Intra-Abdominal Desmoplastic Small Round Cell Tumor: A Case Report and Review of Literature

Introduction: Desmoplastic small round cell tumor is an uncommon and aggressive sarcoma affecting young males, its symptomatology is non-specific. Abdominal and pelvic CT scans help to orient, while anatomopathological and cytogenetic studies provide a definitive diagnosis. Case Report: We report a case of a 21 years old male who complains of left hypochondral pain, physical examination revealed a painful mass on left hypochondrium. Abdominal computed tomography revealed intersplenorenal mass and one on left iliac fossa. He underwent a laparoscopic exploration and the resultant biopsy revealed a desmoplastic small round cell tumor. He underwent a neoadjuvant chemotherapy followed by surgery and consolidative radiochemotherapy with no recurrence during the 6 months follow up. Discussion: Desmoplastic small round cell is very rare mesenchymal tumor, it is described as a result from the chromosomal translocation t (11; 22) (p13; q12) by Gerald and Rosai in 1991, it affects usually young male, patients consult for abdominal pain or mass, Computed abdominal and pelvic tomography is the first examination to request, it allows diagnostic orientation and classification, while exploratory laparoscopy and biopsy establish diagnosis with certainty showing Well-defined islands of small, round cells, embedded in abundant desmoplastic stroma with the expression of epidermal markers (cytokeratin, Epithelial Membrane Antigen), mesenchymal markers (desmin, vimentin) and neural markers (neuron specific enolase reactivity). The current treatment is based on neoadjuvant chemotherapy followed by surgery and consolidative radiochemotherapy, but the prognosis is poor. Conclusion: Desmoplastic round cell tumor is an uncommon tumor of the young subject, it must be evoked in the presence of adequate imaging, the multimodal treatment is the current standard, but the prognosis remains poor. Future genetic therapies focused on developing targeted immunotherapy, might promise more optimistic ...