肾脏局灶性囊性病变2011.9.20

,Localized Cystic Disease of the Kidney 肾脏局灶性囊性病变,IntroductionLocalized cystic disease of the kidney is a benign nonsurgical condition.肾局灶囊性病变是一种良性的非手术治疗的肾脏疾病 Its imaging and clinical features are characterized and differentiated from autosomal dominant polycystic kidney disease, multilocular cystic nephroma, and cystic neoplasm.它的影像学和临床特征与常染色体显性遗传性多囊肾病、多房性囊性肾脏肿瘤、肾脏肿瘤囊变有区别,Introduction,Localized cystic disease of the kidney has been referred to by a number of different names, including segmental cystic disease of the kidney when it involves just one portion of one kidney, or unilateral cystic disease of the kidney when it involves all of one kidney.肾脏局灶性囊性病变有不同的名称，包含肾的阶段性囊肿性病变（它累及一个肾的某一部分），或者肾脏的单侧囊性病变（累及一个肾脏的全部）,Introduction,It has also been referred to as “unilateral polycystic disease” . The latter term may be misleading and should be discarded because this lesion bears no relationship to autosomal dominant polycystic kidney disease, which is a familial disease with a variety of associated conditions ， none of which are seen in localized cystic disease.它亦指单侧的先天性多囊肾，但后者术语可能被误解,应该抛弃,因为这种病变与常染色体显性遗传性多囊肾并没有关系,后者是一个家族性疾病，有各种各样的相关条件，几乎没有一个表现为肾脏局灶性囊性病变。,Introduction,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若病变单侧发病，在团块状肿块内出现不同尺寸的多发囊肿被正常（或萎缩）的肾组织分隔多提示为囊性肿瘤Clinical presentations included hematuria, flank pain, palpable abdominal mass, and localized cystic disease as an incidental finding临床表现包括血尿,腹痛、可触及的腹部肿块和偶然发现局部囊性病变,None of the patients had a family history of autosomal dominant polycystic kidney disease.所有的病人均无常染色体显性遗传性多囊肾病的家族史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熟悉肾脏的局部囊性疾病和它的影像学表现是重要的,以避免不必要的外科手术，并且和常染色体显性遗传性多囊肾相区别,Introduction,Introduction,Cystic diseases of the kidney encompass a wide spectrum of causes, manifestations, and treatments肾脏囊性病变的病因、临床表现与治疗各不相同Localized cystic disease is an uncommon presentation of renal cystic disease and has been confused with unilateral autosomal dominant polycystic kidney disease, multilocular cystic nephroma, or cystic neoplasm.肾脏局灶性囊性病变是肾脏囊性疾病的一个少见表现形式，容易和单侧常染色体显性遗传性多囊肾、多房性囊性肿瘤、肾肿瘤囊变混淆,CT imaging,Fig. 1A. 46-year-old man who presented with microscopic hematuria and lower urinary tract symptoms attributed to prostatitis. contrast-enhanced axial CT scan at level of middle portion of right kidney shows involvement by localized cystic disease. Note symmetric excretion of contrast medium. 图1A.46岁男性前列腺炎引起镜下血尿和下尿路症状。增强横轴位CT扫描，对比剂对称性充填，在右肾的中间部分被局灶性囊性病变侵犯,CT imaging,Fig. 1B. Same a patient. CT scan slightly inferior to A shows multiple simple cysts separated by attenuated renal tissue (arrow).图1 B.同一病人， 稍低于A图层面CT扫描显示多个单纯囊肿之间被纤细的肾组织分割开(箭头示)。,CT imaging,Fig. 1C. Same a patient. CT scan inferior to B has more masslike appearance.图1C,同一病人， 稍低于B图层面CT扫描显示多个囊肿外观,Fig. 1D. Same a patient. CT scan of lower pole inferior to C that, if viewed in isolation, could be confused with cystic neoplasm with enhancing septations. Arrow denotes attenuated renal tissue. CT at 8-year follow-up examination (not shown) showed no change in appearance or size of cysts. (Courtesy of Gold New York, NY)图1 D 同一病人， 低于C图层面CT扫描，如果孤立的看,容易和囊性肿瘤正在强化的分割混淆。箭头指示纤细的肾组织。CT检查随访8年，囊肿的形态和大小没有变化,CT imaging,CT imaging,Fig. 2A. 67-year-old man who was incidentially found to have left renal abnormality diagnosed as localized cystic disease of kidney. CT scan obtained normally enhancing renal tissue (arrow) separated by simple cysts.图2A.67岁男性，偶然发现左肾异常诊断为左肾囊性病变。CT扫描肾组织(箭头示)正常强化且被囊肿分割,CT imaging,Fig. 2B. Same a patient. CT scan corresponding to A obtained 6 years later shows radiographic stability. Uninvolved right kidney remained normal.图2B.同一病人，对应A图6年后CT扫描，左肾病变未见明显变化。右肾保持正常。,CT imaging,Fig. 2C. Same a patient. Unenhanced axial CT scan of lower pole of left kidney shows scattered calcifications in cyst walls (solid arrows). Regions of interest (circled) measure 1-10 H. Note incidental small duodenal lipoma (open arrow).图2C 同一病人,左肾下极平扫横轴位CT显示囊肿壁有散在钙化 (实箭头示)。兴趣区测量为1-10H（环）。注意附带的十二指肠小脂肪瘤 (空箭头示),CT imaging,Fig. 2D. Same a patient. contrast-enhanced axial CT scan corresponding to C shows no enhancement in corresponding regions of interest (circled), which continue to measure 1-10 H. Note enhancing attenuated renal tissue. Calcification in the cyst walls was not a prominent feature and was seen in only four cases. (Courtesy of Shah H, Little Rock, AR)图2D. 同一病人， 对应C图的横轴位增强CT显示在相应的感兴趣区没有强化（环），CT值为1-10H。注意肾组织有纤细强化。囊肿壁的钙化不是显著的特征，仅4例可见钙化,CT imaging,Fig. 3A. 50-year-old man admitted for evaluation of syncopal episodes and occasional right-sided flank pain. contrast-enhanced axial CT scan shows characteristic findings of localized cystic disease.图3 A.50岁男性病人表现为晕厥频繁发作和偶然右腰痛。增强轴位CT扫描显示典型的右肾囊肿。,MRI imaging,Fig. 3B. Same a patient . T2-weighted half-Fourier acquistion single-shot turbo spin-echo (HASTE) axial image corresponding to A shows multiple cysts in right kidney.图3 B 同一病人， 磁共振半傅里叶采集的T2加权图像，显示A图相应层面的右肾多发囊肿,MRI imaging,Fig. 3C. Same a patient . T2-weighted HASTE coronal image shows involvement of entire kidney by localized cystic disease and single small cyst in left kidney.图3 同一病人，磁共振T2加权冠状位图像显示囊肿累及右肾全部，并且左肾有一个小囊肿,MRI imaging,Fig. 3D. Same a patient . MR arteriogram shows slightly attenuated right main renal artery (arrow) with splaying of intrarenal vessels around multiple cysts. Although entire kidney is involved with cysts, there is enough remaining functioning parenchyma so that excretion of contrast medium is present.图3 D 同一病人，MR动脉血管成像显示右肾动脉略纤细(箭头示) ，并且肾内血管的分支围绕在囊肿周围。尽管整个肾脏被囊肿累及，剩余有功能的肾实质足够排泄目前的对比剂。,Fig. 4. 62-year-old man who was incidentally found to have cystic abnormality of right kidney. CT findings were interpreted as possible cystic neoplasm and patient underwent right nephrectomy. Pathologic evaluation showed findings of localized cystic disease. Although interpreted as possible cystic neoplasm, CT scan viewed with adjacent sections is characteristic of localized cystic