|Year : 2014 | Volume
| Issue : 4 | Page : 267-269
Sarcomatoid renal clear cell carcinoma presenting with polycythemia: A case report with review of literature
Monika Rathi1, Satish Kumar Budania2, Mohammad Khalid3, Ankur Mittal4
1 Department of Pathology, Lala Lajpat Rai Memorial Medical College, Meerut; Department of Pathology, Teerthanker Mahaveer Medical College and Research Center, Moradabad, India
2 Department of Psychiatry, Subharti Medical College, Meerut, India
3 Department of Surgery, Mayo Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
4 Department of Urology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
|Date of Web Publication||16-Oct-2014|
228/16, Saket Colony, North Civil Lines, Muzaffarnagar, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Clear cell carcinomas (CCCs) are the common renal cell tumors. Sarcomatoid change in the clear cell tumors is uncommon. Not only this sarcomatoid change raises the grade of the tumors thereby adversely affecting the prognosis but it also may pose diagnostic problems. We discuss a case of diabetic female who presented with abdominal mass and polycythemia. She presented with grade IV and stage I CCC with sarcomatoid change. The tumor belonged to stage I. Left sided nephrectomy and splenectomy was done to treat the tumor. Postoperative radiotherapy was done. The patient was followed-up for a period of 2 years, no recurrence was noted. We report this case due to its unusual clinical and histological presentation.
Keywords: Clear cell carcinoma, renal cell carcinoma, sarcomatoid change
|How to cite this article:|
Rathi M, Budania SK, Khalid M, Mittal A. Sarcomatoid renal clear cell carcinoma presenting with polycythemia: A case report with review of literature. Int J Health Allied Sci 2014;3:267-9
|How to cite this URL:|
Rathi M, Budania SK, Khalid M, Mittal A. Sarcomatoid renal clear cell carcinoma presenting with polycythemia: A case report with review of literature. Int J Health Allied Sci [serial online] 2014 [cited 2021 Sep 28];3:267-9. Available from: https://www.ijhas.in/text.asp?2014/3/4/267/143072
| Introduction|| |
Renal clear cell carcinoma (RCCC) constitutes two-third to three-quarter of all renal cell carcinoma (RCC). Sarcomatous change is seen in 5% cases of RCCC.  Although sarcomatoid change can be seen in any histological subtype of renal carcinoma but majority (more than 80%) of sarcomatous change is seen associated with clear cell RCC. , We report a case of 60-year-old, diabetic female with stage I CCC with associated sarcomatoid change.
| Case report|| |
The 60-year-old female, diabetic for 10 years presented to the surgery outpatient department with a history of left-sided renal mass and hematuria for 1 day. Hematological investigations revealed polycythemia. All other investigations, including blood culture and sensitivity were normal. Ultrasound sonography revealed hypoechoic area with necrosis and calcification in the upper pole of the left kidney with co-incidental cholelithiasis [Figure 1]. Contrast-enhanced computed tomography (CT) revealed that tumor was neither extending into adrenals, Gerota's fascia, vena cava nor was any distant metastasis seen. Left sided radical nephrectomy with splenectomy was done to treat polycythemia. Gross examination showed mass in the upper pole of the kidney measuring 4 cm × 4 cm [Figure 2]. Microscopy showed tumor cells arranged in nests and separated by fibrovascular septa. Nuclei showed pleomorphism, prominent nucleoli, clumped, hyperchromatic nuclei corresponding to grade IV tumor as per Furhman grading  [Figure 3],[Figure 4] and [Figure 5]. Also seen were areas of sarcomatous changes with spindle cells showing moderate pleomorphism in 20% of the tumorous area. So, histologically it was diagnosed as RCC with sarcomatoid change. Tumor was found to be limited by renal capsule and thus belonged to stage I, T1aN0M0 category. The patient's polycythemia got relieved postoperatively. The patient was given radiotherapy postoperatively and was followed-up for a period of 2 years, no recurrence was noted.
|Figure 3: Clear cells in renal cell carcinoma separated by blood vessels (×100)|
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|Figure 4: Clear cell type renal cell carcinoma showing nuclear pleomorphism and prominent nucleoli (H and E, ×400)|
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|Figure 5: Renal clear cell carcinoma showing sarcomatous component (H and E, ×100)|
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| Discussion|| |
Renal cell carcinomas are generally tumors of adults (55-60 years). The male to female ratio is 2:1. Incidence of bilaterality is 1%. Cigarette smoking and hypertension are the risk factors for RCC. 
Clinically, it presents with hematuria (59%), flank pain (41%) and abdominal mass (45%). However, this classic triad of symptoms is present in only <10% of the patients. Other paraneoplastic manifestations of RCC include hypercalcemia, polycythemia, hepatic dysfunction, amyloidosis, fever, and weight loss. , RCC can be detected on intravenous pyelography, CT, ultrasound.  CCCs are associated with loss of genetic material on 3p chromosome. Other genetic abnormality include alterations on chromosomes 14, 8 and 9. 
Grossly, CCC is globular masses that protrude beyond the normal contour. Rarely, they may grow inward or may be diffusely infiltrative. Cut surface shows variegated appearance and may show edema, hemorrhage, necrosis and renal cysts but in cases of sarcomatous change, it may show whitish appearance. 
Microscopically, it shows diffuse pattern of clear cells separated by blood vessels. Clearing of cytoplasm is due to the presence of glycogen. Cytoplasm may range from optically clear to deeply granular.  Sarcomatoid renal cell carcinomas (SRCC) associated with sarcomatous change are more commonly associated with high-grade tumors.  Grading is done as per Furhman grading scheme.  Grade I shows round, uniform nuclei approximately 10 mm in diameter with a minute or absent nucleoli. Grade II tumor shows slightly irregular nuclear contours and diameters of approximately 15 mm with nucleoli visible at ×400. Grade III shows moderately to markedly irregular nuclear contours and diameter of approximately 20 mm with large nucleoli visible at ×100. Grade IV shows nuclei similar to those of grade III, but also multilobular or multiple nuclei or bizarre nuclei and heavy clumps of chromatin. 
On immunohistochemistry, CCC shows positivity for CK8 and CK18. Co-expression of keratin and vimentin is a rule in CCC RCC.  It has been demonstrated that SRCC are associated with higher Ki-67 expression level than the carcinoma components.  Immunohistochemical analysis has shown that sarcomatoid areas still express cytokeratin AE1/AE3 and vimentin in 97% and 56% of cases, respectively. This helps to differentiate SRCC from sarcomas. Also, it has been seen that the in markers expressed in mesenchymal tissue and sarcomas, such as desmin and actin, are not frequently expressed in SRCCs. , Ultrastructurally, electron microscopy also helps in the confirmation of SRCC as the epithelial components such as desmosomes and basal lamina are not visualised in sarcomatous components. 
Clear cell carcinoma has a bad prognosis and is resistant to chemotherapy, radiotherapy and immunotherapy. It may invade the renal vein and may reach up to the right atrium. Surgery is the mainstay of treatment. Chemotherapy has also been used as adjuvant treatment in cases with the possibility of high recurrence rates.  Grading carries prognostic significance. Furthermore, sarcomatoid change is associated with poor outcome.  Hence while reporting the histological subtype of CCC, one should actively search for the sarcomatoid component. Sarcomatoid change in RCC is associated with losses on 13q (75%) and 4q (50%) and increased p53 mutations. Furthermore, polycythemia is seen in only 1-8% of RCC. So one should consider RCC as one of the differential diagnosis of polycythemia. 
| Acknowledgment|| |
We acknowledge Engineer Ayush for his technical help.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]