Pancreatoblastoma in Children: The Expert/Partner Diagnostic and Therapeutic Recommendations

Background and Aims: Pancreatoblastoma (PBL) is a rare malignant epithelial pancreatic neoplasm affecting typically young children. Complete surgical resection is the mainstay of treatment but optimal therapeutic protocol has to be established. Methods: We present international consensus recommendations for the management of pediatric PBL, established by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within EUfunded PARTNER (Paediatric Rare Tumors Network-European Registry) project. The Consensus Conference Standard Operating Procedure methodology was adopted to grade the levels of evidence and strength of recommendations. Results: Pancreatoblastoma can involve any part of pancreas and is usually diagnosed in advanced stages. Serum alpha-fetoprotein is often elevated reflecting tumor burden [Level IV; Grade A]. Histopathological examination is mandatory for diagnosis. Preoperative radiologic work-up should include abdominal MRI and chest CT [IV; A]. Complete surgical resection with negative margins (R0) is the cornerstone of treatment [IV; A] and should be performed in experienced centers. We recommend 2 courses of PLADO (cisplatin, doxorubicine) after R0 surgery and 4 PLADO in case of microscopic residues (R1) or resected involved lymph nodes. In unresectable and metastatic PBL, neo-adjuvant PLADO (2-6 courses) is advised [IV; B]. Feasibility of complete resection should be evaluated every 2 courses. In case of inadequate response, ICE (ifosfamide, carboplatin, etoposide) [IV; C] or VAC (vincristine, dactinomycine, cyclophosphamide) regimens are alternatives [V; C]. The impact of radiotherapy in incompletely resected PBL remains unproven, and potential side effects in young children have to be considered. Long-term monitoring of pancreatic, auditory, cardiac and renal functions is mandatory [IV; B]. Conclusions: The prognosis is usually favorable in completely resected pediatric PBL. Unresectable and/or metastatic PBL may become amenable to complete delayed surgery after neo-adjuvant PLADO chemotherapy. Discussion in multidisciplinary teams with oncologists and surgeons and enrolment of patients in the national or European database are mandatory. EU Funding: PARTNER GA n.777336 - 3rdHP (2014-2020)