LGCmain
Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 8249
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size


 
LETTER TO EDITOR Table of Contents   
Year : 2008  |  Volume : 51  |  Issue : 4  |  Page : 573-576
Primary pleomorphic undifferentiated sarcoma of the kidney: A rare renal tumor


Departments of Pathology, Maulana Azad Medical College and Hindu Rao Hospital, New Delhi, India

Click here for correspondence address and email
 

How to cite this article:
Gupta R, Gupta S, Aggarwal D, Singh S. Primary pleomorphic undifferentiated sarcoma of the kidney: A rare renal tumor. Indian J Pathol Microbiol 2008;51:573-6

How to cite this URL:
Gupta R, Gupta S, Aggarwal D, Singh S. Primary pleomorphic undifferentiated sarcoma of the kidney: A rare renal tumor. Indian J Pathol Microbiol [serial online] 2008 [cited 2020 Feb 27];51:573-6. Available from: http://www.ijpmonline.org/text.asp?2008/51/4/573/43774


Malignant fibrous histiocytoma (MFH) is a common soft tissue sarcoma; however, its origin from the renal parenchyma or renal capsule is extremely rare having been reported in only a few cases. [1] We report a similar rare case of a 72-year-old male who presented with complaints of pain in the right upper abdomen for the past 1 year. This was associated with reduced appetite and loss of weight. However, there was no history of hematuria, voiding difficulty, or fever. A physical examination revealed a fixed, non tender mass in the right lumbar region. Routine investigations showed anemia (hemoglobin 8.4g/dl, hematocrit 25%) and a raised erythrocyte sedimentation rate (102 mm in the first hour). Biochemical studies were within normal limits. Imaging studies, including an abdominal ultrasonography (USG) and a computed tomography (CT) scan, revealed an 11x8 cm mass with inhomogeneous enhancement in the upper pole of the right kidney. There was extensive cortical destruction and the mass was extending into the renal vein. However, no involvement of the inferior vena cava or lymphadenopathy was noted. With a pre-operative diagnosis of renal cell carcinoma involving the right kidney, a right radical nephrectomy was performed.

Grossly, the specimen revealed a large, yellowish-white tumor measuring 10x8 cm with areas of hemorrhage and necrosis in the upper pole of the right kidney. There was renal vascular invasion; however, no perinephric fat infiltration was noted. Multiple sections showed a tumor composed of predominantly spindle cells arranged in short fascicles with interspersed histiocytic cells including multinucleated giant cells. Marked nuclear pleomorphism and brisk mitotic activity (10-12 per HPF) were seen. Areas of necrosis and focal inflammatory infiltrate were also present. The tumor was infiltrating into the adjacent renal parenchyma and extending to involve perinephric fat [Figure 1]. On immunohistochemistry, the spindle cells stained for vimentin, while histiocytic cells were positive for CD68. The tumor cells were negative for cytokeratin [Figure 2], epithelial membrane antigen, smooth muscle actin, and S-100 protein. Further immunohistochemistry could not be performed.

A final pathological diagnosis of primary malignant fibrous histiocytoma (pleomorphic undifferentiated sarcoma) of the right kidney was made. [2] The patient was referred for post-operative chemotherapy; however, he developed severe neutropenia with sepsis and died after receiving two cycles of chemotherapy.

Primary renal sarcomas, in general, are rare tumors and account for up to 3% of all renal malignancies. [1] No definite criteria have been defined for the diagnosis of primary renal MFH; however, it has been suggested that (1) there should be no history of sarcoma elsewhere to exclude metastasis, (2) the gross appearance must be compatible with origin in the kidney, (3) sarcomatoid renal cell carcinoma must be excluded, and (4) metastasis, if present, should be smaller than the renal tumor. MFH arising from renal parenchyma or capsule is an extremely rare phenomenon and constitutes less than 6% of renal sarcomas. [1] Only a few cases of renal MFH were found in the available literature. [3] We add another case of this rare entity to the literature.

In the current World Health Organization classification of soft tissue tumors, MFH is considered synonymous with pleomorphic undifferentiated sarcoma. In a recent consensus meeting, it was suggested that this terminology of MFH would be dropped in future classification. After exclusion of other differentiation, the tumor may be classified as an undifferentiated pleomorphic sarcoma. [4] The clinical features of the available previously reported cases of renal MFH are summarized in [Table 1]. A pre-operative radiologic diagnosis of MFH has not been possible in previous reports and simple or radical nephrectomy was undertaken with a suspicion of renal cell carcinoma. [1] Thus, histopathological examination remains the only modality for a correct diagnosis of this rare entity.

The histological differential diagnoses of renal MFH would include other benign and malignant lesions of the kidney. Xanthogranulomatous pyelonephritis (XGPN), a reactive renal pseudotumor, also shows histiocytes, mixed inflammatory infiltrates, and fibroblastic proliferation, which sometimes may show mitotic activity. [5] Reactive fibrohistiocytic proliferations associated with previous surgery or in response to foreign material may also cause a diagnostic dilemma. [3] The most important diagnostic problem is excluding sarcomatoid renal cell carcinoma. This may be achieved by extensive tissue sampling and immunohistochemistry (sarcomatoid carcinoma is positive for cytokeratin and vimentin while MFH is positive for vimentin but negative for cytokeratin). [3] A recently described entity, anaplastic sarcoma of the kidney (ASK), also needs to be considered. ASK was reported as a polyphenotypic tumor composed of a spindle cell component, round to oval cells in myxoid stroma, and divergent differentiation like chondroid and osteoid matrix. Widespread anaplastic nuclear features were noted in the reported cases. Immunohistochemistry performed on a few of the cases showed positivity for vimentin and desmin and negative staining for cytokeratin, MYOD1, NB84a, CD34, CD99, WT1, and CD56. [6] The reported cases underwent radical surgical excision as the therapeutic modality of choice. An incomplete resection leads to recurrence and poor prognosis. The prognosis of renal MFH is extremely poor- 25% of the reported patients died within 1 year of surgery. [3]

In conclusion, primary renal pleomorphic undifferentiated sarcoma (malignant fibrous histiocytoma) is an extremely rare tumor with no distinctive radiological features. The pathologists should be aware of the rare occurrence of this tumor in the kidney.

 
   References Top

1.Kitajima K, Kaji Y, Morita M, Okuda Y, Sugimura K. Malignant fibrous histiocytoma arising from the renal capsule. Magn Reson Med Sci 2003;2:199-202.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Joseph TJ, Becker DI, Turton AF. Renal malignant fibrous histiocytoma. Urology 1991;37:483-9.  Back to cited text no. 2  [PUBMED]  
3.Ptochos A, Karydas G, Iosifidis N, Tyrothoulakis E, Papazafiriou G, Kehagia-Koutoufari T. Primary renal malignant fibrous histiocytoma: A case report and review of literature. Urol Int 1999;63:261-4.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Fletcher CD. The evolving classification of soft tissue tumors: An update based on the new WHO classification. Histopathology 2006;48:3-12.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Singh SK, Mandal AK, Agarwal MM, Das A. Primary renal inflammatory malignant fibrous histiocytoma: A diagnostic challenge. Int J Urol 2006;13:1000-2.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]
6.Vujanic GM, Kelsey A, Perlman EJ, Sandstedt B, Beckwith JB. Anaplastic sarcoma of the kidney: A clinicopathologic study of 20 cases of a new entity with polyphenotypic features. Am J Surg Pathol 2007;31:1459-68.  Back to cited text no. 6    

Top
Correspondence Address:
Ruchika Gupta
162, Pocket-B, Sarita Vihar, New Delhi - 110 076
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.43774

Rights and Permissions


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1]

This article has been cited by
1 Sarcoma indiferenciado pleomórfico primario renal: reporte de caso y revisión de la literatura
Milagros Abad-Licham,Daniel Carbajal-Vásquez,Patricia Coral-González,Teresa Cusma-Quintana,Eloy Silva-Capuñay,Juan Astigueta-Pérez
Gaceta Mexicana de Oncología. 2015; 14(6): 363
[Pubmed] | [DOI]
2 Sarcoma pleomórfico indiferenciado renal primario: reporte de caso y revisión de la literatura
Milagros Abad-Licham,Daniel Carbajal-Vásquez,Patricia Coral-González,Teresa Cusma-Quintana,Eloy Silva-Capuñay,Juan Astigueta-Pérez
Gaceta Mexicana de Oncología. 2015; 14(5): 297
[Pubmed] | [DOI]
3 Long-Term Results of Radical Surgery and Adjuvant Radiotherapy in a Case of Primary Malignant Fibrous Histiocytoma of the Kidney
Luigi Cormio,Francesca Sanguedolce,Paolo Massenio,Giuseppe Di Fino,Giuseppe Lucarelli,Vito Mancini,Pantaleo Bufo,Giuseppe Carrieri
The Open Urology & Nephrology Journal. 2014; 7(1): 1
[Pubmed] | [DOI]
4 Un nuevo caso de histiocitoma fibroso maligno dependiente de cápsula renal
R. Sierra Labarta,M.J. Gil Sanz,G. Muñoz González,L.A. Rioja Sanz
Actas Urológicas Españolas. 2010; 34(1): 122
[Pubmed] | [DOI]
5 A new case of malignant fibrous histiocytoma arising from the renal capsule
R. Sierra Labarta,M.J. Gil Sanz,G. Muñoz González,L.A. Rioja Sanz
Actas Urológicas Españolas (English Edition). 2010; 34(1): 122
[Pubmed] | [DOI]
6 A new case of malignant fibrous histiocytoma arising from the renal capsule | [Un nuevo caso de histiocitoma fibroso maligno dependiente de cápsula renal]
Sierra Labarta, R., Gil Sanz, M.J., Muñoz González, G., Rioja Sanz, L.A.
Actas Urologicas Espanolas. 2010; 34(1): 122-124
[Pubmed]



 

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


    References
    Article Figures
    Article Tables

 Article Access Statistics
    Viewed3327    
    Printed82    
    Emailed2    
    PDF Downloaded202    
    Comments [Add]    
    Cited by others 6    

Recommend this journal