Dana Kanaan, Khalil Ghandour, Maha Barbar, Iyad Sultan, Hadeel Halalsheh
Pediatric appendiceal NETs carry excellent prognoses following conservative surgery. Pancreatic NETs represent a high-risk subgroup with aggressive behavior. As the first documented GEP-NET from Jordan and the Arab world, these findings align with international patterns and support site-specific, risk-adapted management while highlighting the need for multicenter collaboration across the region.
BACKGROUND: Pediatric gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are exceedingly rare. Data from Jordanian and Arab world populations are absent from international registries, with only limited data available from Türkiye, leaving management strategies largely extrapolated from adult and Western literature. This study documents the clinical features, management, and outcomes at a tertiary oncology center in Jordan.
METHODS: We conducted a retrospective, single-center cohort study of patients 18 years or younger diagnosed with GEP-NETs at the King Hussein Cancer Center (January 2006 to May 2025). We analyzed demographics, presentation, tumor characteristics, treatment, and survival.
RESULTS: Ten patients were identified (8 male, median age 13 y). Primary sites were the appendix (8/10) and pancreatic head (2/10). Nine patients presented with abdominal pain; one with a pancreatic primary presented with obstructive jaundice. Histologically, 9 were low- or intermediate-grade (grade 1 to 2) and 9 were localized. Nine patients underwent surgery (8 appendectomy±hemicolectomy; 1 Whipple procedure). One patient with metastatic pancreatic NET was managed nonsurgically. Two patients with advanced/metastatic pancreatic NETs received systemic therapy. Appendiceal tumors were universally indolent with excellent outcomes postsurgery, whereas pancreatic tumors exhibited aggressive clinical behavior, including nodal involvement and distant metastasis. After a median follow-up of 46.6 months, 1 patient died of metastatic disease and 1 developed recurrence; both had pancreatic primaries.
CONCLUSIONS: Pediatric appendiceal NETs carry excellent prognoses following conservative surgery. Pancreatic NETs represent a high-risk subgroup with aggressive behavior. As the first documented GEP-NET from Jordan and the Arab world, these findings align with international patterns and support site-specific, risk-adapted management while highlighting the need for multicenter collaboration across the region.