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 Table of Contents  
LETTER TO THE EDITOR
Year : 2016  |  Volume : 21  |  Issue : 2  |  Page : 158-159

Clear cell sarcoma of the kidney occurring in an adult


1 Department of Pathology, Employees' State Insurance Corporation Medical College and PGIMSR, Bengaluru, Karnataka, India
2 Department of Pathology, KLE University's Jawaharlal Nehru Medical College, Belgaum, Karnataka, India

Date of Web Publication31-Aug-2016

Correspondence Address:
Panduranga Chikkannaiah
Department of Pathology, Employees' State Insurance Corporation Medical College and PGIMSR, Bengaluru-560 010, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9903.189532

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How to cite this article:
Chikkannaiah P, Suranagi VV, Gosavi M, Bannur H. Clear cell sarcoma of the kidney occurring in an adult. J Mahatma Gandhi Inst Med Sci 2016;21:158-9

How to cite this URL:
Chikkannaiah P, Suranagi VV, Gosavi M, Bannur H. Clear cell sarcoma of the kidney occurring in an adult. J Mahatma Gandhi Inst Med Sci [serial online] 2016 [cited 2023 Mar 29];21:158-9. Available from: https://www.jmgims.co.in/text.asp?2016/21/2/158/189532

Sir,

A 25-year-old male patient presented with a history of pain abdomen and mass per abdomen since 2 months. On general physical examination pallor was present, blood pressure was 120/80 mm Hg. Per abdomen examination was soft, tender bimanually palpable mass was present in left lumbar and hypochondriac region. Routine hematological investigations showed hemoglobin of 8 g% and biochemical investigations were normal. Urinary cortisol was 130.8 mg/day. Sonography revealed a mixed echoic mass (16 cm × 15.8 cm × 15.2 cm) with few small cystic lesions in the upper left quadrant in the upper pole of the kidney. Computerized tomography (CT) showed mixed density mass lesion (16 cm × 16.6 cm × 16 cm) in left suprarenal region with mass effect. Inferiorly, the lesion was seems to extend up to lower pole of the left kidney; the fat plane was well-defined. The adrenal gland was not separated from the lesion [Figure 1]. With the above features, a clinical diagnosis of renal cell carcinoma was made and with informed consent left radical nephrectomy with hilar lymphadenectomy was performed. At surgery, the tumor was found to infiltrate the psoas muscle through gerotas fascia. Gross examination of the nephrectomy specimen showed irregular mass arising from the upper pole (16 cm × 14 cm × 12 cm). Cut surface revealed solid and cystic, a gray tumor with areas of necrosis, myxoid, and cystic degeneration [Figure 2]a. Microscopic examination revealed tumor cells, round to spindle with amphophilic to the vacuolated cytoplasm, hyperchromatic nuclei with fine dispersed chromatin and indistinct nucleoli. The cells were arranged in sheets with a descriptive branching array of blood vessels and also in pseudo rosette [Figure 2]b and [Figure 2]c. The tumor cells were positive for vimentin [Figure 2]d and negative for cytokeratin and carcinoembryonic antigen (CEA). Lymph nodes were free of tumors and no systemic metastasis on positron emission tomography scan. With the above features a diagnosis of clear cell sarcoma, stage 2 (according to updated National Wilms Tumor Study 5 definition) was offered.
Figure1: Sagittal section of the computerized tomography scan showing large well-defined mixed density mass lesion in the left suprarenal region. Note the extension of the lesion up to lower pole of left kidney

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Figure2:(a) Gross photograph of a cut section of nephrectomy specimen showing irregular mass arising from the upper pole it is solid gray-white in color with cystic and mucoid degeneration.(b) Microphotograph is showing tumor cells, round to spindle with amphophilic to vacuolated cytoplasm, hyperchromatic nuclei with fine dispersed chromatin, and indistinct nucleoli. The cells were arranged in sheets with a descriptive branching array of blood vessels(HandE, ×10).(c) High power of the same(HandE, ×40).(d) Microphotograph showing diffuse positivity for vimentin(vimentin, ×10)

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Clear cell sarcoma of the kidney (CCSK) is an uncommon tumor with an incidence of 4% in children.[1] Occurrence of this tumor in adults is rare with only few case reports and short series.[2],[3] The average age at diagnosis is 2–4 years; however it has been reported in 3-day-old infant and also in 65-year-old patient. Males are commonly affected.[1],[2] The tumor is distinct from wilm's tumor in having unfavorable prognosis, high propensity for bone metastasis and better survival with addition of doxorubicin to treatment protocol.[1],[3]

These tumors arise from the medullary region of the kidney. The exact cell of origin of this neoplasm is not known. The absence of WT-1 gene proves the stem cell origin. Chromosomal translocation such as (1;6) (p32.3;q21) and gain of chromosome 1q and 11q are also been reported.[4] The most characteristic feature of CCSK is its propensity for metastasis with an incidence of 40–60%. Bones are commonly involved followed by lung, retroperitoneum, brain, liver, nasopharynx, neck, scalp, epidural space, paraspinal area, abdominal wall, axilla, and orbit. Bony metastasis is both lytic and sclerotic.[5] The common differential diagnosis of this tumor is wilm's tumor in children, and clear cell carcinoma and undifferentiated renal neoplasm in adults. The absence of nephrogenic rests and negativity for cytokeratin and CEA differentiate it from wilm's tumor.[4] In adult patients, this tumor needs to be differentiated from clear cell carcinoma and sarcomatoid carcinoma.[3]

Sonography is the initial modality of investigating abdominal masses, it will identify the renal origin of the mass and differentiate solid from cystic lesions. CT shows inhomogeneous enhancement with attenuation less than the normal kidney tissue but the changes observed are nonspecific and can be seen in all pediatric renal neoplasms. However, it will help surgeon to localize the tumor and surrounding tissue and gives a plane for surgery. As per the latest National Wilm's Tumor Staging-5, CCSK at all stages is treated with radical nephrectomy followed by chemotherapy with vincristine, cyclophosphamide, doxorubicin, and etoposide for 24 weeks and radiotherapy. The overall survival recorded is 69%. The four prognostic factors identified in the literature are (a) treatment with doxorubicin (b) age (c) stage (d) presence of necrosis. Follow-up is essential for these tumors as there are chances of recurrence.[4]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Viswanathan S, Dave BK, Desai SB. Clear cell sarcoma of the kidney – A study of seven cases over a period of three years. Indian J Pathol Microbiol 2007;50:270-3.  Back to cited text no. 1
[PUBMED]    
2.
Benchekroun A, Zannoud M, el Alj HA, Nouini Y, Marzouk M, Faik M. Clear cell sarcoma of the kidney: 3 case reports. Prog Urol 2002;12:469-73.  Back to cited text no. 2
    
3.
Kural AR, Onal B, Ozkara H, Cakarir C, Ayan I, Agaoglu FY. Adult clear cell sarcoma of the kidney: A case report. BMC Urol 2006;6:11.  Back to cited text no. 3
    
4.
Sharma SC, Menon PA. Clear cell sarcoma of the kidney. J Postgrad Med 2001;47:206-7.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.
Franco A, Dao TV, Lewis KN, Biddinger PW. A case of clear cell sarcoma of the kidney. J Radiol Case Rep 2011;5:8-12.  Back to cited text no. 5
    


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