| Summary: | Introduction: Pancreatoduodenectomy (PD) is rarely required in the paediatric population but is the only curative option for certain pathologies such as pancreatoblastoma of the head of the pancreas. Pancreatoblastoma is a rare childhood tumour originating from pancreatic exocrine epithelial cells. It is the most common malignant pancreatic tumour in children. Case report: We describe the case of a 3-year-old girl with Beckwith-Wiedemann (BW) syndrome, with a background of liver resection aged 18 months for hepatoblastoma. On surveillance imaging, a mass lesion was identified in the head of her pancreas, which was further characterised on dedicated MRI pancreas and endoscopic ultrasound. Fine needle aspirate confirmed the diagnosis of pancreatoblastoma. Because of portal and superior mesenteric venous abutment on the initial imaging, a neo-adjuvant approach was taken with good tumour response to cisplatin and doxorubicin, followed by a Whipple's pancreatoduodenectomy (PD). Conclusion: Using intraoperative video footage, we detail the operative technique of pancreatoduodenectomy in the paediatric population. Modifications to standard reconstruction included a dunking pancreatojejunostomy instead of the more standard Blumgart technique. The patient's background of BW renders a lifelong risk of further solid-organ tumour formation. This case demonstrates how each tumour should be individually assessed with a full MDT approach despite any perceived complexity.
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