Solid pseudopapillary and malignant pancreatic tumors in childhood: A systematic review and evidence quality assessment |
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| Authors: | Konstantinos S. Mylonas Ilias P. Doulamis Diamantis I. Tsilimigras Dimitrios Nasioudis Dimitrios Schizas Peter T. Masiakos Cassandra M. Kelleher |
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| Affiliation: | 1. Department of Pediatric Surgery, Massachusetts General Hospital, Boston, Massachusetts;2. Pediatrics Working Group, Society of Junior Doctors, Athens, Greece;3. Surgery Working Group, Society of Junior Doctors, Athens, Greece;4. First Department of Surgery, Laiko General Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece;5. Department of Obstetrics and Gynecology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania |
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| Abstract: | A systematic review of 32 studies reporting on 489 children with pancreatic tumors was performed. The most prevalent histologic subtype was solid pseudopapillary tumor (SPT) (61.3%). Pancreaticoduodenectomy was the most commonly performed operation (48%). Neoadjuvant chemo/radiotherapy was utilized in 3.9, 36.2, 25, and 27.8% of patients with SPTs, pancreatoblastomas (PBLs), neuroendocrine tumors, and exocrine carcinomas, respectively. Adjuvant chemotherapy (75.6%) and radiation (34%) were most commonly utilized in PBLs. All‐cause mortality was highest in exocrine carcinomas (50%). Overall, 98.8% of patients with SPTs survived. PBL exhibited the highest recurrence rate (14.7%) within a mean of 23.5 months. |
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| Keywords: | pancreatic neuroendocrine tumors pancreatic tumors pancreatoblastoma pediatric solid pseudopapillary tumors |
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