Pancreatoblastoma in an Adult Patient with Late Recurrence

Abstract

Pancreatoblastoma (PB) is among the most frequent pancreatic tumor in childhood, but exceedingly rare in adults. The prognosis of PB in adults is very poor with a mean survival time of 10 months. The case describes a 36-year-old woman with a painful palpable tumor in the upper part of the abdomen. The complete resection of the tumor confirmed a 14 cm well-circumscribed tumor with heterogeneous morphological appearance with three main components – neuroendocrine, acinar and squamous. The morphologic and immunohistochemical features proved to be consistent with PB. The patient did not receive any adjuvant chemotherapy. Seventy-two months later a bulky relapsing abdominal tumor was discovered. The histology revealed undifferentiated tumor with solid sheets of medium large atypical cells, areas of tumor necrosis and high mitotic index. Despite the different morphology in the relapse, neuroendocrine differentiation was documented with immunohistochemistry. The case presents a rare malignant tumor of the exocrine pancreas in adult patient. The discussion focuses on the characteristic macroscopic and histological characteristics, immunohistochemical profile and molecular genetics, considering a list of common differential diagnostic entities. This study underlines the importance of recognizing the pancreatoblastoma as a malignant tumor in non-paediatric group in which surgical resection is the best choice of treatment, associated with long-term survival.