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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 51  |  Issue : 3  |  Page : 409-410
Primary renal fibrosarcoma: A rare case report and review of literature


1 Department of Pathology, Lady Hardinge Medical College and Associated Hospitals, New Delhi, India
2 Department of Surgery, Lady Hardinge Medical College and Associated Hospitals, New Delhi, India

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   Abstract 

Primary renal fibrosarcoma is a rare tumor. We describe a case of primary renal fibrosarcoma in a 54-year-old male who presented with abdominal lump and intermittent hematuria.

Keywords: Fibrosarcoma, renal, tumor, malignancy, renal cell carcinoma

How to cite this article:
Agarwal K, Singh S, Pathania O P. Primary renal fibrosarcoma: A rare case report and review of literature. Indian J Pathol Microbiol 2008;51:409-10

How to cite this URL:
Agarwal K, Singh S, Pathania O P. Primary renal fibrosarcoma: A rare case report and review of literature. Indian J Pathol Microbiol [serial online] 2008 [cited 2019 Dec 9];51:409-10. Available from: http://www.ijpmonline.org/text.asp?2008/51/3/409/42541



   Introduction Top


Fibrosarcoma of kidney is a rare tumor. [1] Microscopically, this tumor may be confused easily with leiomyosarcoma or sarcomatoid renal cell carcinoma (RCC). Immunohistochemistry for cytokeratin (CK), vimentin, desmin and S-100 helps to rule out these lesions.

We are presenting a rare case of primary renal fibrosarcoma, which was histologically diagnosed as sarcomatoid RCC; however, immunohistochemistry helped in establishing its diagnosis.

The rarity of this lesion prompted this case report.


   Case history Top


A 54-year-old male, a chronic smoker and alcoholic presented to surgical OPD with complaints of anorexia, weight loss, intermittent hematuria and pain in the lumbar region. This was followed by lump on the same side 3 months later. The lump had gradually increased in size. On examination, the patient was mildly anemic and there was no other positive finding on general or systemic examination. Per abdomen examination revealed a mass measuring 12 10 cm in the right lumbar region, which was firm to hard in consistency. Liver and spleen were not palpable and there was no free fluid in the abdomen. Kidney function tests were within normal limits. Ultrasound abdomen and contrast enhanced computer tomography showed a lobulated mass in the right kidney with heterodense enhancement. No extension in renal vein and inferior vena cava was seen. Clinical diagnosis of RCC was kept. The patient underwent right radical nephrectomy.

Grossly we received specimen of the right kidney, which was enlarged measuring 17.5 12.5 9 cm. External surface was bosselated and partially encapsulated. Cut surface showed a lobulated firm grayish-white tumor, involving almost whole kidney and reaching up to the capsule [Figure 1]. The tumor infilterated the pelvis; however, ureter was uninvolved. Microscopic examination showed a partially encapsulated tumor tissue composed of spindle cells in fascicular arrangement with intermixed inflammatory infiltrate [Figure 2A]. Cells were spindle to round having ill-defined, scant to moderate eosinophilic cytoplasm. Nuclei were spindle to oval with moderate anisonucleosis, irregular nuclear membrane and irregularly distributed chromatin [Figure 2B]. There were areas showing calcification, necrosis with foreign body giant cell reaction. Based on the morphological findings, differential diagnosis of sarcomatoid RCC and sarcoma kidney was kept. Following this immunohistochemistry was put up with panel of markers including PAN CK, EMA, S-100, desmin, smooth muscle actin (SMA) and vimentin. Tumor cells were negative for most of these markers, except for vimentin that was diffusely positive in most of the tumor cells. Based on the morphology and immunohistochemical findings, a final diagnosis of fibrosarcoma kidney was made.

The patient made an uneventful recovery following surgery. The patient came for 6-month follow-up postsurgery and there were no symptoms or evidence of metastasis.


   Discussion Top


Primary renal sarcoma is a rare entity accounting for 1% of all renal neoplasm and the majority being leiomyosarcoma.[2] Primary renal fibrosarcoma is a very rare tumor. In the past, these cases were grouped under common term sarcoma. Some reported cases may actually represent sarcomatoid RCC. With recent improvement in histochemical techniques, pathologists are now able to separate actual cases of primary fibrosarcoma kidney. Cavaliere et al. [3] found a single case in 10-year survey of priming renal sarcoma and Grignon et al.[4] found a single case in a study of 17 cases of priming sarcoma of kidney.

Fibrosarcoma is seen in age group of 40-60 years with male and female being equally affected. It originates from the capsule of kidney, which contains much of fibrous and connective tissue. Tumor is large, solid and fleshy with infiltrative margins. Histologically, they show a uniform fasciculated growth pattern with varying amount of collagen. The major differential diagnosis is leiomyosarcoma and sarcomatoid RCC.

They can be differentiated only on immunohistochemistry, as fibrosarcoma are diffusely positive for vimentin and negative for CK and desmin, whereas sarcomatoid RCC and leiomyosarcoma are positive for CK and desmin, respectively.

Tumor is manifested by rapid growth and invades renal vein in nearly half of the cases. Nephrectomy is the only mode of treatment. Five-year survival rates are generally poor and have been reported to be as low as 10%. [5] Tumor is resistant to radiotherapy and chemotherapy. Pettirssen [6] in his review found only two long-term survivors among 21 cases. In the present case, the patient was free of symptoms 6 months postsurgery.


   Conclusion Top


Priming renal fibrosarcoma is a rare renal sarcoma that must be differentiated from other renal sarcomas and sarcomatoid RCC, as it has poor survival rate and is resistant to radiotherapy and chemotherapy. This case is presented due to its rarity and to highlight the importance of immunohistochemistry in diagnosing such cases.

 
   References Top

1.Kansara V, Powell I. Fibrosarcoma of kidney. Urology 1980;16:419-21.  Back to cited text no. 1  [PUBMED]  
2.Grignon DJ, Ro J Y, Ayala AG. Mesenchymal tumors of the kidney. In : Eble JN, editor. Tumors and tumor like conditions of the kidneys and ureters. New York: Churchill Livingstone; 1990. p. 123.  Back to cited text no. 2    
3.Cavaliere A, Fratini D, Legitimo C, Tosi F, Lolli E. Renal fibrosarcoma: Single cell in a ten year survey. Pathologica 1984;76:615-2.  Back to cited text no. 3    
4.Grignon DJ, Ayala AG, Ro JY, el-Naygar A, Papadopoulos NJ. Primary sarcoma of the kidney: A clinicopathologic and DNA flow cytometric study of 17 cases. Cancer 1990;65:1611-8.  Back to cited text no. 4    
5.Judd ES, Donal JM. Sarcoma of the kidney of the adult: A review of twenty cases with a report of a case. Ann Surg 1931;96:1028-35.  Back to cited text no. 5    
6.Pettirssen RO. Mesencaymal neoplasms of kidney. In urologic pathology., Philadelphia: JB Lippencott; 1986. p. 113.  Back to cited text no. 6    

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Correspondence Address:
Smita Singh
E-112, Narwana Apartment, Plot No.89, I.P. Extn., New Delhi - 110 092
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.42541

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    Figures

  [Figure 1], [Figure 2A], [Figure 2B]

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    Abstract
    Introduction
    Case history
    Discussion
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    References
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