Youngest known case of a pancreatic perivascular epithelioid cell tumor

Perivascular epithelioid cell tumors (PEComas) are a rare type of mesenchymal tumor that can arise in any part of the body. As of 2024, 37 cases of pancreatic PEComas had been reported in the literature with patients ranging in age from 17 to 74 years old. This is the youngest reported case of a pancreatic PEComa in an 8-year-old female presenting with abdominal pain and test findings of biliary obstruction. Magnetic resonance cholangiopancreatography identified the location, size, and obstructive effects of the mass. These findings prompted the performance of an endoscopic retrograde cholangiopancreatography and endoscopic ultrasound with fine-needle aspiration to obtain a tissue sample that revealed the diagnosis of PEComa based on the morphological features and immunohistochemistry. Our patient's tumor was benign, successfully resected, and to date, the child has not had tumor recurrence based upon postsurgical serial imaging, which is consistent with most cases of pancreatic PEComas.