{"entity":{"id":"desmoplastic-small-round-cell-tumour","kind":"cancer","name":"Desmoplastic small round cell tumour","aka":["DSRCT","Desmoplastic small round cell tumor","Intra-abdominal desmoplastic small round cell tumour","EWSR1-WT1 sarcoma"],"tldr":"Desmoplastic small round cell tumour is a very rare, aggressive sarcoma of adolescents and young men that grows across the lining of the abdomen as many nodules, driven by a fusion of the EWSR1 and WT1 genes. It is treated with intensive chemotherapy, surgery to remove every nodule, sometimes heated chemotherapy into the abdomen, and radiotherapy, yet most patients relapse within three years.","summary":"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).\n\nHow 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.\n\nHow common: 0.2 to 0.5 per million a year (Journal of Cancer Epidemiology 2014).\n\nTreatment: multi-agent Ewing-type chemotherapy (vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide and etoposide), complete cytoreductive surgery with or without hyperthermic intraperitoneal chemotherapy, whole-abdominal radiotherapy in some centres, and pazopanib, sunitinib or mTOR inhibitors for relapsed disease; trials are the recommended route given the outcomes (Cancers 2021).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Desmoplastic_small-round-cell_tumor","links":[{"label":"NCI PDQ: childhood soft tissue sarcoma treatment","url":"https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq"},{"label":"Journal of Cancer Epidemiology 2014: incidence and outcomes of desmoplastic small round cell tumour, SEER","url":"https://doi.org/10.1155/2014/680126"},{"label":"Cancers 2021: desmoplastic small round cell tumour, molecular abnormalities and emerging therapy (review)","url":"https://doi.org/10.3390/cancers13030498"}],"tags":["subtype-page","wave4","rare"],"related":["sarcoma","ewing-sarcoma","rhabdomyosarcoma","peritoneal-mesothelioma","childhood-cancers","adamantinoma"],"cancers":[],"sections":[],"technologies":["hipec"],"targets":["wt1","pdgfra"],"drugs":["vincristine","doxorubicin","cyclophosphamide","ifosfamide","etoposide","pazopanib","sunitinib"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"sarcoma","burden":"Very rare: 192 cases in the US SEER registry, with peak incidence at ages 20 to 24 and age-adjusted rates of 0.5 per million in black and 0.2 per million in white Americans (Journal of Cancer Epidemiology 2014).","subtypes":["Intra-abdominal desmoplastic small round cell tumour (peritoneal; the usual form)","Extra-abdominal desmoplastic small round cell tumour (thoracic, paratesticular; rare)","Desmoplastic small round cell tumour with liver metastases at diagnosis"],"biomarkers":["EWSR1-WT1 fusion (FISH or RT-PCR); WT1 C-terminus immunohistochemistry","Polyphenotypic staining (cytokeratin, desmin, neural markers)","Peritoneal cancer index and completeness of cytoreduction","PDGFR-alpha and VEGF expression (targets)"],"standardOfCare":[{"setting":"All cases","approach":"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.","refs":["sarcoma","vincristine","doxorubicin","cyclophosphamide","ifosfamide","etoposide","hipec","pazopanib","sunitinib"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"sarcoma"},"route":"/cancers/desmoplastic-small-round-cell-tumour/","neighbours":{"cancer":[{"id":"adamantinoma","kind":"cancer","name":"Adamantinoma of bone","route":"/cancers/adamantinoma/"},{"id":"childhood-cancers","kind":"cancer","name":"Childhood cancers (all types)","route":"/cancers/childhood-cancers/"},{"id":"ewing-sarcoma","kind":"cancer","name":"Ewing sarcoma","route":"/cancers/ewing-sarcoma/"},{"id":"peritoneal-mesothelioma","kind":"cancer","name":"Peritoneal mesothelioma","route":"/cancers/peritoneal-mesothelioma/"},{"id":"rhabdomyosarcoma","kind":"cancer","name":"Rhabdomyosarcoma","route":"/cancers/rhabdomyosarcoma/"},{"id":"sarcoma","kind":"cancer","name":"Sarcomas (soft tissue, bone, GIST)","route":"/cancers/sarcoma/"}],"technology":[{"id":"hipec","kind":"technology","name":"HIPEC / PIPAC (intraperitoneal chemotherapy)","route":"/technologies/hipec/"}],"target":[{"id":"pdgfra","kind":"target","name":"PDGFRA","route":"/targets/pdgfra/"},{"id":"wt1","kind":"target","name":"WT1","route":"/targets/wt1/"}],"drug":[{"id":"cyclophosphamide","kind":"drug","name":"Cyclophosphamide","route":"/drugs/cyclophosphamide/"},{"id":"doxorubicin","kind":"drug","name":"Doxorubicin","route":"/drugs/doxorubicin/"},{"id":"etoposide","kind":"drug","name":"Etoposide","route":"/drugs/etoposide/"},{"id":"ifosfamide","kind":"drug","name":"Ifosfamide","route":"/drugs/ifosfamide/"},{"id":"pazopanib","kind":"drug","name":"Pazopanib","route":"/drugs/pazopanib/"},{"id":"sunitinib","kind":"drug","name":"Sunitinib","route":"/drugs/sunitinib/"},{"id":"vincristine","kind":"drug","name":"Vincristine","route":"/drugs/vincristine/"}]}}