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Extra‐appendicular neuroendocrine tumors: A report from the TREP project (2000‐2020)
Author(s) -
Virgone Calogero,
Ferrari Andrea,
Chiaravalli Stefano,
De Pasquale Maria Debora,
Inserra Alessandro,
D'Angelo Paolo,
Ogunleye Martina Funmilayo,
Crocoli Alessandro,
Vallero Stefano,
Cesaro Simone,
Alaggio Rita,
Bisogno Gianni,
Dall'Igna Patrizia
Publication year - 2021
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.28880
Subject(s) - medicine , neuroendocrine tumors , disease , overall survival , pancreas , oncology
Abstract Background Extra‐appendicular neuroendocrine tumors (NETs) are very rare tumors. While diagnostic and therapeutic guidelines are well established for adults, data on children and adolescents are lacking. Patients and Methods Patients with a diagnosis of extra‐appendicular NET registered on the Tumori Rari in Età Pediatrica ‐ Rare Tumors in Pediatric Age (TREP) from 2000 to 2020 were analyzed. Clinical characteristics including patients’ presentation, tumor features, treatment, and outcome were reviewed. Results Twenty‐seven patients with extra‐appendicular NET registered on TREP with a median age of 173 months. The primary site was the pancreas (12) or bronchi (10) in the majority of cases. Other primary sites included the thymus, Meckel's diverticulum, and liver. Thirteen (48%) of tumors extended beyond the organ of origin: four invaded neighboring organs and/or regional nodes and nine involved distant metastases. The 3‐year event‐free survival (EFS) for those with localized disease was superior to those with metastatic disease (66.6% 95% CI 5‐95% vs 33% 95% CI 5‐68%, respectively; P = .005). A complete resection was feasible in 17 patients. The 3‐year EFS in these patients was superior to those with no or incomplete resection (R0 vs R1/R2, respectively; P = .007). Overall, 16 children had no evidence of disease at follow‐up, and one is alive with disease; five died, and five were lost to follow‐up. Conclusions Data from our experience demonstrated a wide heterogeneity of presentation and outcome of these tumors. Localized disease and complete surgical resection were the main prognostic factors of good outcome. Other therapies may have a role in prolonging survival in metastatic disease.