcomes.