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Desmoplastic Small Round Cell Tumor
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.
Because of the rarity of this disease it is essential that all desmoplastic small round cell patients be treated at Sarcoma Centers or by a sarcoma specialist. Pediatric patients should if at all possible be treated at a Childrens Hospital with a sarcoma program. Adults should ask for a referral to a center that specializes in sarcoma.The cell of origin for desmoplastic small round 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 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). Disease relapse is common.
However, according to emedicine reports a "combination of aggressive surgical debulking and systemic chemotherapy with cyclophosphamide, doxorubicin, and vincristine interspersed with ifosfamide, etoposide, and mesna (P6 protocol) appears to lead to an improved outcome in patients with desmoplastic small cell tumors."
Oncologists who have had previous experience with DSRCT will sometimes 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 spreads rapidly so the treatment must be aggressive:
- Second Line Chemotherapy
- Clinical Trials - Phase I and II Clinical Trials give the chance of unique medicines never tried before. The down side is no one knows if they work. Not many trials specifically for DSRCT but you may be able to find one for soft tissue sarcoma, solid tumors, or Ewing's sarcoma.
- 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. Biopsy may be the only way to tell if there has been a relapse, but this is rarely recommended.
- Disease relapse can occur at any point due to the aggressiveness of DSRCT.
- Maintain local control of disease.
- Complications from the disease or treatment include: ascites, blood clots, low thyroid, hormone deficiency, intestinal blockage, colostomy, allergies, staph and blood infections, diarrhea, constipation, mucositis , central line infection, fungus infections, neutropenia, shortness of breath, neurological, nerve damage, gall bladder, spleen, diaphragm, edema, radiation burns, graft vs host rejection.
- Further Complications: Chemobrain, learning disabilities. Developing MDS or a secondary cancer such as leukemia.
- Once there is no evidence of disease. Request a full body PET scan to establish a baseline to be used in case of relapse. This also will serve the purpose of making sure that no disease remains.
- Maintenance Drugs. Some patients are trying alternative/complementary treatments to maintain disease free status, some patients are using daily Vioxx, Celebrex, or VP-16. Some have tried Irinotecan and Temodar.
- Follow up CT or PET scans should be done every two or three months for two or more years. After that if no evidence of disease than CT or PET every six months for two years. After that, go to one year . . preferably a PET scan or combo CT/PET.
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, radio frequency ablation, cyberknife, maintenance drugs, targeted chemotherapy, and clinical trials.
Desmoplastic small round cell tumor can metastasize to the liver, lungs, diaphragm, spleen, lymph nodes, skull, bones, spinal cord, and brain.
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
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DSRCT References includes links, bibliography, cites, and acknowledgements for all information found on the DSRCT: Wiki.
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.