What is DSRCT?
Desmoplastic Small Round Cell Tumour (DSRCT) is a very rare and aggressive type of soft tissue cancer that most often arises in the abdomen or pelvis. It typically affects older children, adolescents and young adults, and is more common in males.
DSRCT is a type of sarcoma made up of small round tumour cells surrounded by dense fibrous (desmoplastic) tissue. Diagnosis is confirmed using specialised pathology testing, including the characteristic *EWSR1–WT1* gene fusion.
Epidemiology
DSRCT is one of the rarest solid tumours. Key points include:
- Incidence: It is extremely rare, with an estimated incidence of around 0.2–0.5 cases per million people per year.
- Age distribution: Most commonly diagnosed in teenagers and young adults, typically between 10 and 30 years of age. It can occur in children as young as 5 and in adults up to their 40s, but this is unusual.
- Sex distribution: There is a strong male predominance. Approximately 80–90% of patients are male.
- Ethnicity: Some studies suggest higher rates in individuals of European ancestry, although data is limited.
- Primary site: In more than 85% of cases, DSRCT arises within the abdomen or pelvis, often presenting with multiple tumour deposits. Less commonly, it can arise in the chest, paratesticular region or other soft tissue sites.
- Stage at diagnosis: Because symptoms are often vague, most patients present with advanced or widespread disease at diagnosis.
- Cause: No environmental, familial or inherited risk factors have been identified. The disease is defined by a specific, random genetic fusion (*EWSR1–WT1*), but this cannot be passed on or inherited.
Symptoms and Presentation
Symptoms can be non-specific and may include:
- Abdominal pain or discomfort
- Abdominal swelling or bloating
- Feeling full quickly or reduced appetite
- Unintentional weight loss
- Changes in bowel habits or bowel obstruction
- Fatigue or general unwellness
Because the tumour often spreads widely across the lining of the abdomen, many patients present late.
How is DSRCT diagnosed?
Imaging
Diagnosis usually begins with imaging such as:
- Ultrasound
- CT or MRI to map tumour size and spread
- PET-CT to identify active tumour and detect disease outside the abdomen
Biopsy
Confirmation requires a tissue sample obtained by:
- Image-guided needle biopsy
- Laparoscopic biopsy
Pathology testing includes immunohistochemistry and molecular studies to identify the *EWSR1–WT1* fusion. All cases should be discussed in a multidisciplinary sarcoma meeting.
Treatment Options
Treatment for DSRCT is complex and involves multiple therapies. Plans are personalised based on disease extent, tumour behaviour and patient factors.
1. Chemotherapy
Most patients receive intensive multi-agent chemotherapy as the first treatment to shrink tumour deposits and manage disease throughout the body.
2. Surgery (Cytoreductive Surgery / Peritonectomy)
If the tumour responds to chemotherapy, major surgery may be possible. This may include:
- Cytoreductive surgery – removal of all visible tumour throughout the abdomen
- Peritonectomy – removal of peritoneal surfaces affected by tumour
These operations are highly specialised and are performed only in centres with expertise in peritoneal surface malignancies and sarcoma surgery.
3. HIPEC (Heated Intraperitoneal Chemotherapy)
In selected cases, surgery is combined with HIPEC:
- A heated chemotherapy solution is circulated inside the abdomen after tumour removal
- This targets microscopic cancer deposits that cannot be seen or safely removed
HIPEC is not appropriate for all patients and decisions are made individually.
4. Radiotherapy
Radiotherapy may be used:
- After surgery to reduce risk of tumour returning
- For symptom control if surgery is not possible
5. Clinical Trials
Because DSRCT is rare, clinical trials are a key part of treatment development. Options may include:
- New chemotherapy combinations
- Targeted therapies
- Immunotherapy
- Novel agents or treatment sequences
6. Supportive and Palliative Care
Supportive care—including nutrition support, pain management and psychological support—is essential throughout treatment. In advanced cases, palliative care focuses on comfort and quality of life.
Training and Expertise Required for DSRCT Surgery
Surgery for DSRCT—especially cytoreductive surgery, peritonectomy and HIPEC—is among the most complex procedures in cancer surgery. Surgeons performing these operations require:
- Specialist training in surgical oncology
- Expertise in sarcoma surgery
- Fellowship training in high-volume peritonectomy/HIPEC centres
- Regular multidisciplinary team involvement
- Access to specialised anaesthesia, critical care and oncology services
Only a small number of centres and surgeons have the expertise to safely perform these procedures.
Dr Sandler trained MD Anderson Cancer Center in Houston, Texas in 2017 and 2018, specifically to treat DSRCT. He has started a DSRCT treatment program at Westmead Hospital along with specialist medical and radiation oncologists.
Your First Appointment
At the initial consultation, patients can generally expect:
- Review of all scans, pathology and medical history
- Clear explanation of the diagnosis
- Discussion of whether further tests are required
- Overview of treatment options, including chemotherapy, surgery, HIPEC and radiotherapy
- Time for questions and involvement of family or support people
It can be helpful to bring a support person and a list of questions.
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