Elaine Jayne Buckley1, K Molik2, J Mellinger1. 1SIU-SOM, 2HSHS Pediatric Surgery
INTRODUCTION: Solid pseudopapillary tumors are rare neoplasms accounting for 2-3% of pancreatic malignancies with a low risk of recurrence and metastasis. Pancreatic malignancies are less common in pediatric populations, though small case series have identified that pseudopapillary tumors comprise between 20-70% of pediatric pancreatic neoplasms. As these tumors have a low risk of metastasis, the mainstay of treatment has remained surgical excision. Several surgical approaches have been described from extensive resections such as pancreaticoduodenectomy to local enucleation. We present a case of enucleation of a large pseudopapillary tumor from the pancreatic head complicated by pancreatic fistula. A literature review was performed given the rarity of this tumor to review surgical approaches, to compare complications and long-term outcomes, and to identify specific strategies to decrease the risk of pancreatic fistula.
CASE DESCRIPTION: A 13 year-old female presented with 6 months of abdominal pain. Computed tomography identified a right upper quadrant mass felt to be consistent with a lipoma. Follow up CT at 6 months suggested the mass was more likely a Gastrointestinal Stromal Tumor (GIST), and surgical resection was recommended. Enucleation of the mass was chosen in view of a well-circumscribed appearance, clear operative tissue planes, and concern for long-term morbidity of a more extensive resection given the patient’s young age. Pathology demonstrated an 8.5cm pseudopapillary tumor with negative margins. Her post-operative course was complicated by a grade B pancreatic fistula, managed with nutritional support, external drain maintenance, and endoscopic stenting. The patient achieved healing of the pancreatic fistula after four months.
RESULTS: Our literature review demonstrates no difference in recurrence, mortality or morbidity between types of surgery. Pancreatic fistula contributed to the majority of postoperative morbidity in all cases. Recommendations for enucleation include small (2-4 cm) tumors with between 2 to 5mm margin from the main pancreatic duct. Techniques identified to minimized post-operative pancreatic fistula include preoperative imaging of the duct anatomy, preoperative pancreatic stent placement, and intraoperative ultrasound to identify the pancreatic duct. Some literature supports preservation of pancreatic parenchyma, particularly in younger patients, to reduce endocrine and exocrine dysfunction given the low rates of recurrence and metastasis with this rare neoplasm.
CONCLUSION: Our case demonstrates complications of enucleation of a large pseudopapillary tumor with successful multidisciplinary post-operative management. With the risk reduction strategies identified, we suggest that enucleation may be considered for pseudopapillary tumors in younger patients to preserve pancreatic parenchyma and long-term pancreatic function.
Presented at the SAGES 2017 Annual Meeting in Houston, TX.
Abstract ID: 87285
Program Number: P519
Presentation Session: iPoster Session (Non CME)
Presentation Type: Poster
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