Published in:
01-09-2019 | Case Report
A Case Report of Abdominal Desmoplastic Small Round Cell Tumor in a Young Tunisian Woman
Authors:
Karim Nacef, Mohamed Ali Chaouch, Rym Bouriga, Mohamed Ben Khalifa, Asma Chaouch, Mossab Ghannouchi, Moez Boudokhane
Published in:
Journal of Gastrointestinal Cancer
|
Issue 3/2019
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Excerpt
Desmoplastic small round cell tumour (DSRCT) is a rare and highly aggressive variety of abdominal sarcoma [
1]. It was recently described, in 1989, by Gerald and Rosai [
2]. It affects young males, especially adolescents with an annual incidence of 0.1 cases per million [
3]. The cell of origin is not yet known. But, a peritoneal origin is commonly admitted [
4]. It is postulated to originate from the serosal lining also because it is frequently found in the mesothelial-lined surface, but an epithelial, neurogenic and blastomeric origin are reported [
5]. Due to the small number of patients, treatment guidelines have been difficult to define. In this article, we report a case of an intra-abdominal DSRCT in a young adult Tunisian woman and we proceed to clarify the major characters of this rare tumour. …