Solid pseudopapillary tumor of the pancreas: a single-institution 20-year series of pediatric patients

Abstract Purpose Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm. The objective of this study was to review our institution's experience and provide an update on current management in the pediatric population. Methods Our pathology database identified all patients with SPT...

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Bibliographic Details
Published in:Journal of pediatric surgery 2012-06, Vol.47 (6), p.1217-1222
Main Authors: Speer, Allison L, Barthel, Erik R, Patel, Moneil M, Grikscheit, Tracy C
Format: Article
Language:English
Subjects:
Adolescent
Biomarkers, Tumor - analysis
Child
Cystadenoma, Papillary - chemistry
Cystadenoma, Papillary - diagnosis
Cystadenoma, Papillary - epidemiology
Cystadenoma, Papillary - surgery
Ethnic Groups - statistics & numerical data
Female
Frantz tumor
Humans
Los Angeles - epidemiology
Male
Neoplasm Recurrence, Local - epidemiology
Pancreas - injuries
Pancreatectomy
Pancreatectomy - methods
Pancreatectomy - statistics & numerical data
Pancreatic Neoplasms - chemistry
Pancreatic Neoplasms - diagnosis
Pancreatic Neoplasms - epidemiology
Pancreatic Neoplasms - surgery
Pancreaticoduodenectomy
Pancreaticoduodenectomy - methods
Pancreaticoduodenectomy - statistics & numerical data
Pediatric pancreatic tumor
Pediatrics
Prognosis
Retrospective Studies
Solid pseudopapillary tumor of the pancreas
Surgery
Treatment Outcome
β-catenin
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Summary:Abstract Purpose Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm. The objective of this study was to review our institution's experience and provide an update on current management in the pediatric population. Methods Our pathology database identified all patients with SPT for a 20-year period (1991-2011). Demographics, clinical characteristics, operative details, pathology, and outcomes data were retrospectively reviewed. Results Eleven patients with SPT were identified. Most were female and Hispanic. Median age at resection was 14 years (9-17 years). Most patients presented with abdominal pain. Diagnostic imaging was most commonly an ultrasound or computed tomography. All tumors were resected en bloc. Median greatest tumor diameter was 5 cm (3.5-12 cm). Median length of stay was 8 days (5-19 days). Complications included pancreatic leak, chyle leak, delayed gastric emptying, fat malabsorption, and incisional keloid. Recurrence developed after 2.5 years in 1 patient with positive surgical margins. There were no metastases or deaths. Median follow-up was 1.4 years (0.6-5.9 years). Conclusion This pediatric series of SPT from a single institution corroborates previous reports in the literature. In our experience, SPT behaves like a low-grade malignancy and has an excellent prognosis. Surgical resection is dictated by tumor location and remains the treatment of choice.
ISSN:0022-3468
1531-5037
DOI:10.1016/j.jpedsurg.2012.03.026