Outcome after surgery for solid pseudopapillary pancreatic tumors in children

Report from the TREP project-Italian Rare Tumors Study Group

Alessandro Crocoli, Chiara Grimaldi, Calogero Virgone, Maria Debora De Pasquale, Giovanni Cecchetto, Simone Cesaro, Gianni Bisogno, Valerio Cecinati, Alessandra Narciso, Daniele Alberti, Andrea Ferrari, Patrizia Dall'Igna, Marco Spada, Alessandro Inserra

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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.

Original languageEnglish
Pages (from-to)e27519
JournalPediatric Blood and Cancer
DOIs
Publication statusE-pub ahead of print - Oct 26 2018

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Neoplasms
Pancreaticoduodenectomy
Head
Pediatrics
Pancreatic Fistula
Recurrence
Pancreatic Ducts
Abdominal Pain
Fistula
Registries
Rupture
Pancreas
Pathologic Constriction
Ischemia
Therapeutics

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Outcome after surgery for solid pseudopapillary pancreatic tumors in children : Report from the TREP project-Italian Rare Tumors Study Group. / Crocoli, Alessandro; Grimaldi, Chiara; Virgone, Calogero; De Pasquale, Maria Debora; Cecchetto, Giovanni; Cesaro, Simone; Bisogno, Gianni; Cecinati, Valerio; Narciso, Alessandra; Alberti, Daniele; Ferrari, Andrea; Dall'Igna, Patrizia; Spada, Marco; Inserra, Alessandro.

In: Pediatric Blood and Cancer, 26.10.2018, p. e27519.

Research output: Contribution to journalArticle

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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.",
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T1 - Outcome after surgery for solid pseudopapillary pancreatic tumors in children

T2 - Report from the TREP project-Italian Rare Tumors Study Group

AU - Crocoli, Alessandro

AU - Grimaldi, Chiara

AU - Virgone, Calogero

AU - De Pasquale, Maria Debora

AU - Cecchetto, Giovanni

AU - Cesaro, Simone

AU - Bisogno, Gianni

AU - Cecinati, Valerio

AU - Narciso, Alessandra

AU - Alberti, Daniele

AU - Ferrari, Andrea

AU - Dall'Igna, Patrizia

AU - Spada, Marco

AU - Inserra, Alessandro

N1 - © 2018 Wiley Periodicals, Inc.

PY - 2018/10/26

Y1 - 2018/10/26

N2 - 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.

AB - 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.

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JO - Pediatric Blood and Cancer

JF - Pediatric Blood and Cancer

SN - 1545-5009

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