Pancreaticoduodenectomy, a procedure rarely performed in children, can lead to significant morbidity. Rhabdomyosarcoma is the most frequent soft tissue sarcoma in pediatrics. Treatment consists of chemotherapy, while local control can be achieved through either surgery, radiotherapy or both. In this brief report, we describe the case of a 15-year-old adolescent who underwent a pancreaticoduodenectomy for a presumed solid pseudopapillary tumor of the head of the pancreas, ultimately diagnosed as a fusion-positive rhabdomyosarcoma. We review the ensuing severe side effects of the treatments, and discuss the role of biopsies for pancreatic tumors in pediatrics.
