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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT  
Year : 2012  |  Volume : 23  |  Issue : 6  |  Page : 1241-1242
Renal pleomorphic undifferentiated sarcoma: A rarity


The Institute of Post-Graduate Medical Education and Research (IPGMER) and Seth Sukhlal Karnani Memorial (SSKM) Hospital, Kolkota, West Bengal, India

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Date of Web Publication17-Nov-2012
 

   Abstract 

A 70-year-old male presented with progressive weight loss for eight months. Radiological imaging showed a large tumor in the right kidney. The patient underwent right open radical nephrectomy and histopathology revealed pleomorphic undifferentiated sarcoma (PUS) earlier known as malignant fibrous histiocytoma (MFH). One year after surgery, the patient developed pulmonary metastasis. Unfortunately, the patient died after six months.

How to cite this article:
Kumar S, Bansal P, Tiwari P, Kundu AK. Renal pleomorphic undifferentiated sarcoma: A rarity. Saudi J Kidney Dis Transpl 2012;23:1241-2

How to cite this URL:
Kumar S, Bansal P, Tiwari P, Kundu AK. Renal pleomorphic undifferentiated sarcoma: A rarity. Saudi J Kidney Dis Transpl [serial online] 2012 [cited 2020 Jun 4];23:1241-2. Available from: http://www.sjkdt.org/text.asp?2012/23/6/1241/103567

   Introduction Top


The most common primary renal malignant tumors are renal cell carcinoma, which account for 3% of all adult malignancies. Pleomorphic undifferentiated sarcoma (PUS) earlier known as malignant fibrous histiocytoma (MFH) is thought to represent a primitive mesenchymal tumor with partial fibroblastic and histiocytic differentiation. Majority affect the limb structures. Renal PUS constitute only 1-3% of all malignant tumors. We report a case of MFH of kidney due to its rarity.


   Case Report Top


A 70-year-old male patient presented with history of gradual loss of weight for eight months. There was no history of hematuria, stone disease, hypertension or diabetes. On examination he was found to have a lump in right lumbar region which was ballotable on palpation. His hemoglobin was 10 gm% and renal function tests were normal (urea 18 mg%, creatinine 0.7 mg%). Liver function tests were normal. Serum calcium was 9.1 mg%. X-ray chest was normal. Ultrasound revealed a mass occupying the lower part of the right kidney. Contrast-enhanced computed tomography (CT) scan confirmed a large tumor in the right kidney. Fat planes with surrounding structures were maintained and renal vessels were normal [Figure 1]. Histopathological examination revealed spindle cells arranged in patchy storiform pattern with considerable nuclear pleomorphism, foci of necrosis and hyalinization. There were multinucleated giant cells, increased mitotic activity and foamy histiocytes [Figure 1].
Figure 1: Computer-enhanced computed tomography scan – large tumor of right kidney and HPE showing spindle cells in patchy storiform pattern.

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The patient was regularly followed-up with CT scan at six months intervals. It was noted that the patient developed cannon ball secondaries at one year after surgery and was therefore referred to the radiotherapy department. Unfortunately, the patient died after six months.


   Discussion Top


Pleomorphic undifferentiated sarcoma (PUS) earlier known as MFH was first described by O'bren and Stout in 1964. The histogenesis of the tumors is controversial. The tumor cells have shown markers of both mesenchymal cells and cells of mononuclear phagocytic system. [1] Its behavior is like that of sarcoma and, as such, Ghandur-Mnaymanch in 1987 suggested the name to be changed to fibrous histocystic sarcoma in order to reflect its behavior rather than MFH, as if indicating a lymphoma. Approximately 12-14% of all PUS/MFH are documented in the literature. [2] The most common symptoms are palpable abdominal tumor, fever, fatigue, weight loss and gastrointestinal problems. [3] The factors denoting poor prognosis are old age, tumor size, location, either superficial or deep, tumor necrosis, high mitotic count, degree of infiltration and distant metastasis. [4] The best treatment is radical removal of the tumor, [5] although this tumor has a strong predilection for local recurrence. Fifty percent of renal PUH died within one year. [2] Local recurrence is about 20%, and distant metastasis 30%. If there is local infiltration or distant metastasis, treatment with chemotherapy may be indicated, although the response is only 30%. Radiotherapy offers little, if any, benefit. The overall prognosis is poor and the five-year survival rate is less than 14%. [3] In contrast, there are reported cases (two) of longer survival. Our patient did not develop local recurrence but developed pulmonary metastasis and died.

 
   References Top

1.Strauchen JA, Bona D. A Malignant fibrous histiocytoma. Expression of monocyte/macrophage differentiation antigens detected with monoclonal antibodies. Am J Pathol 1986;124: 303-9.  Back to cited text no. 1
    
2.Clinton S, Steyn J. Renal Malignant Fibrous Histiocytoma. Br J Urol 1990;65:109-11.  Back to cited text no. 2
    
3.Arny MM, Soulee H, Living JC. Malignant fibrous histocytoma: A retrospective study of 167 cases. Cancer 1980;45:167-78.  Back to cited text no. 3
    
4.Abbert H, Wagner R, Becht E. Malignant fibrous histocytoma of renal capsule. J Cancer Res Clin Oncol 1981;100:285-93.  Back to cited text no. 4
[PUBMED]    
5.Schneider JA, König H, Hertel E. Malignant Fibrous Histiocytoma of the Renal Capsule. Br J Urol 1992;69:435-6.  Back to cited text no. 5
    

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Correspondence Address:
Suresh Kumar
IPGMER and SSKM Hospital, Kolkota, West Bengal
India
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DOI: 10.4103/1319-2442.103567

PMID: 23168856

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    Abstract
   Introduction
   Case Report
   Discussion
    References
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