Department of Radiology, Hôpital Beaujon, Assistance Publique des Hôpitaux de Paris, Université Paris Diderot, Sorbonne Paris Cité, INSERM unité 773 CRB3, Paris, France.
Clin Radiol. 2013 Jul;68(7):e362-9. doi: 10.1016/j.crad.2013.01.024. Epub 2013 Mar 26.
To evaluate the diagnosis and presentation of liver tumours in patients with congenital portosystemic shunts (CPS).
Eight patients were diagnosed in Hôpital Beaujon as having CPS. All patients underwent Doppler ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and histological examination of liver tumours. CPS were classified according to anatomy and the amount of portal flow deviated to the systemic circulation as: total, subtotal, or partial. Liver tumours were diagnosed by needle core biopsy (n = 5) or surgery (n = 3). Clinical follow-up was available in all patients but one (mean follow-up 36 months; range 1-5 years).
Six patients had total CPS, one patient had a subtotal CPS, and the last had a partial CPS. All patients presented with multiple liver nodules (range four to >15). The tumours were characterized as focal nodular hyperplasia (FNH; n = 4), FNH with hepatocellular adenoma (n = 2), and regenerative nodular hyperplasia (n = 2). In four of seven patients (57%) that had follow-up, tumours showed enlargement or new lesions appeared.
In this series of CPS patients, tumours were all benign, multiple, and of hepatocellular origin, and different tumours were present simultaneously in two patients. Tumour enlargement or new nodules were common during follow-up.
评估先天性门体分流（CPS）患者肝肿瘤的诊断和表现。
在博让医院诊断出 8 例 CPS 患者。所有患者均行多普勒超声、计算机断层扫描（CT）、磁共振成像（MRI）和肝肿瘤组织学检查。根据解剖学和门静脉向体循环分流的程度将 CPS 分为完全性、部分性和不完全性。肝肿瘤通过肝穿刺活检（n=5）或手术（n=3）进行诊断。除 1 例患者外（平均随访 36 个月；范围 1-5 年），所有患者均获得临床随访。
6 例患者为完全性 CPS，1 例患者为部分性 CPS，最后 1 例为不完全性 CPS。所有患者均表现为多发肝结节（范围 4-15 个）。肿瘤特征为局灶性结节性增生（FNH；n=4）、FNH 伴肝细胞腺瘤（n=2）和再生性结节性增生（n=2）。在 7 例有随访的患者中（57%），肿瘤增大或出现新病灶。
在本系列 CPS 患者中，肿瘤均为良性、多发且来源于肝细胞，2 例患者同时存在不同类型的肿瘤。随访期间肿瘤增大或出现新结节较为常见。
