【双语病例】肾母细胞瘤VS肾脏透明细胞肉瘤CT

Case  1

History: A 2-year-old child presents with nausea and vomiting.

病史:2岁儿童,有恶心、呕吐的症状。

An abdominal radiograph is shown below.

腹部平片如下所示

【病例】肾母细胞瘤VS肾脏透明细胞肉瘤

Ultrasound images   超声图像

【病例】肾母细胞瘤VS肾脏透明细胞肉瘤

【病例】肾母细胞瘤VS肾脏透明细胞肉瘤

【病例】肾母细胞瘤VS肾脏透明细胞肉瘤

CT images  CT图像

【病例】肾母细胞瘤VS肾脏透明细胞肉瘤

Findings

There is a mixed echogenic mass in the right kidney containing solid and cystic components. CT confirms a large mass centered at the lower pole of the right kidney.

影像表现

右肾见一囊实性成分的混合性回声肿物,CT示右肾下极为中心可见一较大肿物。

Differential diagnosis

  • Wilms\’ tumor
  • Neuroblastoma
  • Nephroblastomatosis
  • Multicystic dysplastic kidney
  • Renal cell carcinoma

鉴别诊断

  • Wilms瘤(肾母细胞瘤)
  • 神经母细胞瘤
  • 肾母细胞瘤病
  • 多囊性发育不良肾
  • 肾细胞癌

Diagnosis: Wilms\’ tumor

最后诊断:肾母细胞瘤

Discussion  讨论

Wilms\’ tumor is the most common malignancy of the kidneys in children. The tumor can grow asymptomatically and is often identified on physical exam as an abdominal mass after significant growth. Children are usually identified with the tumor between ages 1 year and 5 years. Prognosis is very good with a cure rate of greater than 90%.

肾母细胞瘤是儿童最常见的肾脏恶性肿瘤,可无症状,肿瘤明显长大后因发现腹部肿物而就诊。1岁至5岁为肿瘤好发年龄。预后较好,治愈率超过90%。

Radiologic overview  影像综述

Given that Wilms\’ affects the pediatric population, the initial imaging study is usually ultrasound. On ultrasound, Wilms\’ appears as a large, heterogeneous mass of the kidney. Wilms\’ often invades the renal vein and inferior vena cava (IVC), and there may be signs of tumor thrombus invasion on color Doppler.

由于肾母细胞瘤主要见于儿童,所以首要的影像检查方式为超声。在超声上,主要表现为肾脏较大肿物,回声不均,可侵犯肾静脉及下腔静脉,彩色多普勒超声可见癌栓。

After identification on ultrasound, chest CT is usually the next study to stage the tumor. The lungs are the most common location for metastasis. On CT, there is a large heterogeneous mass that replaces the kidney and displaces adjacent organs. There is no engulfment of vessels as seen with neuroblastoma and a “claw sign” is often seen as the tumor displaces the normal renal parenchyma. In 5% of cases, Wilms\’ involves the contralateral kidney, so special attention should be made in evaluating both kidneys.

In this case, there is a heterogenous mass involving the right kidney.

超声明确病变后,常行胸部CT来为肿瘤进行分期,肺是最常见的转移部位。在CT上,肾脏可被较大肿物替代,密度不均,周围脏器受压移位。不会见到神经母细胞瘤样的血管包埋,周围正常肾实质移位表现为“爪征”。在5%的肾母细胞瘤患者,对侧肾脏可受累,因此尤其注意要评估双侧肾脏。

Key points

  • Wilms\’ is the most common renal tumor in children. Peak age of incidence is 3 years.
  • Wilms\’ can grow to a very large size before it is detectable on exam or is symptomatic.
  • Ultrasound is the usual initial imaging test and will show a large, heterogenous mass arising from the kidney.
  • There can be invasion of the renal vein and IVC.
  • Wilms\’ tends to displace vessels rather than envelop them, which is what differentiates it from neuroblastoma.

关键点:

  • 肾母细胞瘤是儿童最常见的肾脏肿瘤,发病高峰年龄为3岁;
  • 肿瘤体积较大,可因体检或出现症状而发现;
  • 超声通常为首要检查,表现为肾脏较大的不均质肿物;
  • 可见肾静脉及下腔静脉受侵;
  • 肿瘤倾向于推移血管而不是包埋,借此可与神经母细胞瘤向鉴别。

Case  2

History: A 28-month-old boy presents with a palpable lump in the left abdomen, first noticed one week prior.

病史:28月大的男婴,一周前发现其左腹部可触及一肿物。

Images from contrast-enhanced CT of the abdomen are shown below.

腹部CT增强扫描图像如下所示

【病例】肾母细胞瘤VS肾脏透明细胞肉瘤

【病例】肾母细胞瘤VS肾脏透明细胞肉瘤

【病例】肾母细胞瘤VS肾脏透明细胞肉瘤

【病例】肾母细胞瘤VS肾脏透明细胞肉瘤

Findings

Axial and coronal contrast-enhanced CT images of the abdomen demonstrate a large well-circumscribed mass arising from the left renal medulla with mass effect upon and associated claw sign from the adjacent renal parenchyma. The mass demonstrates heterogeneous enhancement with large hypoattenuating components, corresponding areas of necrosis, and cystic change. The left adrenal gland is separately identified and normal in appearance. There is a hypodense left para-aortic lymph node, corresponding to nodal metastasis. No masses are seen in the contralateral kidney.

影像表现

腹部轴位及冠状CT增强图像示左肾髓质见一较大肿物,边界清楚,向上压迫肾实质,与邻近肾实质间形成爪征。肿物不均质强化,可见大片无强化区,提示坏死、囊变。左肾上腺正常显示。腹膜后主动脉旁可见增大淋巴结,提示淋巴结转移。对侧肾脏未见肿物。

 

Differential diagnosis

  • Clear-cell sarcoma
  • Wilms\’ tumor
  • Primitive neuroectodermal tumor
  • Renal cell carcinoma
  • Mesoblastic nephroma

鉴别诊断

  • 透明细胞肉瘤
  • 肾母细胞瘤
  • PNET
  • 肾细胞癌
  • 中胚层肾瘤

 

Diagnosis: Clear-cell sarcoma of the kidney (CCSK)

最后诊断:肾脏透明细胞肉瘤

 

Key points

Clear-cell sarcoma of the kidney (CCSK)

Clear-cell sarcoma of the kidney is a rare, primary pediatric renal malignancy.

肾脏透明细胞肉瘤是一种罕见的原发性小儿肾脏恶性肿瘤。

Metastases:

Classically associated with metastases to bone and brain:

Osseous metastases have been reported in 40% to 60% cases, which is much higher than the prevalence in Wilms\’ tumor (therefore, a useful feature to guide the differential diagnosis during initial evaluation).

Most prevalent location for metastases at presentation are lymph nodes (approximately 60%).

转移:

可转移至骨骼及脑:骨转移发生率40%-60%,比肾母细胞瘤的发生率更高,这一特点可作为最初评估病变的鉴别诊断点之一。

最常见的为淋巴结转移,约占60%。

Epidemiology

Clear-cell sarcomas of the kidney comprise approximately 5% of primary pediatric renal tumors.

It is the second most common pediatric renal tumor after Wilms\’ tumor.

Mean age of diagnosis is 36 months.

Rarely diagnosed at younger than 6 months of age.

There is a male gender predilection (approximately 2:1).

流行病学:

肾脏透明细胞肉瘤占小儿原发性肾肿瘤的5%,发病率仅次于肾母细胞瘤;平均发病年龄为36个月,6个月以下的患者少见。男女比例为2:1。

Clinical presentation

Abdominal distention

Palpable abdominal mass

Rarer symptoms: vomiting and hypertension

临床表现:

  • 腹部膨胀
  • 可触及腹部肿物
  • 罕见症状:呕吐及高血压

Imaging features

General:

Clear-cell sarcoma of the kidney has a relatively nonspecific imaging appearance, often very similar to Wilms\’ tumor, so diagnosis needs confirmation with tissue histology.

Almost always a unilateral tumor (as opposed to Wilms\’ tumor, which can be bilateral).

Osseous metastases are more common in clear-cell sarcoma of the kidney than Wilms\’ tumor.

CT:

  • CT demonstrates a large, well-circumscribed mass.
  • There are variable proportions of necrotic and cystic components.
  • Shows heterogeneous enhancement.
  • Extracapsular extension is relatively common.
  • Vascular involvement occurs in a small minority of cases.

Radionuclide bone scan: Useful for staging, given the relatively high frequency of bone metastases.

影像表现

肾脏透明细胞肉瘤无特异性影像表现,常类似于肾母细胞瘤,需组织学来明确诊断。常为单侧肾脏肿瘤,与肾母细胞瘤相比,肾脏透明细胞肉瘤发生骨转移更为常见。

CT:

  • 肾脏肿物,体积较大,边界清楚;
  • 有不同程度的坏死、囊变;
  • 不均质强化;
  • 累及肾被膜外,相对较常见;
  • 少部分病例可见血管受累。

放射性同位素骨扫描:考虑到骨转移发生率较高,可借此进行分期。

透明细胞肉瘤:为发生于儿童的罕见恶性肿瘤,起源不明,患者平均年龄36个月。新版WHO肿瘤分类更新了其分子遗传学改变。约10%的肿瘤具有t(10;17)(q22;p13)易位,产生YWHAE-FAM22融合基因,该基因也存在于一部分高级别子宫内膜间质肉瘤中。

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