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An Unusual Presentation of Desmoplastic Small Round Cell Tumour of the Abdomen : Morphological, Immunohistochemical, Ultrastructural, and Molecular Studies
Joint Authors
Wolfswinkel, Karen
Brain, Terry
Khalafallah, Alhossain A.
Seaton, David
Angunawela, Preethika
Source
Case Reports in Oncological Medicine
Issue
Vol. 2011, Issue 2011 (31 Dec. 2011), pp.1-4, 4 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2011-09-19
Country of Publication
Egypt
No. of Pages
4
Main Subjects
Abstract EN
Desmoplastic small round cell tumour (DSRCT) is an aggressive and a rare neoplasm.
We report on a 34-year-old male who had abdominal discomfort with a large intraperitoneal mass.
Histological examination of the tumour biopsy revealed sheets of small round cells.
The cells were positive with vimentin and desmin (with occasional dot positivity) and negative for WT1 and CD 99 with immunohistochemistry.
Cytogenetics showed a translocation disrupting the EWSR 1 gene on 22 q 12 consistent with DSRCT.
Electron microscopic examination showed sparse cytoplasmic organelles.
The patient succumbed 34 months from disease presentation after multiple chemotherapies and thereafter radiotherapy.
In summary, our case exemplifies that it is crucial to combine clinical, histological, and molecular aspects in diagnosing DSRCT especially when characteristic dot positivity with desmin is weak along with deficient marking of WT1 and CD99 by immunohistochemistry.
Histology was also less clear than published examples of this entity with a poor desmoplastic response.
A multidisciplinary approach including early referral to specialised centres is recommended in these cases as tertiary referral centres will be required to substantiate the diagnosis.
American Psychological Association (APA)
Angunawela, Preethika& Khalafallah, Alhossain A.& Wolfswinkel, Karen& Seaton, David& Brain, Terry. 2011. An Unusual Presentation of Desmoplastic Small Round Cell Tumour of the Abdomen : Morphological, Immunohistochemical, Ultrastructural, and Molecular Studies. Case Reports in Oncological Medicine،Vol. 2011, no. 2011, pp.1-4.
https://search.emarefa.net/detail/BIM-452645
Modern Language Association (MLA)
Angunawela, Preethika…[et al.]. An Unusual Presentation of Desmoplastic Small Round Cell Tumour of the Abdomen : Morphological, Immunohistochemical, Ultrastructural, and Molecular Studies. Case Reports in Oncological Medicine No. 2011 (2011), pp.1-4.
https://search.emarefa.net/detail/BIM-452645
American Medical Association (AMA)
Angunawela, Preethika& Khalafallah, Alhossain A.& Wolfswinkel, Karen& Seaton, David& Brain, Terry. An Unusual Presentation of Desmoplastic Small Round Cell Tumour of the Abdomen : Morphological, Immunohistochemical, Ultrastructural, and Molecular Studies. Case Reports in Oncological Medicine. 2011. Vol. 2011, no. 2011, pp.1-4.
https://search.emarefa.net/detail/BIM-452645
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-452645