J Pediatr Hematol Oncol. 2021 Nov 01;43(8):288-293. doi: 10.1097/MPH.0000000000002033.

Treatment Strategies for Children With Relapsed Pancreatoblastoma: A Literature Review.

Journal of pediatric hematology/oncology

Giulia Reggiani, Maria C Affinita, Patrizia Dall'Igna, Calogero Virgone, Silvia Sorbara, Gianni Bisogno

PMID: 33323880 DOI: 10.1097/MPH.0000000000002033

Abstract

Pancreatoblastoma (PB) is a tumor typically seen in childhood. Despite its rarity, there are some internationally agreed recommendations for its first-line treatment, but very little is known about the management of relapse. We reviewed the literature on the treatment and outcome of children with progressing/recurrent PB, and the role of high-dose chemotherapy (HD-CT) or liver transplantation in difficult cases. A first analysis concerned 15 patients: liver metastases were the most frequent cause of first-line treatment failure. Eight patients underwent surgery, only 3 were irradiated. Various second-line chemotherapy regimens were adopted, with evidence of response in 8 children. The most often-used combinations included etoposide, cyclophosphamide/ifosfamide, and cisplatin/carboplatin. Overall, 7 patients are alive with a median follow-up of 24 months (range, 3 to 88 mo). In a separate analysis, considering patients in first-line or second-line treatment, we found 5 of 6 patients alive after HD-CT and 3 of 3 after liver transplantation. Our review shows that the outcome for patients with recurrent PB is not always dismal, especially when surgery is possible. Different chemotherapy combinations can be used, and HD-CT or liver transplantation may be considered in selected cases.

Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.

Conflict of interest statement

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Publication Types

• Journal Article
• Research Support, Non-U.S. Gov't