A Case Report of Abdominal Desmoplastic Small Round Cell Tumor in a Young Tunisian Woman

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. …