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Management of ampullary tumours in children: still a challenge

机译:儿童壶腹肿瘤的管理:仍然是一个挑战

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摘要

This study describes the case of the youngest patient ever reported with ampullary adenoma. The incidence of ampullary adenomas in childhood is unknown. Endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasound are used in adults to assess and treat these lesions, although there are no instruments designed specifically for use in young children. A six-year-old girl was admitted for abdominal pain, vomiting, pruritus and weight loss. Abdominal ultrasound showed biliary tree (8mm) and pancreatic duct dilatation (4mm). Magnetic resonance cholangiopancreatography and computed tomography confirmed these findings, and also showed displacement of the ampulla to the left upper quadrant. An upper endoscopy confirmed a large ampullary adenoma.A laparotomy was performed and a 5cm villous tumour arising from the ampulla was excised. The postoperative course was uneventful. The histology demonstrated adenoma of the ampulla (intestinal type) without low-grade dysplasia. all clinical and radiological parameters are normal at 20 months follow-up.We describe the case of the youngest patient ever reported with ampullary adenoma. Pancreaticoduodenectomy carries high morbidity and mortality rates, and therefore it should be avoided in absence of histologically proven malignancy. We believe that surgical ampullectomy is a safe and oncologically correct procedure until better endoscopic instruments for peadiatric use will be designed.
机译:这项研究描述了有壶腹腺瘤的最年轻患者。童年时期壶腹腺瘤的发病率未知。尽管没有专门设计用于年幼儿童的仪器,但成人仍可使用内窥镜逆行胰胆管造影(ERCP)和内窥镜超声检查来评估和治疗这些病变。一名六岁女孩因腹部疼痛,呕吐,瘙痒和体重减轻而入院。腹部超声显示胆道树(8mm)和胰管扩张(4mm)。磁共振胰胆管造影和计算机断层扫描证实了这些发现,并且还显示了壶腹向左上象限的移位。上消化道内窥镜检查证实壶腹腺瘤较大,行剖腹术并切除5cm长的壶腹肿瘤。术后过程很顺利。组织学证实壶腹腺瘤(肠型)无低度发育异常。随访20个月,所有临床和放射学参数均正常。我们描述了有壶腹部腺瘤的最年轻患者。胰十二指肠切除术具有较高的发病率和死亡率,因此在没有组织学证实的恶性肿瘤的情况下应避免。我们相信,直到设计出更好的内窥镜用于儿科的器械之前,外科壶腹部切除术是一种安全且在肿瘤学上正确的手术。

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