Unresectable Desmoplastic Small Round Cell Tumor: A Case Report and Review of the Literature.

ABSTRACT:

Introduction

Desmoplastic small round cell tumor (DSRCT) is a highly aggressive and rare soft tissue sarcoma that primarily affects young males and typically presents with disseminated abdominopelvic disease. The chromosomal translocation t(11;22)(p13;q12), which generates the EWSR1::WT1 fusion gene, is the defining genetic hallmark that distinguishes DSRCT from other small round cell sarcomas.

Case presentation

We report the case of a 38-year-old male with unresectable intra-abdominal DSRCT treated with the VAC-IE regimen (vincristine, doxorubicin, and cyclophosphamide, alternating with ifosfamide and etoposide) as first-line therapy. He achieved stable disease as the best response, with a progression-free survival of 5 months. As second-line therapy, he received gemcitabin

SUBMITTER: Rueda DA 

PROVIDER: S-EPMC12674665 | biostudies-literature | 2025 Jan-Dec

REPOSITORIES: biostudies-literature