DSRCT

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Category: DSRCT - Includes table of contents for site. Patients and caregivers are welcome to leave comments or make changes, just make sure to leave a note in the Discussion tab. Thanks!

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Introduction
DSRCT is a rare childhood cancer with few known Treatment Options. This Wiki is intended to gather together information from a variety of patients and create a database.

Desmoplastic small round cell tumor is a highly aggressive tumor that frequently occurs as multiple masses in the abdomen. DSRCT is classified as a soft tissue sarcoma that predominately affects boys and young adults. DSRCT in females is extremely rare but has been identified in uterine and ovarian cancers.

The cell of origin for this primitive blue cell tumor is unclear. The tumor shows characteristics of epithelial, mesenchymal and neural differentiation. However, a characteristic translocation between chromosome 11 and 22 has been associated with desmoplastic small round cell tumors involving the EWS (Ewings Sarcoma) and (Wilms Tumor) WT1 genes. This gene abnormality has been useful in confirming diagnosis for the tumor.

Because of the tumors rarity few physcians have seen or treated this disease. There is no standard protocol for treating DSRCT, though the Kushner's P6 protocol and variations have the most support. It is imperative to maintain control over the disease. Patients report that without treatment that the tumors can double and triple in size in 28-40 days (evidence in the WIKI supports this).

Oncologists who have had previous experience with DSRCT frequently recommend the following course of treatment (but there are many variations according to the patients stage of disease and overall health). Depending upon stage of cancer, treatment can last from 8 to 16 months of intensive chemotherapy, surgery, stem cell, and radiation. It is critical to remain as healthy as possible between treatments and critical to stay on a tight schedule. The disease is fast growing and spreds rapidly so the treatment must be aggressive:


 * First Line - High Dose Chemotherapy - P6
 * Debulking Surgery 1
 * Continued High Dose Chemotherapy
 * Second Surgery, etc (if warranted)
 * Stem Cell rescue/transplant
 * Radiation
 * Second Line Chemotherapy
 * Clinical Trials
 * Maintenance Drugs (optional)
 * CT/PET/MRI every 8 or 12 weeks to monitor disease.
 * Please note that 'No evidenence of disease' is the term used when an oncologist/radiologist no longer can see/spot disease on a CT/PET/MRI. It could still be there in microscopic or non-visible areas.
 * Disease relapse can occur at any point due to the aggressiveness of DSRCT.
 * Maintain local control of disease.
 * Complications include: colostomy, allergies, staph and blood infections, central line infection, fungus infections, neutropenia, radiation burns, graft vs host rejection.
 * Further Complications: Developing a secondary cancer such as leukemia.

Current Treatment Options include P6 Protocol established by Memorial Sloan-Kettering Cancer Center, variations of the P6 protocol, surgery, radiation, stem cell rescue, hyperthermic chemoperfusion, radioablation, and maintenance drugs.

This disease is also known as: Desmoplastic small round blue cell tumor; Intraabdominal desmoplastic small round blue cell tumor; Desmoplastic small cell tumor; Desmoplastic sarcoma; DSRCT.

DSRCT has recently been recognized as a rare disease and is entered into the US governments database: National Institue of Health Office of Rare Diseases

'Disclaimer: This site is for informational and idea exchange among patients only. We are not doctors and do not offer medical advice. Please check with your own physician if you have any unusual symptoms or questions concerning treatment.'

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