Metastatic adult pancreatoblastoma: Multimodal treatment and molecular characterization of a very rare disease.

Pancreatology : Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
Anne Katrin BergerChristoph Heining

Abstract

Pancreatoblastoma is a rare malignancy that occurs predominantly in children. Less than 50 adult cases, including 17 patients with metastatic disease, have been published to date. Recent outcome data from children with advanced-stage disease suggest an intensive multimodal treatment approach; however, little is known about the most beneficial therapy in adults. Molecular characterization of pancreatoblastoma is limited to a small number of pediatric cases and revealed few recurrent genetic events without immediate clinical relevance. Patients were treated between 2013 and 2018 at a high-volume German university cancer center. Molecular analyses included whole genome, exome, transcriptome, and fusion gene panel sequencing. Molecularly guided treatment recommendations were discussed within a dedicated molecular tumor board (MTB) embedded in a precision oncology program (NCT MASTER). We identified four adult patients with metastatic pancreatoblastoma. In three patients, local approaches were combined with systemic treatment. Oxaliplatin-containing protocols showed an acceptable tumor control as well as an adequate toxicity profile. Overall survival was 15, 17, 18 and 24 months, respectively. Three tumors harbored genetic alteratio...Continue Reading

Citations

Jun 27, 2021·Pediatric Blood & Cancer·Ewa BienYves Reguerre
Jul 31, 2021·World Journal of Gastroenterology : WJG·Ayo O Omiyale

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