【双语病例】多房性囊性肾瘤1例CT影像表现

21 6月

History: A 52-year-old woman presents for an evaluation of an episode ofgross hematuria and mild flank pain.

病史:52岁女性,肉眼血尿,略感腹部疼痛。

CT urogram was attained for evaluation.

行CTU扫描

【双语病例】多房性囊性肾瘤1例CT影像表现

【双语病例】多房性囊性肾瘤1例CT影像表现

MRI was obtained for further evaluation.

进一步行MR扫描【双语病例】多房性囊性肾瘤1例CT影像表现

【双语病例】多房性囊性肾瘤1例CT影像表现

【双语病例】多房性囊性肾瘤1例CT影像表现

【双语病例】多房性囊性肾瘤1例CT影像表现

Findings 影像表现

CT urogram: Axial nephrographic phase image and coronal maximum intensity projection (MIP) image demonstrate a well-circumscribed, multilocular, thinly septated cystic renal lesion in the left interpolar region. The septa are minimally thickened, without focal nodularity or solid components, and demonstrate enhancement. The cystic components demonstrate attenuation similar to simple fluid. The lesion exerts mass effect on the renal pelvis. There is no excretion of contrast into the cystic components.

CTU:轴位肾实质期及冠状MIP图像示左肾中部见一边界清楚、多房的、有少量分隔的囊性病变。分隔轻度增厚,未见壁结节或实性成分,增强有强化。囊性部分呈均匀液性密度,病变有占位效应,压迫肾盂。排泄期囊内未见造影剂进入。

MRI: The left interpolar multilocular cystic lesion demonstrates T1 and T2 hypointense septa and also T2 hyperintense cystic regions. The apparent diffusion coefficient (ADC) map demonstrates no evidence of restricted diffusion. Postgadolinium image demonstrates mild enhancement of the lesion capsule and internal septations, but without any solid enhancing component.

MRI:左肾中部见一多房囊性病变,分隔在T1WI、T2WI上呈低信号,囊性部分呈长T2信号。ADC图示未见明显弥散受限。增强扫描示囊壁及分隔轻度强化,但未见实性成分。

Differential diagnosis

  • Multilocular cystic nephroma
  • Multicystic dysplastic kidney
  • Simple renal cysts
  • Renal cell carcinoma
  • Wilms tumor

Diagnosis: Multilocular cystic nephroma

鉴别诊断:

  • 多房性囊性肾瘤
  • 多囊肾
  • 单纯性肾囊肿
  • 肾细胞癌
  • 肾母细胞瘤

最后诊断:多房性囊性肾瘤

Key points

Multilocularcystic nephroma

Cystic nephroma is a benign cystic, multilocular renal mass composed of mesenchymal and epithelial tissue.

  • Most often a unilateral lesion.
  • Has Bosniak III imaging features.
  • It is nonhereditary.

囊性肾瘤是一种良性囊性多房性肾肿物,由间质及上皮组织组成。通常发生在单侧;具有Bosniak III级的影像特征;非遗传性。

Pathology

  • Cystic nephromas have a thick fibrous capsule.
  • They have noncommunicating locules with thin septations.
  • They have no aggressive features (i.e., no invasion of adjacent     vascular and lymphatic structures).

病理:囊性肾瘤有较厚的纤维囊壁;各囊腔之间没有交通;病变无侵袭性(不侵犯邻近血管和淋巴结构)

Natural history

  • Mass effect on renal hilum structures can lead to urinary obstruction and consequently infection.
  • Cystic nephromas have an excellent prognosis following resection.

自然病程:占位效应压迫肾门结构能导致尿路梗阻和继发感染;手术切除后预后好。

Epidemiology

Cystic nephroma has a bimodal agedistribution:

  • First peak: 3 months to 4 years old among male patients.
  • Second peak: 40 to 60 years old among female patients.

流行病学:发病年龄呈双高峰分布:

  • 第一个高峰:3个月到4岁的男性患者;
  • 第二个高峰:40至60岁的女性患者。

Clinical presentation

  • Adults present with flank pain, hematuria, and urinary tract infection.
  • Children present with an asymptomatic palpable flank mass.

临床表现:成人表现为腹痛、血尿、尿路感染;儿童表现为无症状的可触及的腹部肿块。

Imaging features

General features:

  • Cystic nephroma appears as a well-circumscribed, multiloculated cystic mass.
  • Can exert mass effect on the renal hilum structures, including the renal pelvis and proximal ureter, leading to obstructive hydronephrosis.

CT:

  • Cystic components demonstrate attenuation similar to or higher than simple fluid, depending on the protein/myxomatous content.
  • No excretion of contrast into the locules.
  • Thin septations are seen, rarely with calcification.
  • There is also enhancement of the capsule, variable mild enhancement of the septa, and no enhancement of the cystic components.

MRI:

T1-weighted:

  • Cystic components: usually hypointense (can be hyperintense due to proteinaceous/hemorrhagic products)
  • Capsule and septa: hypointense

T2-weighted:

  • Cystic components: hyperintense
  • Capsule and septa: hypointense

Diffusion-weighted: No restriction

Postgadolinium:

  • Enhancement of the capsule and septa
  • No solid enhancing components

影像表现

一般特征:囊性肾瘤表现边界清楚的多房囊性肿物,有一定占位效应,压迫肾门区结构,包括肾盂、输尿管近端,从而可导致梗阻性肾积水。

CT:

  • 囊性成分的密度通常与单纯的液体相同,如果含蛋白或黏液成分则密度略高;
  • 排泄期造影剂不进入囊内;
  • 分隔较薄,钙化罕见;
  • 增强扫描囊壁有强化,分隔轻度强化,囊内成分无强化。

MRI:

  • T1WI:囊性成分:通常为低信号,含蛋白或合并出血时表现为高信号;囊壁和分隔呈低信号;
  • T2WI:囊性成分呈高信号,囊壁和分隔呈低信号;
  • DWI:弥散不受限;
  • 增强扫描:囊壁及分隔有强化,无强化的实性成分。

Treatment

Partial or complete nephrectomy is performed in the majority of cases to exclude malignant pathology (cystic renal cell carcinoma cannot be definitively excluded based on imaging features alone).

治疗:大部分病例需要行部分或完全性肾脏切除术,以除外恶性肿瘤(仅凭影像表现无法完全除外囊性肾细胞癌)。

多房性囊性肾瘤知识点图解

【双语病例】多房性囊性肾瘤1例CT影像表现

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