Pancreatoblastoma in children: EXPeRT/PARTNER diagnostic and therapeutic recommendations

Abstract

Pancreatoblastoma (PBL) is a rare malignant epithelial neoplasm that affects typically young children. Signs related to advanced upper-abdominal tumor accompanied by elevated serum α-fetoprotein levels in a young child suggest PBL, however histopathological confirmation is mandatory. The mainstay of the treatment is a complete surgical resection.

Rare Tumors in Children and Adolescents – the STEP Working Groupʼs Evolution to a Prospective Registry

“…Similar cooperative analyses have also been established for colorectal carcinoma and malignant mela-noma 16 23 . These works have been the basis for establishing closer international collaboration with the funding of the European Commission as part of the ExPO-r-Net (European Expert Pediatric Oncology Reference Network for Diagnostics and Treatment) and partner projects 1 3 5 16 17 26 34 37 . By joining international groups into one registry, it becomes possible to gain further knowledge, especially for very rare tumors.…”

“…Second, the establishment of a consultation network has provided a significant benefit to the treating physicians, because they could build on experience gained by other pediatric oncologists confronted with very rare tumors. Third, the first national and international analyses of children and adolescents with VRTs have provided the basis for a more standardized diagnostic assessment and therapy of affected children and adolescents 1 2 3 4 5 7 19 . This development is nicely illustrated by the work on rare gonadal sex-cord stromal tumors 33 .…”

“…Looking at the different types of cancer diagnoses, we see different patterns in this analysis. First, there is a group of patients with specifically pediatric tumors that almost exclusively occur in children, such as pancreatoblastoma 3 . Second, there is a large group of adult cancer diagnoses, such as melanoma, colorectal carcinoma or salivary gland carcinoma, which we see predominantly in the group of adolescent patients 16 34 37 .…”

The French FRACTURE database: A way to improve knowledge on management of children with very rare tumors

“…These rare malignant tumors are less known to pediatricians and even pediatric oncologists. However, in recent years, the European EXPeRT group (European Cooperative Study Group for Pediatric Rare Tumors) published some specific recommendations to help clinicians in treating patients with these rare entities 5–12 . The PARTNER (PAediatric Rare Tumours Network ‐ European Registry) project aims to extend knowledge of theses rare diseases and to build a single European registry that links the existing national registries dedicated to children and adolescents (0–18 years) with VRTs.…”

“…However, in recent years, the European EXPeRT group (European Cooperative Study Group for Pediatric Rare Tumors) published some specific recommendations to help clinicians in treating patients with these rare entities. [5][6][7][8][9][10][11][12] The PARTNER (PAediatric Rare Tumours Network -European Registry) project aims to extend knowledge of theses rare diseases and to build a single European registry that links the existing national registries dedicated to children and adolescents (0-18 years) with VRTs. European countries, where no VRT registry exists, should register their patients directly in this new European registry.…”