Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 

 Table of Contents  
Year : 2014  |  Volume : 10  |  Issue : 4  |  Page : 1104-1106

Clear cell sarcoma of the kidney: A case report

1 Department of Pathology, Medical College, Kolkata, India
2 North Bengal Medical College, Siliguri, West Bengal, India

Date of Web Publication9-Jan-2015

Correspondence Address:
Ayandip Nandi
48/7, Patuapara Lane, P.O. Serampore, Hooghly District, Kolkata - 712 201, West Bengal
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.146106

Rights and Permissions
 > Abstract 

Clear cell sarcoma of the kidney is a rare malignant neoplasm of childhood, known for its aggressiveness, its tendency for recurrence, and to metastasize to bone. We report the observation of 8-month-old child presenting with a large abdominal mass. Clinically, it was diagnosed as Wilm's tumor, and left nephrectomy was done. Grossly, 10 cm × 8 cm × 3.5 cm globular, white, encapsulated, smooth mass uniformly involving the whole kidney was noted. Histologically, the tumor was diagnosed as clear cell sarcoma with renal vein showing presence of tumor embolus in lumen. The tumor was given stage-II (NWTS-5 protocol). Immunohistochemistry showed vimentin positive and cytokeratin negative tumor cells. The child is currently undergoing chemotherapy and has not yet shown any sign of bony metastasis.

 > Abstract in Chinese 



肾透明细胞肉瘤是一种罕见的儿童恶性肿瘤,以其侵袭性,复发的倾向,和骨转移而知名。我们报道一例8个月大的孩子表现为大的腹部巨大包块。在临床上,被诊断为肾母细胞瘤,左肾切除术。大体上,10厘米×8厘米×3.5厘米球状,白色,光滑,质量均匀,有囊包裹,涉及整个肾脏引起了我的注意。组织学上,肿瘤被诊断为肾静脉显示管腔肿瘤栓子透明细胞肉瘤。肿瘤为II期(NWTS 5协议)。免疫组化显示波形蛋白阳性,角蛋白阴性的肿瘤细胞。孩子正在接受化疗,尚未显示出任何骨转移的迹象。


Keywords: Childhood renal neoplasms, clear cell sarcoma of kidney, immunohistochemical markers

How to cite this article:
Nag D, Nandi A, Mandal PK, Biswas PK. Clear cell sarcoma of the kidney: A case report. J Can Res Ther 2014;10:1104-6

How to cite this URL:
Nag D, Nandi A, Mandal PK, Biswas PK. Clear cell sarcoma of the kidney: A case report. J Can Res Ther [serial online] 2014 [cited 2021 Dec 9];10:1104-6. Available from: https://www.cancerjournal.net/text.asp?2014/10/4/1104/146106

 > Introduction Top

Clear cell sarcoma of the kidney (CCSK) is a rare malignant renal neoplasm of childhood (4% of primary renal neoplasms), known for its aggressiveness, its tendency for recurrence, and to metastasize to bone. [1],[2] Its peak incidence is in 3-5 years with slightly male (M: F = 2:1) preponderance. [3]

 > Case report Top

A 8-month-old male child presented with gradually increasing abdominal lump for 3 months. There was no history of hematuria, vomiting, loss of weight. On examination, the patient had pallor, BP-85/35 mm Hg, no lymphadenopathy. A diffuse lump was felt in left loin, about 10 cm in diameter, firm to soft, non-tender, ballotable. His basic investigations revealed:

  • CBC: Hb-11 g%, total leukocyte count: 4500/cmm, differential leucocyte count: N 56 L 36 M 8 , erythrocyte sedimentation rate-13 mm/1 st h
  • Urine R/E: WNL
  • Chest X-ray: No abnormality detected
  • USG whole abdomen: Hypoechoic heterogeneous 10 cm× 8 cm × 3 cm lump uniformly involving left kidney. Liver, spleen, right kidney-WNL.

Abdominal computed tomography scan revealed [Figure 1] 10 × 8 × 2 cm heterogeneous mass involving left kidney. Renal vein and ureter were free. There was no paraaortic lymphadenopathy. Provisional diagnosis of Wilm's tumor was made by radiologist.
Figure 1: Abdominal computed tomography of renal mass

Click here to view

Left total nephrectomy was done and specimen was sent for histopathological examination. On gross examination [Figure 2], a globular encapsulated mass measuring 10 × 8 × 3.5 cm was seen. The cut section was greyish-white, homogeneous with whorled pattern and soft glistening areas at places. No hemorrhage, necrosis and variegated area were apparent on gross. Renal vein and ureter were grossly uninvolved. No lymph nodes were identified in the hilum.
Figure 2: Gross appearance of renal mass

Click here to view

Microscopy revealed monomorphous pattern of polygonal tumor cells [Figure 3] with clear vacuolated cytoplasm, round to oval nucleus with fine chromatin and indistinct nucleoli. Evenly distributed network of vascular septae connected by transverse arcades was characteristic in section. Mitotic count was low. Stump of renal vein showed presence of tumor emboli within the lumen [Figure 4]. Perinephric fat was free of tumor. Tumor was localized by a distinct capsule, and no infiltration was seen in adjacent renal parenchyma.
Figure 3: Microscopic appearance of renal mass showing vascular arcades fibrous septae and clear monotonous tumor cells (H and E, ×400)

Click here to view
Figure 4: Tumor emboli within renal vein (H and E, ×400)

Click here to view

Immunohistochemistry showed strong positivity for vimentin [Figure 5] and negativity for cytokeratin [Figure 6]. Final diagnosis of CCSK with the invasion into the renal vein (stage II NWTS-5 protocol) was made. Patient is currently undergoing chemotherapy and has not yet shown any sign of metastasis.
Figure 5: Strong vimentin positivity (Vimentin, ×400)

Click here to view
Figure 6: Cytokeratin negativity (Pan-CK, ×400)

Click here to view

 > Discussion Top

Clear cell sarcoma is a highly aggressive tumor, metastasis to bone is reported in 17% of cases. [4] Marsden et al. called this tumor "bone metastasizing renal tumor of childhood." [5] 5-year survival is 69%. [3] Hence, accurate and early diagnosis of clear cell sarcoma as a separate entity from Wilm's tumor is of extreme importance. Careful study of morphology; the characteristic delicate arborizing vascular septae, monomorphous population and invasive property will help to distinguish it from more common and less aggressive counterparts of childhood renal neoplasms like Wilm's tumor. [6] Points helpful in this matter are: Foci of blastema are not seen in clear-cell sarcoma; nonrenal elements such as cartilage or muscle are not found in clear cell sarcomas. Clear cell sarcomas are unilateral and unicentric, and sclerotic stroma is uncommon in Wilms tumor before therapy. The vascular pattern typical of clear cell sarcoma is often helpful in distinguishing it from Wilms tumor. The border with the kidney is usually infiltrative in CCSK, whereas the border of Wilms tumor is typically "pushing." Histological variants of CCSK include spindle cell, sclerosing, epithelioid, myxoid, pallisading, storiform and anaplastic clear cell sarcoma. [7] Epithelial variety of CCSK mimics wilm's tumor, which can be differentiated by immunohistochemistry. [8] Combined chemotherapeutic approach with doxorubicin, vincristine, cyclophosphamide, etoposide has been proved very effective in reducing recurrence and metastasis. [9]

 > Conclusion Top

Early diagnosis of clear cell sarcoma is of utmost importance because of its aggressive nature. Many variants of CCSK makes it difficult to distinguish this from more common, but less aggressive childhood renal neoplasms like wilm's tumor.

 > References Top

Sharma SC, Menon PA. Clear cell sarcoma of the kidney. J Postgrad Med 2001;47:206-7.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
Namaoui RY, Castex MP, Vial J, Galinier P, Rubie H, Laprie Mazieres A, et al. Clear-cell sarcoma of the kidney: About a paediatric case. Prog Urol 2010;20:465-8.  Back to cited text no. 2
Argani P, Perlman EJ, Breslow NE, Browning NG, Green DM, D′Angio GJ, et al. Clear cell sarcoma of the kidney: A review of 351 cases from the National Wilms Tumor Study Group Pathology Center. Am J Surg Pathol 2000;24:4-18.  Back to cited text no. 3
Haas JE, Bonadio JF, Beckwith JB. Clear cell sarcoma of the kidney with emphasis on ultrastructural studies. Cancer 1984;54:2978-87.  Back to cited text no. 4
Marsden HB, Lawler W, Kumar PM. Bone metastasizing renal tumor of childhood: Morphological and clinical features, and differences from Wilms′ tumor. Cancer 1978;42:1922-8.  Back to cited text no. 5
Sandstedt BE, Delemarre JF, Harms D, Tournade MF. Sarcomatous Wilms′ tumour with clear cells and hyalinization. A study of 38 tumours in children from the SIOP nephroblastoma file. Histopathology 1987;11:273-85.  Back to cited text no. 6
Murphy WM, Beckwith JB, Farrow GM. Tumors of the kidney. In: Murphy WM, editor. Tumors of the Kidney, Bladder and Related Urinary Structures. Washington D.C.: The Armed Forces Institute of Pathology; 1993. p. 67-81.  Back to cited text no. 7
Ogawa K, Nakashima Y, Yamabe H, Hamashima Y. Clear cell sarcoma of the kidney. An immunohistochemical study. Acta Pathol Jpn 1986;36:681-9.  Back to cited text no. 8
Green DM, Breslow NE, Beckwith JB, Moksness J, Finklestein JZ, D′Angio GJ. Treatment of children with clear-cell sarcoma of the kidney: A report from the National Wilms′ Tumor Study Group. J Clin Oncol 1994;12:2132-7.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  >Abstract>Introduction>Case report>Discussion>Conclusion>Article Figures
  In this article

 Article Access Statistics
    PDF Downloaded177    
    Comments [Add]    

Recommend this journal