The solid-pseudopapillary neoplasm of the pancreas is a rare but enigmatic entity
occurring mainly in young women. Since the first description by V. Frantz in 1959
the terminology of this tumor has continuously changed but it has remained simply
descriptive, because the exact histogenesis is still obscure. Although in majority
of cases the survival is excellent, nevertheless, the expected prognosis is not exactly
predictable. In this review the authors aim to summarize its clinico-pathological
features, the expected biological behavior, the molecular alterations, the immune
phenotype and discuss the putative histogenesis. From diagnostic point of view, the
salient histological characteristic findings are analyzed that would help to differentiate
it from other, look-alike pancreatic tumors, and suggestions are made about the desirable
content of the histological report.
