Pediatric Solid Pseudopapillary Neoplasm[Spn] of The Pancreas–Case Report and Literature Review - Abstract
Background: Pediatric pancreatic malignancies are extremely rare with an age population-adjusted incidence of 0.018 cases per 100,000. In adolescent girls, they usually present as solid pseudopapillary neoplasms[SPNs], low-grade malignant tumors of the exocrine pancreas. Due to their rarity and nonspecific clinical presentation, SPNs are frequently misdiagnosed. The treatment of choice is a complete surgical resection with 5-year survival rate of 97%.
Case Report: We report a 17-year-old girl with SPN presenting with jaundice and itchy skin. She was initially misdiagnosed with hepatoblastoma, and treated with neoadjuvant chemotherapy. The girl later underwent surgical excision, and is in complete remission seven years after the diagnosis.
Conclusion: Although rare, SPNs need to be included in the differential diagnosis of abdominal masses in children. MRI detected, well-defined, encapsulated, heterogeneous mass, especially in adolescent females, should always be suspicious for SPN. Further prospective multi-institutional studies are required in order to standardize preoperative evaluation and reduce the high misdiagnosis rate.