An Autopsy Case of a Desmoplastic Small Round Cell Tumor with a Rapid Fatal Outcome due to Diffuse Cystic Liver Metastases.

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy with a poor prognosis. We report an autopsy case of DSRCT in a 24-year-old male with a rapidly fatal outcome due to unusual diffuse cystic liver metastases. Despite an initial complete response to therapy, the patient developed extensive hepatic involvement leading to acute liver failure. Autopsy revealed widespread DSRCT with cystic degeneration in the liver metastases, which was confirmed by EWSR1 rearrangement. This case highlights the unpredictable nature of DSRCT and its potential for an atypical presentation, while emphasizing the need for improved therapeutic strategies.