Overview

Desmoplastic Small Round Cell Tumor (DSRCT) is a rare, aggressive soft-tissue sarcoma that usually arises in the abdomen or pelvis, primarily affecting children, adolescents, and young adults. It belongs to the “small round blue cell tumor” group and is known for its rapid growth and tendency to spread within the abdominal cavity.

Because DSRCT is uncommon, treatment requires a specialized multidisciplinary team and advanced cancer care technologies.

At I D Cancer Center, we combine precision radiotherapy, modern chemotherapy protocols, and supportive care to improve outcomes and quality of life.

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What Makes DSRCT Unique?

• Originates from primitive stem-like cells

• Often presents as multiple tumors in the abdominal cavity

• Known for extensive desmoplastic (fibrous) tissue reaction

• Characterized by a specific EWS–WT1 gene fusion

• Requires aggressive multimodal treatment

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Common Signs & Symptoms

Symptoms may be vague in early stages:

• Abdominal pain or discomfort

• Abdominal swelling or fullness

• Changes in bowel habits

• Unexplained weight loss

• Fatigue

• Nausea or vomiting

• Loss of appetite

If tumors spread, patients may experience:

• Urinary symptoms

• Bloating

• Fluid in abdomen (ascites)

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Risk Factors

Because DSRCT is a rare disease, exact causes are unknown. Most cases are sporadic, and no lifestyle, hereditary, or environmental factors have been clearly associated.

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Diagnosis at I D Cancer Center

Accurate diagnosis requires a combination of imaging, biopsy, and molecular tests:

1. Imaging Studies

• CT scan of abdomen & pelvis

• MRI for soft tissue evaluation

• PET-CT for staging and metastasis

2. Biopsy & Histopathology

• Core needle biopsy

• Immunohistochemistry (IHC) showing:
  Desmin+, Cytokeratin+, WT1+

3. Genetic / Molecular Testing

• Detection of EWSR1–WT1 fusion gene confirms diagnosis

Each case is reviewed in our Tumor Board Meeting for a tailored treatment plan.

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Treatment Options

Because DSRCT is aggressive, treatment usually involves multiple approaches.

1. Chemotherapy

Intensive chemotherapy regimens are the cornerstone of treatment:

✔ Multi-drug protocols (e.g., P6 protocol)

✔ Helps shrink tumors

✔ Controls disease spread

2. Surgery

Large debulking surgery may be recommended in selected cases to remove as much tumor as possible.

3. Radiation Therapy

At I D Cancer Center, we provide advanced radiotherapy techniques:

• IMRT / IGRT / VMAT

• High-precision abdominal radiotherapy

• Postoperative/adjuvant radiation

• Palliative radiotherapy for symptom relief

Benefits:

✔ Organ-sparing

✔ Better toxicity control

✔ Effective for residual disease

4. Targeted Therapy & Immunotherapy

Used in recurrent or metastatic disease:

• mTOR inhibitors

• Anti-angiogenic drugs

• Clinical trial–based immunotherapies

5. Supportive & Palliative Care

We prioritize symptom management and quality of life:

• Pain management

• Nutritional guidance

• Psychological support

• Physiotherapy & rehabilitation

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Prognosis

Because DSRCT is rare and aggressive, early diagnosis and timely multimodal therapy improve outcomes.

Our focus remains on:

• Maximizing survival

• Preserving function

• Enhancing quality of life

• Offering continuous support to patients and families