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ABSTRACT
Background
BACKGROUND:
Solid Pseudopapillary Epithelial Neoplasms (SPENs) of the pancreas are rare, low grade malignant tumors predominantly affecting young females. They are indolent in nature and often detected incidentally during work up for other medical reasons. Symptomatic cases may present with abdominal pain, a palpable mass, or obstructive jaundice from extrinsic compression of the biliary tree.
AIM:
This case report intents to highlight the clinical presentation, diagnostic workup and the surgical management of a rare pediatric case of SPEN, thus emphasizing the importance of high index of suspicion, early diagnosis and complete surgical resection to achieve favorable outcomes.
Solid Pseudopapillary Epithelial Neoplasms (SPENs) of the pancreas are rare, low grade malignant tumors predominantly affecting young females. They are indolent in nature and often detected incidentally during work up for other medical reasons. Symptomatic cases may present with abdominal pain, a palpable mass, or obstructive jaundice from extrinsic compression of the biliary tree.
AIM:
This case report intents to highlight the clinical presentation, diagnostic workup and the surgical management of a rare pediatric case of SPEN, thus emphasizing the importance of high index of suspicion, early diagnosis and complete surgical resection to achieve favorable outcomes.
Method
We describe a rare case of an 8 year old female, who presented to our institution with a 4 months history of progressive jaundice, dark urine, pale stools and abdominal pain. Laboratory investigations were in keeping with features of obstructive jaundice which was complicated by mild cholangitis. An abdominal ultrasound showed a dilated pancreatic duct, head of pancreas mass and dilated biliary tree. A contrast enhanced CT scan of the abdomen showed a solid mass in the head of the pancreas, with dilatation of the pancreatic duct and biliary tree. She was subsequently optimized for theatre and a Whipple’s procedure was performed. Histopathological analysis confirmed SPEN, with no malignant features. Immunohistochemical staining showed diffuse positivity for CD10 and beta-catenin
Result
The post operative course was unremarkable, with subsequent normal liver functions and complete resolution of jaundice. To date, she remains asymptomatic, almost a year post surgery. Histopathological analysis confirmed an encapsulated neoplasm with solid and pseudopapillary areas, minimal mitotic activity and no evidence of lymphovascular or perineural invasion
Conclusion
SPENs, although rare, should be considered in pediatric patients presenting with head of pancreas mass. Cross sectional imaging is essential in making the diagnosis. Complete surgical resection remains the gold standard treatment . Given their excellent prognosis, close post operative follow up is highly recommended to monitor for recurrence