Downstaging and R0 resection of initially unresectable metastatic well-differentiated grade-3 pancreatic neuroendocrine tumor: a case report.

Abstract

Introduction: High-grade pancreatic neuroendocrine tumors are rare, and most of them are well differentiated. Management of this type of tumor is not yet well established.

Methods: A 28-year-old woman was referred to our hospital for a well-differentiated grade-3 pancreatic neuroendocrine tumor with multiple liver metastases. The patient received neoadjuvant therapy consisting of combination of Capecitabine/Temozolomide chemotherapy and somatostatin analogue.

Results: An excellent regression was observed on both pancreatic and hepatic lesions, and therefore, an aggressive surgical management could be performed with a two-step scheme. Adjuvant hormono-chemotherapy was administered between the two surgeries. The patient achieved six years of disease-free survival following the last therapy.

Discussion and conclusion: We highlight the difference between well and poorly differentiated high-grade neuroendocrine neoplasia and report that aggressive surgery is a valid option even in metastatic presentation at diagnosis.