• Christian Müller, Michael C Kreissl, Silke Klose, Andreas Krause, Verena Keitel, and Marino Venerito.
• Otto-von-Guericke University Hospital, Department of Gastroenterology, Hepatology and Infectious Diseases, Leipziger Str. 44, Magdeburg, Germany.
• Medicine (Baltimore). 2022 Jan 28; 101 (4): e28610e28610.

RationalePancreatic neuroendocrine tumors (pNETs) are rare entities representing 1% to 3% of all malignant pancreatic neoplasms. Current guidelines recommend a combination of streptozocin (STZ) and 5-fluorouracil (5-FU) for patients with metastatic well-differentiated pNETs requiring systemic therapy. The highest median progression-free survival rate reported in previous studies for this combination was 23 months (95% confidence interval 14.5-31.5). However, it remains unclear for how long this regimen can be safely administered.Patient ConcernsWe report about 3 therapy-naïve patients with metastatic G2 (Ki67 10%-15%) pNETs treated with STZ/5-FU, that achieved sustained disease control for longer than 36 months.DiagnosisMetastatic, well-differentiated G2 pNETs.InterventionsSystemic chemotherapy with STZ/5-FU was administered until the disease progressed. In 1 case showing a mixed response, selected metastases of increasing size were additionally treated with surgery and brachytherapy.OutcomesIn our 3 patients with metastatic G2 pNETs, STZ/5-FU induced long-term disease control over 44, 42, and 95 months, respectively. No side effects that led to treatment discontinuation were observed.LessonsIn patients with metastatic G2 pNETs achieving disease control, STZ/5-FU can be safely administered.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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