Alessandro Crocoli1, Chiara Grimaldi2, Calogero Virgone3, Maria Debora De Pasquale4, Giovanni Cecchetto3, Simone Cesaro5, Gianni Bisogno6, Valerio Cecinati7, Alessandra Narciso1, Daniele Alberti8, Andrea Ferrari9, Patrizia Dall'Igna3, Marco Spada2, Alessandro Inserra1. 1. Department of Pediatric Surgery, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy. 2. Department of Pediatric Surgery and Transplantation, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy. 3. Pediatric Surgery, Department of Women's and Children's Health, University of Padua, Padua, Italy. 4. Department of Oncohematology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy. 5. Pediatric Hematology-Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy. 6. Hematology Oncology Division, Department of Women's and Children's Health, University of Padova, Padova, Italy. 7. Pediatric Hematology and Oncology Unit, Department of Hematology, Transfusion Medicine and Biotechnology, Pescara, Italy. 8. Pediatric Surgery Department, ASST Spedali Civili di Brescia, Brescia, Italy. 9. Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy.
Abstract
BACKGROUND: Solid pseudopapillary pancreatic tumors (SPPT) are an extremely rare entity in pediatric patients. Even if the role of radical surgical resection as primary treatment is well established, data about follow-up after pancreatic resection in children are scant. METHODS: A retrospective review of data from the Italian Pediatric Rare Tumor Registry (TREP) was performed. Short-term (<30 days) and long-term complications of different surgical resections, as well as long-term follow-up were evaluated. RESULTS: From January 2000 to present, 43 patients (male:female = 8:35) were enrolled. The median age at diagnosis was 13.2 years (range, 7-18). Nine children had an incidental diagnosis, whereas 26 complained of abdominal pain and 4 of palpable mass. Tumors arose either from the head of pancreas (n = 14) or from body/tail (n = 29): only one patient presented with metastatic disease. Resection was complete in all patients (cephalic duodenopancreatectomy vs distal resection). At follow-up (median, 8.4 years; range, 0-17 years), one recurrence occurred in a patient with intraoperative rupture. All patients are alive. Three pancreatic fistulas occurred in the body/tail group, whereas four complications occurred in the head group (one ileal ischemia, two stenosis of the pancreatic duct, and one chylous fistula). CONCLUSION: Surgery is the best therapeutic option for these tumors; hence, complete resection is mandatory. Extensive resections, including cephalic duodenopancreatectomy, are safe when performed in specialized centers. Long-term follow-up should be aimed to detect tumor recurrence and to evaluate residual pancreatic function.
BACKGROUND: Solid pseudopapillary pancreatic tumors (SPPT) are an extremely rare entity in pediatric patients. Even if the role of radical surgical resection as primary treatment is well established, data about follow-up after pancreatic resection in children are scant. METHODS: A retrospective review of data from the Italian Pediatric Rare Tumor Registry (TREP) was performed. Short-term (<30 days) and long-term complications of different surgical resections, as well as long-term follow-up were evaluated. RESULTS: From January 2000 to present, 43 patients (male:female = 8:35) were enrolled. The median age at diagnosis was 13.2 years (range, 7-18). Nine children had an incidental diagnosis, whereas 26 complained of abdominal pain and 4 of palpable mass. Tumors arose either from the head of pancreas (n = 14) or from body/tail (n = 29): only one patient presented with metastatic disease. Resection was complete in all patients (cephalic duodenopancreatectomy vs distal resection). At follow-up (median, 8.4 years; range, 0-17 years), one recurrence occurred in a patient with intraoperative rupture. All patients are alive. Three pancreatic fistulas occurred in the body/tail group, whereas four complications occurred in the head group (one ileal ischemia, two stenosis of the pancreatic duct, and one chylous fistula). CONCLUSION: Surgery is the best therapeutic option for these tumors; hence, complete resection is mandatory. Extensive resections, including cephalic duodenopancreatectomy, are safe when performed in specialized centers. Long-term follow-up should be aimed to detect tumor recurrence and to evaluate residual pancreatic function.
Authors: Simone de Campos Vieira Abib; Chan Hon Chui; Sharon Cox; Abdelhafeez H Abdelhafeez; Israel Fernandez-Pineda; Ahmed Elgendy; Jonathan Karpelowsky; Pablo Lobos; Marc Wijnen; Jörg Fuchs; Andrea Hayes; Justin T Gerstle Journal: Ecancermedicalscience Date: 2022-02-17
Authors: Ahmed ElHaddad; Paolo Gasparella; Christoph Castellani; Georg Singer; Erich Sorantin; Klara Zach; Holger Till Journal: European J Pediatr Surg Rep Date: 2019-08-21