![]() However, the optimal dose remains to be determined. In NWTS 1-5, radiation therapy was given to the flank or abdomen at total doses of 1080-3500 cGy and to total doses of 1080-2100 cGy on AREN0321. However, the lack of treatment uniformity among reported patients makes it difficult to determine if radiotherapy is effective for MRT outside the CNS. Similarly, anecdotal reports suggest a benefit from the use of radiotherapy as part of multimodal therapy for MRT. In Europe, the registry study (Eu-Rhab) for all rhabdoid tumors (AT/RT, RTK, and MRT) recommends using combination therapy including surgery, radiotherapy, and chemotherapy with permissive use of high-dose chemotherapy with stem cell rescue. This study showed an overall 2-year progression-free survival of 42% in the entire cohort and of 39% for those < 3 years of age. Considering all data, the recently closed COG AT/RT protocol ACNS0333 used a regimen incorporating surgery, conventional chemotherapy, age- and stage-directed radiation, and three cycles of high-dose chemotherapy with stem cell rescue. Additionally, there are reports that describe the successful use of high-dose chemotherapy with stem cell rescue to treat non-CNS MRT. This later study included infants younger than 3 years of age. Subsequently, the Dana Farber Consortium AT/RT study, which was a multisite study of multimodal therapy incorporating surgery, age- and stage-directed radiation, and a systemic and intrathecal conventional chemotherapeutic regimen based on a modified IRS-III regimen, demonstrated a 2-year progression-free survival of 53%. ![]() Jude Children’s Research Hospital from 1984-2003 showed that AT/RT presenting in patients older than 3 demonstrated a 2-year event-free survival of 78% when treated with a combination of radiation and high-dose alkylating therapy. These treatments have improved the survival rates of patients with AT/RT from historical controls.Ī review by Tekautz et al of children treated at St. Current treatment involves a multimodal approach with a combination of surgery, radiation therapy, and various chemotherapy regimens (systemic and intrathecal) that typically included cisplatin, etoposide, vincristine, ifosfamide, doxorubicin, actinomycin, cyclophosphamide, methotrexate, and intrathecal agents with or without autologous stem-cell rescue. Like its extra-CNS counterparts, AT/RT results in an unfavorable prognosis and is characterized by resistance to chemotherapy. Insights into the treatment of MRT may be derived from the experience with atypical teratoid/rhabdoid tumors (AT/RT) of the CNS. There was a trend toward improved outcomes for low stage patients (100% 4-year EFS for 2 patients with Stage 1 disease and 80% for 5 patients with Stage 2 disease), but continued dismal outcomes for those with higher stage disease. The 4-year event-free survival (EFS) and overall survival (OS) estimates for the entire cohort of patients were 23.1% and 33.33%, respectively, with a median time to progression of 2.7 months. On the basis of these reports, the most recent COG study AREN0321, regimen UH-1, used cyclophosphamide-carboplatin-etoposide (CCE) alternating with VDC. Subsequent case reports documented successful outcomes in patients with metastatic MRT treated with ifosfamide-carboplatin-etoposide (ICE) or ifosfamide-etoposide (IE) alternating with vincristine-doxorubicin-cyclophosphamide (VDC). However, this strategy, did not improve outcomes. To try to improve these results, investigators in NWTS-5 used a regimen consisting of carboplatin-etoposide alternating with cyclophosphamide. With these agents, the estimated survival rate for patients with MRT was only 23%. Chemotherapy for MRT was historically based on therapy for a Wilms tumor, which included vincristine, actinomycin, and doxorubicin with or without cyclophosphamide. After the primary tumor is surgically removed, chemotherapy and radiotherapy are indicated as adjuvant treatment for malignant rhabdoid tumor (MRT).
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