Pancreatoblastoma in Adults: A Review of the Literature

Abstract

Background: Pancreatoblastoma is a very uncommon neoplasm in adults and its management represents a great challenge with regards to different treatment options. Given the rarity of the disease, the aim of this study was to review our personal experience with adult pancreatoblastoma as well as the cases reported in the literature in order to support clinicians observing this entity. Methods: Adult patients with histologically proven pancreatoblastoma were identified from our prospective database of pancreatic resections. After a search on the Medline database, a review of all cases was performed as well, focusing on clinical, radiological and hystopathological features and treatment options. Results: At our Institution, 2 adult males, 26 and 69 years old, underwent successful pancreatic resection for pancreatoblastoma. The diagnosis of pancreatoblastoma mainly depends on the pathological findings characterized by squamoid corpuscles at histopathology. Only 21 cases of adult pancreatoblastoma have been identified in the literature. In general, despite aggressive treatment, pancreatoblastoma in adults is associated with poorer outcome than in children, with a median survival time of 18.5 months. Both our patients are disease free after 15 months (case 2) and 51 months (case 1). The latter represents the most successful result in long-term disease-free survival. Conclusion: Pancreatoblastoma is a rare neoplasm in adults. The differential diagnosis includes nonfunctional pancreatic endocrine tumor, acinar cell carcinoma, solid pseudopapillary tumor and adenocarcinoma. Surgical resection is the only treatment associated with long-term survival. Chemotherapy may play a role as palliative treatment in advanced disease.