Slywotzky C M, Bosniak M A
Department of Radiology, New York University Medical Center, Tisch HW 202, 560 First Ave., New York, NY 10016, USA.
AJR Am J Roentgenol. 2001 Apr;176(4):843-9. doi: 10.2214/ajr.176.4.1760843.
Localized cystic disease of the kidney is a benign nonsurgical condition. Its imaging and clinical features are characterized and differentiated from autosomal dominant polycystic kidney disease, multilocular cystic nephroma, and cystic neoplasm.
Localized cystic disease was diagnosed in 18 patients on the basis of a review of imaging studies, clinical histories, and pathologic proof in four of the 18 patients. Average age at diagnosis was 54 years (age range, 24-83 years). Fifteen of the patients (83%) were men. CT was performed on 18 patients, sonography on nine, excretory urography on six, arteriography on four, and MR imaging on two.
Localized cystic disease was unilateral in all patients and characterized by multiple cysts of various sizes separated by normal (or atrophic) renal tissue in a conglomerate mass suggestive of cystic neoplasm. In some patients, involvement of the entire kidney, which was suggestive of unilateral autosomal dominant polycystic kidney disease, was seen. No cysts were seen in the contralateral kidney in 14 patients, and only one or two scattered small cysts were present in four patients. Clinical presentations included hematuria, flank pain, palpable abdominal mass, and localized cystic disease as an incidental finding. None of the patients had a family history of autosomal dominant polycystic kidney disease. Ten patients underwent follow-up (follow-up range, 1-12 years); nine patients underwent imaging follow-up and one patient underwent clinical follow-up, which showed stability of disease. Four patients underwent nephrectomy for suspected renal neoplasm.
Familiarity with localized cystic disease of the kidney and its imaging findings is important to avoid unnecessary surgery and to differentiate the disease from autosomal dominant polycystic kidney disease.
肾局限性囊性疾病是一种良性非手术性病症。对其影像学和临床特征进行描述，并与常染色体显性遗传性多囊肾病、多房性囊性肾瘤及囊性肿瘤相鉴别。
回顾性分析18例肾局限性囊性疾病患者的影像学检查、临床病史，并对其中4例患者进行病理证实。诊断时的平均年龄为54岁（年龄范围24 - 83岁）。15例患者（83%）为男性。18例患者行CT检查，9例行超声检查，6例行排泄性尿路造影，4例行动脉造影，2例行磁共振成像。
所有患者均为单侧发病，表现为大小不一的多个囊肿，被正常（或萎缩）肾组织分隔成一团块状，形似囊性肿瘤。部分患者可见整个肾脏受累，提示单侧常染色体显性遗传性多囊肾病。14例患者对侧肾脏未见囊肿，4例患者对侧肾脏仅有1 - 2个散在小囊肿。临床表现包括血尿、胁腹痛、可触及的腹部肿块以及偶然发现的局限性囊性疾病。所有患者均无常染色体显性遗传性多囊肾病家族史。10例患者接受随访（随访时间1 - 12年）；9例行影像学随访，1例行临床随访，疾病均保持稳定。4例患者因怀疑肾肿瘤而行肾切除术。
熟悉肾局限性囊性疾病及其影像学表现对于避免不必要的手术以及鉴别该疾病与常染色体显性遗传性多囊肾病很重要。
