Adult pancreatoblastoma: A rare malignant tumor of the pancreas

Pancreatoblastoma (PB) is a rare malignant tumor in adults. It has an overall incidence of 0.004 per 100,000 inhabitants. Its diagnosis with fine-needle aspiration (FNA) is difficult due to multiple differentiation lines present on PB that overlap with other tumors. A 76-year-old male patient presented with jaundice, weight loss of 10 kg in 6 months, and appetite loss. Abdominal computed tomography scan showed a tumor in the pancreas head. Transendoscopic ultrasound with FNA biopsy revealed a malignant epithelial neoplasia compatible with PB with immunohistochemistry CK19 (+), P63(+), synaptophysin (–), and Ki67 50%. The patient underwent surgical resection and adjuvant chemotherapy. Currently, the patient is in follow-up with the latest imaging showing no evidence of disease at 32 months after his initial diagnosis. PB is an uncommon malignant neoplasia with an aggressive behavior. Its diagnostic and therapeutic protocols are unclear. Its preoperative diagnosis may be difficult since its imaging features and serological markers are non-specific. However, FNA may be useful in some situations. Surgical resection is currently the recommended treatment that is associated with the best long-term survival.