Choyke P L, Glenn G M, Walther M M, Zbar B, Weiss G H, Alexander R B, Hayes W S, Long J P, Thakore K N, Linehan W M
Department of Radiology, Warren G. Magnuson Clinical Center, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892.
AJR Am J Roentgenol. 1992 Dec;159(6):1229-34. doi: 10.2214/ajr.159.6.1442389.
Von Hippel-Lindau disease is a multisystem disorder predisposing to renal cysts and cancer. The growth and development of these renal lesions have not been documented previously. We reviewed serial CT scans to determine the rates and patterns of growth of renal lesions associated with von Hippel-Lindau disease.
Twenty-eight patients with von Hippel-Lindau disease and renal involvement, including the spectrum from simple cysts to solid masses, had follow-up examinations for at least 1 year (mean, 2.4 years; range, 1-12 years) with serial contrast-enhanced abdominal CT. Renal lesions were measured and characterized. Surgical correlation was available in 12 patients.
Two hundred twenty-eight lesions (eight lesions per patient) were detected. On the basis of their CT appearance, 168 lesions (74%) were classified as cysts, 18 (8%) as cysts with solid components, and 42 (18%) as solid masses. Among 12 patients with pathologic confirmation, the solid components of cystic lesions and solid lesions almost always contained renal carcinoma. The majority of cysts remained the same size (71%) or enlarged (20%); 9% became smaller or entirely involuted during the follow-up period. Although it is generally presumed that renal cysts are precursors to cancers, the transformation of a simple cyst to a solid lesion was observed in only two patients. Among the 42 solid lesions, all but two enlarged with time, with a mean doubling time of 10 months.
The renal lesions associated with von Hippel-Lindau disease exhibited wide differences in growth. The majority of renal cysts grew slowly but some involuted. Transition to solid renal cancer was rare among cysts. Complex cystic and solid lesions contained neoplastic tissue that uniformly enlarged. These data may be used to help predict the progression of renal lesions in von Hippel-Lindau disease.
冯·希佩尔-林道病是一种易患肾囊肿和癌症的多系统疾病。此前尚未记录这些肾脏病变的生长和发展情况。我们回顾了系列CT扫描结果，以确定与冯·希佩尔-林道病相关的肾脏病变的生长速率和模式。
28例患有冯·希佩尔-林道病且有肾脏受累的患者，其肾脏病变范围从单纯囊肿到实性肿块，接受了至少1年（平均2.4年；范围1 - 12年）的系列腹部增强CT随访检查。对肾脏病变进行了测量和特征描述。12例患者有手术相关性资料。
共检测到228个病变（每位患者8个病变）。根据CT表现，168个病变（74%）被分类为囊肿，18个（8%）为有实性成分的囊肿，42个（18%）为实性肿块。在12例有病理证实的患者中，囊性病变和实性病变的实性成分几乎均含有肾癌。大多数囊肿大小不变（71%）或增大（20%）；9%在随访期间变小或完全消退。尽管一般认为肾囊肿是癌症的前身，但仅在2例患者中观察到单纯囊肿转变为实性病变。在42个实性病变中，除2个外均随时间增大，平均倍增时间为10个月。
与冯·希佩尔-林道病相关的肾脏病变在生长方面表现出很大差异。大多数肾囊肿生长缓慢，但有些会消退。囊肿转变为实性肾癌的情况很少见。复杂囊性和实性病变均含有肿瘤组织且均会增大。这些数据可用于帮助预测冯·希佩尔-林道病患者肾脏病变的进展。
