Congenital pancreatoblastoma: a case report

Ruol, Michele; Dall’Igna, Patrizia; Alaggio, Rita; Toffolutti, Tiziana; Cattarossi, Luigi; Gamba, Piergiorgio

doi:10.1016/j.epsc.2014.12.004

Cited by 1 publication

(3 citation statements)

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“…Pancreatoblastoma (PBL), although rare, is one of the most common pancreatic exocrine tumours in Childhood [ 31 ]: It accounts for 10%–20% of all pancreatic tumours and typically presents in the first decade of life, with a median age at diagnosis of 5 years [ 11 , 25 ] The congenital form, defined as tumour detected before 3 months of age, is even less common, with the description of 15 cases until 2015 [ 31 ].…”

Section: Pancreatoblastoma (Pbl)mentioning

confidence: 99%

“…Patients with Beckwith–Wiedemann syndrome have an increased risk of developing this tumour; the syndrome is identified in up to 60% of cases of PBL developing during early infancy and in 5% of children developing the tumour later in life [ 32 ]. In the review presented by Ruol et al [ 31 ], 7 out of 15 cases of congenital PBL were affected by Beckwith Wiedemann Syndrome (BWS). PBL has also been associated with familial adenomatous polyposis syndromes [ 31 ].…”

Section: Pancreatoblastoma (Pbl)mentioning

confidence: 99%

“…In the review presented by Ruol et al [ 31 ], 7 out of 15 cases of congenital PBL were affected by Beckwith Wiedemann Syndrome (BWS). PBL has also been associated with familial adenomatous polyposis syndromes [ 31 ].…”

Section: Pancreatoblastoma (Pbl)mentioning

confidence: 99%

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International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines

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Most children with tumors will require one or more surgical interventions as part of the care and treatment, including making a diagnosis, obtaining adequate venous access, performing a surgical resection for solid tumors (with staging and reconstruction), performing procedures for cancer prevention and its late effects, and managing complications of treatment; all with the goal of improving survival and quality of life. It is important for surgeons to adhere to sound pediatric surgical oncology principles, as they are closely associated with improved local control and survival. Unfortunately, there is a significant disparity in survival rates in low and middle income countries, when compared to those from high income countries.The International Society of Paediatric Surgical Oncology (IPSO) is the leading organization that deals with pediatric surgical oncology worldwide. This organization allows experts in the field from around the globe to gather and address the surgical needs of children with cancer. IPSO has been invited to contribute surgical guidance as part of the World Health Organization Initiative for Childhood Cancer. One of our goals is to provide surgical guidance for different scenarios, including those experienced in High-(HICs) and Low-and Middle-Income Countries (LMICs). With this in mind, the following guidelines have been developed by authors from both HICs and LMICs. These have been further validated by experts with the aim of providing evidence-based information for surgeons who care for children with cancer.We hope that this initiative will benefit children worldwide in the best way possible.

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