Choyke P L, Walther M M, Glenn G M, Wagner J R, Venzon D J, Lubensky I A, Zbar B, Linehan W M
Department of Radiology, Warren G. Magnuson Clinical Center, National Institutes of Health, Bethesda, MD 20892-1182, USA.
J Comput Assist Tomogr. 1997 Sep-Oct;21(5):737-41. doi: 10.1097/00004728-199709000-00014.
Our goal was to describe the imaging features of hereditary papillary renal cancer syndrome (HPRC), a new familial cancer syndrome.
Members of seven kindreds with HPRC comprising 78 individuals were screened with contrast-enhanced CT and abdominal US. MRI was performed in three patients. Enhancement values and doubling times of solid masses were determined from CT scans.
Seventeen of 78 (22%) patients were affected. The HPRCs demonstrated lower enhancement (mean change in enhancement = 31 HU) than a comparable group of clear cell tumors (mean change in enhancement = 67 HU: p = 0.00001). The median tumor doubling time on serial CT was 18 months. The HPRCs were relatively hypovascular, enhanced uniformly, and grew slowly. Therefore, careful measurements of the region of interest should be obtained before and after intravenous administration of contrast medium. Though US detected only 45% of the lesions visualized on CT, it was useful in determining if lesions were cystic. Contrast-enhanced MRI demonstrated similar characteristics to contrast-enhanced CT.
The tumors of patients with HPRC pose some diagnostic difficulties because they can be missed by US, are small, and enhance poorly on CT. CT is preferable to US as a screening tool because of its higher sensitivity in detecting small lesions, and when contrast media cannot be administered, MR is a suitable alternative to CT.
我们的目标是描述遗传性乳头状肾癌综合征（HPRC）这一新型家族性癌症综合征的影像学特征。
对7个患有HPRC的家族中的78名成员进行了对比增强CT和腹部超声检查。对3例患者进行了MRI检查。从CT扫描中确定实性肿块的强化值和倍增时间。
78例患者中有17例（22%）患病。HPRC的强化程度低于一组可比的透明细胞肿瘤（强化平均变化 = 31 HU）（透明细胞肿瘤强化平均变化 = 67 HU：p = 0.00001）。连续CT上肿瘤的中位倍增时间为18个月。HPRC相对血供较少，强化均匀，生长缓慢。因此，在静脉注射造影剂前后应仔细测量感兴趣区域。虽然超声仅检测到CT上显示病变的45%，但它有助于确定病变是否为囊性。对比增强MRI显示出与对比增强CT相似的特征。
HPRC患者的肿瘤存在一些诊断困难，因为它们可能被超声漏诊，体积小，且在CT上强化不佳。CT作为筛查工具优于超声，因为其在检测小病变方面具有更高的敏感性，当不能使用造影剂时，MR是CT的合适替代方法。
