Abstract

Pancreatoblastoma (PB) is a rare neoplasm (1) in adults with a total of only 39 cases that have been reported in the literature. PB is an aggressive and malignant tumor, exhibiting high rates of local invasion, recurrence and distant metastatic potential. (3) Alpha-fetoprotein (AFP) is the tumor marker used most often in PB. (2) Symptoms are usually vague and radiological features are non-specific. (3) This is a case of an 18-year-old female who was diagnosed with a rare pathology of PB, after 6 months of follow-up, is still healthy and well. In Vicente Sotto Memorial Medical Center, there has been only 2 cases of Pancreatoblastoma, hence, the rarity of this disease. Based on the literature, there has been no published case report in the Philippines with regards to this disease. This paper reviews the very rare cases of adult pancreatoblastoma reported in the literature with a discussion of the clinicopathological features, treatment modalities and management outcomes. This case has several distinguishing features from previously reported cases. Firstly, pancreatoblastoma is more common in children as compared to adults. In addition, in adults presenting with pancreatoblastoma usual symptoms are weight loss, nonspecific abdominal pain. However, in this case, the patient was noted to have abdominal mass, which is usually seen in pediatric age group from 1 to 8 years old. Lastly, in adults, usually 35% present with metastasis to the lymph or liver. However, in this patient during the frozen section of the lymph node, the result was negative for metastasis. In addition, in the final histopathologic result, all 18 lymph nodes were negative for tumor metastasis. Pancreatoblastoma can have an atypical clinical picture with large primary tumor and nonspecific symptoms in adult patient which may present a diagnostic challenge. Given its rarity, a high index of suspicion is required to correctly diagnose this condition. Due to its rarity, no guidelines currently exist on the management protocols for PB. (3) Surgery is considered to be the chief treatment strategy, while the role of chemotherapy and radiotherapy remains unclear. In this patient, instead of the standard pancreaticoduodenectomy, the pylorus-preserving pancreaticoduodenectomy was performed which resulted to less need for blood transfusion, and shorter hospital stay. The current literature is limited with regards to the management, follow-up and screening for pancreatoblastoma.