Results of multimodal treatment for desmoplastic small round cell tumors

Feasibility of Administering A Modified Outpatient Regimen of Vincristine, Doxorubicin, Cyclophosphamide Alternating with Ifosfamide, and Etoposide (VAC/IE) For High-Risk Desmoplastic Small Round Blue Cell Tumor and the Role of Tumor Profiling in Selecting Treatments

“…Median survival ranges from 14 to 25 years [14] with some reports with patients as young as 5 months [33]. 3-year overall survival is dismal at around 44% and the 5-year survival rate a dismal 15% [8] with a slightly higher survival reported among those undergoing debulking including 1-2 mm implants [34] of at least 90% of tumor absence of extraperitoneal metastasis [32]. Radiotherapy to high-risk sites, and myeloablative chemotherapy with stem-cell rescue is applied in selected cases especially those with residual disease [31].…”

“…Aggressive surgical debulking with the goal to remove >90% of tumor [8] is the cornerstone of any curative intent strategy.…”

“…Without consolidative therapy with either radiation or myeloablative treatment [7][8][9][10]36] in most of the studies and relapses occur early without consolidation DSRCT is alkylator-sensitive and dose-responsive. Doxorubicin is a common denominator in the treatment of patients who either achieved long-term survival or had responsive tumors [36,38].…”

“…Genomic and Molecular assessment may help determine the ideal regimen that will help achieve maximal tumor debulking which is crucial to survival [8,9] since large abdominal mass is a usual presentation is aggressive and occurs in adolescents and young adults [8,10].…”

Diagnosis of desmoplastic small‐round‐cell tumor by cytogenetic analysis: a case report

“…This regimen has a risk of toxicity that requires intensive transfusions and antibiotic support 4, 18. Complete surgical resection is also associated with improved survival.…”