Loading
4 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Desmoplastic small round cell tumour is a very rare sarcoma of adolescents and young men that grows across the lining of the abdomen as many nodules, driven by an EWSR1-WT1 fusion. Treatment is intensive chemotherapy, surgery to remove every nodule, sometimes heated abdominal chemotherapy, and radiotherapy. Most still relapse within three years, so it is treated at a sarcoma centre running trials.
Desmoplastic small round cell tumour is a small round cell sarcoma of the WHO soft tissue classification defined by the EWSR1-WT1 fusion, which up-regulates PDGFR-alpha, VEGF and other proliferation and vascular genes; it affects adolescents and young adults with a male predominance and arises from the serosal surface of the abdominal cavity (Cancers 2021). In 192 SEER cases the peak incidence was at 20 to 24 years, incidence was higher in black than white Americans, survival did not differ by sex or ethnicity, and radiotherapy after surgery was associated with a survival advantage in the adjusted analysis (Journal of Cancer Epidemiology 2014). Current management combines chemotherapy, radiotherapy and aggressive cytoreductive surgery with hyperthermic intraperitoneal chemotherapy, but most patients recur and die within three years; pazopanib, sunitinib and mTOR inhibitors have been evaluated in small trials (Cancers 2021).
How it differs from its parent: a fusion-defined sarcoma of the peritoneum rather than a limb or retroperitoneal mass, spreading as peritoneal carcinomatosis with liver metastases, treated on Ewing-type chemotherapy backbones because of its EWSR1 fusion, and with a surgical strategy borrowed from peritoneal surface oncology.
| Setting | Approach | Guideline |
|---|---|---|
| All cases | Ewing-type multi-agent chemotherapy, complete cytoreductive surgery with or without hyperthermic intraperitoneal chemotherapy, radiotherapy in some centres; pazopanib, sunitinib or mTOR inhibitors on relapse; trials recommended. | not mapped |