Pancreatoblastoma: an Atypical Presentation and a Literature Review

Excerpt

Pancreatoblastoma is a rare, malignant epithelial neoplasm of the pancreas. It primarily occurs in the pediatric population and is extremely uncommon in adults. Annual incidence is around 0.004 cases per 100,000 persons. However, it can account for up to 25% of pancreatic malignancies in children less then 10 years of age [1]. To date, 40 case reports of pancreatoblastomas have been identified since first described in 1986 in patients between the ages of 18 to 78 years [2, 3]. Presenting symptoms are nonspecific, and abdominal pain is the most common initial complaint (44%). A palpable mass, weight loss, jaundice, diarrhea, melena, splenomegaly, abdominal distention, and symptoms from mass effect on adjacent organs may also be present [3‐5]. Although slow growing, the long-term prognosis of a pancreatoblastoma is poor. Complete surgical resection is the initial preferred treatment, and the Italian Tumori Rari in Eta’ Pediatrica project has developed chemotherapy guidelines consisting of cisplatin followed by doxorubicin [6]. Here, we describe an atypical presentation and a literature review of metastatic pancreatoblastoma arising in the pancreatic tail of a 34-year-old male, associated with an elevation of cancer antigen 19-9 (Ca 19-9). …