Since only 157 cases of this type of tumor were reported from 1959 to 2005, they are considered to be a rare entity. They account for 0.2% to 2.7% of all pancreatic exocrine tumors 6.
Desde 1959 al 2005 solamente se han reportado 157 casos de este tipo de tumor, por lo que se considera una entidad poco frecuente, constituyendo el 0,2-2,7% de los tumores exocrinos del páncreas 6.