Desmoplastic Small Round Cell Tumor (DSRCT) has a very poor prognosis. This report illustrates novel
chemotherapy and local control interventions in a 5-year old patient. The patient was treated in the outpatient setting, achieved remission, with excellent quality of life. The patient presented with massive
ascites and >1000 abdominal
tumors.
Neoadjuvant chemotherapy included
vincristine (1.5 mg/m(2)),
ifosfamide (3 g/m(2)/day x 3),
dexrazoxane/
doxorubicin (750/75 mg/m(2)), and
etoposide (150 mg/m(2)). Continuous hyperthermic peritoneal perfusion (CHPP) with
cisplatin (100 mg/m(2)) was given after extensive
cytoreductive surgery. This was followed by
irinotecan (10 mg/m(2)/day x 5 x 2 weeks) +
temozolomide monthly x 2, then abdominal radiation 30 Gy with simultaneous
temozolomide (100 mg/m(2)/day x 5). A total of 12 cycles of
irinotecan and temozolamide were given. Except for initial
chemotherapy, subsequent courses were in the outpatient setting. Focal retroperitoneal relapse at 18 months was treated with IMRT with
bevacizumab (5 mg/kg) and 2 perihepatic
metastases with
radio frequency ablation/
cryoablation followed by chronic outpatient
maintenance chemotherapy (
valproic acid,
cyclophosphamide, and
rapamycin). Almost 2 years from diagnosis, the patient maintained an excellent quality of life. This is a novel approach to the treatment of children with massive abdomino-pelvic DSRCT.