Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive type of cancer that primarily affects children, adolescents and young adults. It is characterized by the formation of small, round, blue cells surrounded by dense fibrous tissue (desmoplasia).

DSRCT most commonly arises in the abdomen or pelvis, although it can also occur in other locations such as the chest, extremities or retroperitoneum (area behind the abdominal cavity). The tumor typically arises from the lining of the abdominal cavity (peritoneum) and may involve multiple organs within the abdomen and pelvis.

  • DSRCT primarily affects children, adolescents and young adults, with the majority of cases diagnosed in individuals between the ages of 10 and 30 years old. However, it can occur at any age.
  • The symptoms of DSRCT can vary depending on the location and extent of the tumor. Common symptoms may include:
    • Abdominal pain or discomfort
    • Abdominal distension (swelling)
    • Palpable mass in the abdomen or pelvis
    • Weight loss
    • Fatigue
    • Symptoms related to compression of nearby organs or structures, such as difficulty breathing or urinary symptoms
  • Diagnosis of DSRCT typically involves imaging studies such as CT scan or MRI to visualize the tumor and assess its size and extent. A biopsy of the tumor is then performed to confirm the presence of small round blue cells and desmoplastic stroma, which are characteristic features of DSRCT.Treatment for DSRCT usually involves a combination of surgery, chemotherapy and radiation therapy. The primary goal of treatment is to achieve complete surgical resection of the tumor whenever feasible, followed by adjuvant chemotherapy and radiation therapy to target any remaining cancer cells and reduce the risk of recurrence. DSRCT is often challenging to treat due to its aggressive nature and tendency to spread widely within the abdominal cavity.

The prognosis for DSRCT is generally poor, with a high risk of recurrence and metastasis even with aggressive treatment. 

The overall survival rate for DSRCT remains low, highlighting the need for early detection, prompt intervention and ongoing research to develop more effective treatment strategies.