These results are consistent with the observation that there was no dif ference

Heated cisplatin is given at a dose 100 to 150 mg m2 intraperitoneally after optimal cytoreductive surgery. The series reported by the MDACC showed that this method is safe and might have activity in paediatric patients, and a survival benefit has also been reported. [You must be registered and logged in to see this link.] Further studies are required before this could be widely adopted. Radiotherapy Whole abdominopelvic radiotherapy after max imal surgery was first reported by Kushner et al. at the MSKCC as part of a multimodality treatment using the P6 protocol, with the aim of improving local control. A total dose of 30 Gy was delivered post operatively, with simultaneous boost given to sites of gross residual disease. Conventional two dimensional radiotherapy was associated with significant gastrointestinal and haemato logic toxicities, with long term side effects including small bowel obstruction and ureteral stenosis.

For this reason, the use of WAP intensity modulated radiation therapy was studied by Pinnix et al. at the MDACC. All of the eight patients had received prior chemotherapy and surgical debulking. One patient was still disease free 20 months after treatment, although the rest experienced either local or distant failure after a median [You must be registered and logged in to see this link.] of 8. 73 months from WAP IMRT. A retrospective analysis at the MSKCC looked at 31 patients who under went WAP RT, either with conventional two dimensional radiotherapy or IMRT after chemotherapy and maximal debulking surgery. IMRT was associated with lower incidence of acute gastrointestinal and haematologic toxicities. The 3 year overall survival and progression free survival rates were 50% and 24%, respectively.

Anecdotally, a patient in this series who received WAP RT developed a serious malabsorption syndrome subsequent to gastrointestinal damage. [You must be registered and logged in to see this link.] Given the limited data of efficacy, WAP RT is currently not routinely used in the management of DSRCT. Targeted therapies In recent years, targeted therapies have been studied in DSRCT. Drugs that have shown activity against this disease include the TKI sunitinib and the mTOR in hibitor temsirolimus. In our cohort of patients, other non standard agents used include the anti IGF 1R antibody figitumumab, the TKIs axitinib, pazopanib, sorafenib and sunitinib, as well as the mTOR inhibitor sirolimus. The number of patients is too small to draw any conclusion about their efficacy.

Due to the fact that DSRCT has a predilection to occur in young males, Fine et al. discovered that androgen receptor is expressed in 37% of DSRCT. Six of their patients were treated with combined androgen blockade and three attained a clinical benefit. In our study, one patient had received the gonadotropin releasing hormone agonist goserelin. However, no significant anti tumoural efficacy was noted. Chromosomal translocation resulting in the fusion of the EWSR1 and WT1 genes is the molecular characteristic of DSRCT. The resulting fusion protein has been found to activate the IGF 1R gene promoter, causing the expression of this anti apoptotic receptor tyrosine kinase. The understanding of this mechanism has provided a novel target for the treatment of this disease.