Desmoplastic Small Round Cell Tumor

Definition

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

Added:January 10, 2025

Updated:January 10, 2025

Overview

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive form of cancer that primarily affects adolescents and young adults. It is most commonly found in the abdomen, but can also occur in other parts of the body. DSRCT is characterized by the presence of small, round cancer cells surrounded by dense fibrous tissue, which gives it its name.

Key Information

DSRCT is an aggressive tumor that is challenging to diagnose and treat. It is more common in males than females and often presents with nonspecific symptoms such as abdominal pain, a palpable mass, or gastrointestinal disturbances. The tumor is associated with a specific chromosomal translocation, t(11;22)(p13;q12), which results in the EWS-WT1 gene fusion, a hallmark of this cancer.

Clinical Significance

DSRCT is clinically significant due to its aggressive nature and poor prognosis. Early diagnosis is challenging as symptoms are often vague and can be mistaken for other conditions. The tumor's tendency to spread within the abdominal cavity and to other organs can complicate treatment and worsen outcomes.

Treatment & Management

Treatment for DSRCT typically involves a combination of surgery, chemotherapy, and radiation therapy. Surgical removal of the tumor is often attempted, but complete resection can be difficult due to the tumor's location and spread. Chemotherapy regimens may include agents such as vincristine, doxorubicin, and cyclophosphamide, among others. Radiation therapy may be used to target residual disease or in cases where surgery is not feasible. Clinical trials and newer targeted therapies are also being explored.

Patient Resources

Patients diagnosed with DSRCT and their families can access a variety of resources for support and information. Organizations such as the American Cancer Society and Cancer.net provide educational materials, support groups, and guidance on managing the disease. It is important for patients to discuss all available treatment options with their healthcare team and consider seeking a second opinion from a specialist experienced in treating rare cancers.

Frequently Asked Questions

What are the symptoms of DSRCT?

Symptoms often include abdominal pain, a palpable mass, and gastrointestinal issues. Due to its rarity, symptoms can be mistaken for other conditions.

How is DSRCT diagnosed?

Diagnosis typically involves imaging studies such as CT or MRI scans, followed by a biopsy to confirm the presence of the tumor and the characteristic genetic mutation.

What is the prognosis for DSRCT?

The prognosis for DSRCT is generally poor due to its aggressive nature and tendency to spread. Early detection and comprehensive treatment can improve outcomes.

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