【双语病例】肾透明细胞肉瘤1例

16 4月

病史、CT影像

病史

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

28个月男孩,左腹部可触及包块,发现1周余。

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

【双语病例】肾透明细胞肉瘤1例

【双语病例】肾透明细胞肉瘤1例

【双语病例】肾透明细胞肉瘤1例

【双语病例】肾透明细胞肉瘤1例

  1. There is a mass arising from the left adrenal gland.

True

False

2.There is a hypodense left para-aortic lymph node.

True

False

3.There is associated retroperitoneal hemorrhage.

True

False

更多问题……

CT also demonstrates a destructive lesion in the L1 vertebral body (not shown in previous images).

CT可见L1椎体溶骨性病变(上图中未给出)

4.Which of the following is the most likely diagnosis given the presence of osseous metastasis, the imaging findings, and patient demographics?

Angiomyolipoma

Renal cell carcinoma

Clear-cell sarcoma

Mesoblastic nephroma

Medullary carcinoma

5.Stage I and II disease for the above diagnosis are defined by imaging features.

True

False

6.Which of the following is the most common location for metastasis of the above tumor?

Regional lymph nodes

Bones

Lungs

Brain

Contralateral kidney

影像表现及诊断

影像表现

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 (image 1). There is a hypodense left para-aortic lymph node (image 3), corresponding to nodal metastasis. No masses are seen in the contralateral kidney.

横断位及冠状位增强CT示左肾髓质巨大肿块,边界清楚,与邻近肾实质呈“claw sign”,占位效应明显。增强后,肿块不均匀强化,内见大片低密度影,提示为坏死囊变。左侧肾上腺与肿块界限清楚,未见明显异常(图1)。腹主动脉旁可见稍低密度结节,考虑为转移性淋巴结(图3)。对侧肾脏未见明显肿块。

鉴别诊断

  • Clear-cell sarcoma

    透明细胞肉瘤

  • Wilms\’ tumor

    肾母细胞瘤

  • Primitive neuroectodermal tumor

    原发性神经外胚层瘤

  • Renal cell carcinoma

    肾细胞癌

  • Mesoblastic nephroma

    中胚层肿瘤

诊断

Clear-cell sarcoma of the kidney (CCSK)

肾透明细胞肉瘤

更多问题……

7.Clear-cell sarcoma of the kidney is most commonly diagnosed at which of the following ages?

Younger than 6 months

2 to 4 years

6 to 10 years

12 to 18 years

8.Which of the following syndromes/conditions has a strong association with clear-cell sarcoma of the kidney?

Beckwith-Wiedemann syndrome

Trisomy 18

Cryptorchidism

Cross fused renal ectopia

None of the above

9.Which of the following neoplasms is derived from the renal medulla?

Renal cell carcinoma

Wilms\’ tumor

Angiomyolipoma

Mesoblastic nephroma

Clear-cell sarcoma of the kidney

10.Which of the following statements is FALSE?

Prognosis of clear-cell sarcoma of the kidney was historically worse than Wilms\’ tumor.

Clear-cell sarcoma of the kidney is more often bilateral than Wilms\’ tumor.

Staging for clear-cell sarcoma of the kidney is based on surgical pathology.

Presence of osseous metastases corresponds to stage IV disease.

11.Which of the following chemotherapies is added to the standard regimen for patients with stage IV disease?

Carboplatin

Vincristine

Cyclophosphamide

Doxorubicin

Etoposide

答案

  1. False
  2. True
  3. False
  4. Clear-cell sarcoma
  5. False
  6. Regional lymph nodes
  7. 2 to 4 years
  8. None of the above
  9. Clear-cell sarcoma of the kidney
  10. Clear-cell sarcoma of the kidney is more often bilateral than Wilms\’ tumor.
  11. Carboplatin

讨论

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

    透明细胞肉瘤是一种少见的、儿童原发性肾脏恶性肿瘤。

  • Histology: There are several variants, the most common of which is myxoid type, although most tumors have a mix of different histologic types.

    组织可分为多种类型,其中最常见的是粘液型,而大部分肿瘤是由多种不同的组织学类型混合构成。

分期

Staging is according to the National Wilms\’ Tumor Study system:

本病的分期方法与美国肾母细胞瘤研究的分期系统类似:

  • I: Organ-confined tumor, intact renal capsule, no involvement of vessels, and no residual tumor at surgical margins.

    I期:肿块局限于肾内,未达到肾包膜,血管无受侵,手术可完全切除。

  • II: Tumor extension beyond renal capsule but still completely resected, no residual tumor at surgical margins, tumor can involve vessels in the renal sinus, and spillage of tumor during surgery or biopsy to the flank

    II期:肿瘤侵犯肾包膜,但仍可完全切除。肿瘤侵犯肾窦内血管。手术或活检时,有部分肿瘤组织散落。

  • III: Unresectable primary tumor, lymph node metastases, residual tumor at surgical margins, tumor spillage into peritoneal surfaces

    III期:肿瘤不能完全切除,淋巴结转移,手术切除后边缘肿瘤参与,肿瘤组织散落至腹膜表面

  • IV: Hematogenous spread to distant organs or extra-abdominal lymph nodes

    IV期:血行转移至其他脏器或腹外淋巴结转移。

转移

  • Classically associated with metastases to bone and brain:可转移至骨骼和脑:
    • 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).

      据文献报道,40%-60%的病例可见骨转移,这一数字高于肾母细胞瘤转移的发病率(因此,这个可以作为初次诊断时两者的鉴别点)

    • Rates of metastases have decreased with current, more aggressive chemotherapy regimens, but the percentage of recurrent metastases occurring in the brain are increasing due to relatively poor penetration of systemic cytotoxic agents (as compared with extracranial disease).

      由于高强度化疗的应用,透明细胞肉瘤转移的发病率有所下降,但由于全身性细胞毒性药物难以渗透至脑组织,所以脑转移的发病率逐渐增加。

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

    本病最常见的转移部位是淋巴结转移(约60%)

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