Kievit Linda, Kræmer Pia, Hamilton-Dutoit Stephen, Grønbæk Henning
aDepartment of Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus, Denmark.
bDepartment of Endocrinology, Aarhus University Hospital, Aarhus, Denmark.
Case Rep Gastroenterol. 2018 Feb 1;12(1):56-62. doi: 10.1159/000486389. eCollection 2018 Jan-Apr.
A 37-year-old male, who at the age of 8 years had been treated for right-sided Wilms' tumor with nephrectomy, radiotherapy, and chemotherapy, presented with noncirrhotic portal hypertension (NCPH), grade 2 esophageal varices, and ascites. A CT scan demonstrated hypoplasia of liver segments 2 and 3. A liver biopsy showed portal tract fibrosis without cirrhosis, with histological features of NCPH. Liver vein catheterization showed a normal portal pressure gradient of 5 mm Hg while spleen to hepatic vein pressure was 29 mm Hg. NCPH after therapy for Wilms' tumor is described in children within the first few years after treatment. This is the first case report in which the patient first presented symptoms as an adult, many years after cancer treatment.
一名37岁男性，8岁时因右侧肾母细胞瘤接受了肾切除术、放疗和化疗，现出现非肝硬化性门静脉高压（NCPH）、2级食管静脉曲张和腹水。CT扫描显示肝段2和3发育不全。肝活检显示门静脉纤维化但无肝硬化，具有NCPH的组织学特征。肝静脉插管显示门静脉压力梯度正常为5 mmHg，而脾静脉至肝静脉压力为29 mmHg。肾母细胞瘤治疗后的NCPH在治疗后的头几年内在儿童中有所描述。这是首例患者在癌症治疗多年后成年才首次出现症状的病例报告。
