Loading
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.
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.
How common: 0.2 to 0.5 per million a year (Journal of Cancer Epidemiology 2014).
Treatment: 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).
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).
Bone sarcomas favour the fast-growing ends of long bones (osteosarcoma) or the shaft (Ewing), soft tissue sarcomas the deep muscle compartments; spread is through the blood to the lungs, rarely via lymph nodes.
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Adamantinoma of bone, Dedifferentiated chordoma, Poorly differentiated chordoma (SMARCB1-deficient), Leiomyosarcoma, Liposarcoma, Synovial sarcoma, Sarcomas (soft tissue, bone, GIST), Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Epithelioid sarcoma, Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), Chondrosarcoma, Angiosarcoma, Undifferentiated pleomorphic sarcoma (UPS), Myxofibrosarcoma, Alveolar soft part sarcoma, Perivascular epithelioid cell tumour (PEComa), Epithelioid haemangioendothelioma, Malignant peripheral nerve sheath tumour (MPNST), Retroperitoneal sarcoma, Soft tissue sarcoma of the extremity (localised and advanced)
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.
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Query for this cancer: (TITLE:"Desmoplastic small round cell tumour" OR ABSTRACT:"Desmoplastic small round cell tumour" OR TITLE:"DSRCT" OR ABSTRACT:"DSRCT" OR TITLE:"Desmoplastic small round cell tumor" OR ABSTRACT:"Desmoplastic small round cell tumor" OR TITLE:"Intra-abdominal desmoplastic small round cell tumour" OR ABSTRACT:"Intra-abdominal desmoplastic small round cell tumour" OR TITLE:"EWSR1-WT1 sarcoma" OR ABSTRACT:"EWSR1-WT1 sarcoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Desmoplastic small round cell tumour, not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
QT: both Pazopanib and Sunitinib prolong the QT interval (known and known risk).. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Take on an empty stomach (1 hour before or 2 hours after food).
See all on the product pages:CyclophosphamideDoxorubicinEtoposideIfosfamidePazopanibSunitinibVincristine·Printable cards in the navigator
Newly diagnosed? Read the first 60 days with Desmoplastic small round cell tumour, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.