Journal of Postgraduate Medicine
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Year : 2010  |  Volume : 56  |  Issue : 3  |  Page : 201-205  

A t (11; 22) (p13; q12) EWS-WT 1 positive desmoplastic small round cell tumor of the maxilla: An unusual case indicating the role of molecular diagnosis in round cell sarcomas

B Rekhi1, R Basak2, SB Desai3, NA Jambhekar1 
1 Department of Pathology, Tata Memorial Center, Dr. E.B. Road, Parel, Mumbai, India
2 Department of Molecular Pathology Advanced Center for Treatment, Research and Education in Cancer, Kharghar, Navi Mumbai, India
3 Department of Pathology, Tata Memorial Center, Dr. E.B. Road, Parel, Mumbai; Department of Molecular Pathology Advanced Center for Treatment, Research and Education in Cancer, Kharghar, Navi Mumbai, India

Correspondence Address:
B Rekhi
Department of Pathology, Tata Memorial Center, Dr. E.B. Road, Parel, Mumbai
India

A desmoplastic small round cell tumor (DSRCT) is an uncommon tumor characterized by polyphenotypic expression and a specific reciprocal translocation t (11; 22) (p13; q12). It has been rarely identified in the head and neck region. Herein, we describe a DSRCT in the maxilla of a young man, who was initially diagnosed with a primitive neuroectodermal tumor (PNET), based on histopathological appearance of a round cell tumor, with MIC2 and -FLI-1 positivity, on immunohistochemistry (IHC). Diagnosis of a DSRCT was confirmed on molecular analysis with positive -RT-PCR and sequencing results for EWS-WT1 transcript and negativity for EWS-FL1. The case is presented to highlight the value of molecular diagnosis in round cell sarcomas at uncommon sites, especially when similar IHC markers can be expressed in a PNET and a DSRCT. An exact diagnosis of a round cell sarcoma has a therapeutic relevance.


How to cite this article:
Rekhi B, Basak R, Desai S B, Jambhekar N A. A t (11; 22) (p13; q12) EWS-WT 1 positive desmoplastic small round cell tumor of the maxilla: An unusual case indicating the role of molecular diagnosis in round cell sarcomas.J Postgrad Med 2010;56:201-205


How to cite this URL:
Rekhi B, Basak R, Desai S B, Jambhekar N A. A t (11; 22) (p13; q12) EWS-WT 1 positive desmoplastic small round cell tumor of the maxilla: An unusual case indicating the role of molecular diagnosis in round cell sarcomas. J Postgrad Med [serial online] 2010 [cited 2022 May 24 ];56:201-205
Available from: https://www.jpgmonline.com/article.asp?issn=0022-3859;year=2010;volume=56;issue=3;spage=201;epage=205;aulast=Rekhi;type=0


 
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