# Desmoplastic small round cell tumour

Source: https://onco.cc/cancers/desmoplastic-small-round-cell-tumour/  
OnCo record `desmoplastic-small-round-cell-tumour` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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).

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).

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: DSRCT; Desmoplastic small round cell tumor; Intra-abdominal desmoplastic small round cell tumour; EWSR1-WT1 sarcoma
- Tags: subtype-page; wave4; rare
- 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)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/desmoplastic-small-round-cell-tumour/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/desmoplastic-small-round-cell-tumour/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/desmoplastic-small-round-cell-tumour/#what-it-is [3 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/desmoplastic-small-round-cell-tumour/#finding-it [4 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/desmoplastic-small-round-cell-tumour/#treating-it [1 setting, 1 decision with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/desmoplastic-small-round-cell-tumour/#evidence
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/desmoplastic-small-round-cell-tumour/#science [5 targets]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/desmoplastic-small-round-cell-tumour/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/desmoplastic-small-round-cell-tumour/#living-with-it [8 questions, 6 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/desmoplastic-small-round-cell-tumour/coming/ [7 medicines]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/desmoplastic-small-round-cell-tumour/data/ [25 connected records]

## Standard of care

- 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. ([Sarcomas (soft tissue, bone, GIST)](https://onco.cc/cancers/sarcoma/), [Vincristine](https://onco.cc/drugs/vincristine/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [Etoposide](https://onco.cc/drugs/etoposide/), [HIPEC / PIPAC (intraperitoneal chemotherapy)](https://onco.cc/technologies/hipec/), [Pazopanib](https://onco.cc/drugs/pazopanib/), [Sunitinib](https://onco.cc/drugs/sunitinib/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Desmoplastic_small-round-cell_tumor
- NCI PDQ: childhood soft tissue sarcoma treatment: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq
- Journal of Cancer Epidemiology 2014: incidence and outcomes of desmoplastic small round cell tumour, SEER: https://doi.org/10.1155/2014/680126
- Cancers 2021: desmoplastic small round cell tumour, molecular abnormalities and emerging therapy (review): https://doi.org/10.3390/cancers13030498

## Connected records

- cancers: [Adamantinoma of bone](https://onco.cc/cancers/adamantinoma/), [Childhood cancers (all types)](https://onco.cc/cancers/childhood-cancers/), [Ewing sarcoma](https://onco.cc/cancers/ewing-sarcoma/), [Peritoneal mesothelioma](https://onco.cc/cancers/peritoneal-mesothelioma/), [Rhabdomyosarcoma](https://onco.cc/cancers/rhabdomyosarcoma/), [Sarcomas (soft tissue, bone, GIST)](https://onco.cc/cancers/sarcoma/)
- technologies: [HIPEC / PIPAC (intraperitoneal chemotherapy)](https://onco.cc/technologies/hipec/)
- targets: [PDGFRA](https://onco.cc/targets/pdgfra/), [WT1](https://onco.cc/targets/wt1/)
- drugs: [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [Pazopanib](https://onco.cc/drugs/pazopanib/), [Sunitinib](https://onco.cc/drugs/sunitinib/), [Vincristine](https://onco.cc/drugs/vincristine/)

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JSON: https://onco.cc/api/v1/entities/desmoplastic-small-round-cell-tumour.json