ICD-11 code : XH27L5;
Preferred Label : Pancreatoblastoma;
ICD-11 definition : Pancreatoblastoma is a very rare malignant epithelial neoplasm of the pancreas showing
multiple lines of differentiation including acinar differentiation and squamoid nests.
Endocrine and ductal differentiation, and a distinct mesenchymal component, can also
be seen. Most pancreatoblastomas occur in children. Pancreatoblastoma is the most
common malignant pancreatic neoplasm of childhood. It has been reported in newborns
with Beckwith-Wiedemann syndrome. Grossly, pancreatoblastomas are usually large and
well-demarcated, and are often palpable in children. They are fully malignant neoplasms.;
Origin ID : 733863058;
Automatic exact mappings (from CISMeF team)
Pancreatoblastoma is a very rare malignant epithelial neoplasm of the pancreas showing
multiple lines of differentiation including acinar differentiation and squamoid nests.
Endocrine and ductal differentiation, and a distinct mesenchymal component, can also
be seen. Most pancreatoblastomas occur in children. Pancreatoblastoma is the most
common malignant pancreatic neoplasm of childhood. It has been reported in newborns
with Beckwith-Wiedemann syndrome. Grossly, pancreatoblastomas are usually large and
well-demarcated, and are often palpable in children. They are fully malignant neoplasms.
