Association between Solid Pseudopapillary Pancreatic Tumor (Frantz’s Tumor) and Left Portal Hypertension in Pediatrics: Case Report
DOI:
https://doi.org/10.32635/2176-9745.RBC.2023v69n4.4350Keywords:
pancreas, pediatrics, hypertension, portalAbstract
Introduction: The solid pseudopapillary tumor of the pancreas is a rare pathology, accounting for less than 3% of all pancreatic exocrine tumors. This tumor occurs mainly in young women, between 20 and 30 years old, has low malignant potential, causing symptoms only when they are
large. Case report: Female patient, 14 years of age, presented hematemesis and varicose veins in the gastric fundus. After submitting to computed tomography, a hypodense mass of hypovascular behavior was shown in
the pancreatic tail, raising the suspicion of primary pancreatic neoplasia promoting compression of the splenic vein. The patient underwent bodycaudal pancreatectomy associated with splenectomy in February 2021. The biopsy of the hepatic nodule, evidenced on abdominal ultrasound, confirmed metastasis and the patient underwent subsequent hepatic segmentectomy in July 2021. Conclusion: The case reported is extremely rare because it is a Frantz tumor in childhood associated with left portal hypertension due to tumor compression of the splenic vein, which consequently presented upper digestive hemorrhage as an initial clinical manifestation; similar cases are not easily found in the literature in the pediatric age range.
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