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Familial Nasopharyngeal Carcinoma 6

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<strong>Nasopharyngeal</strong> Cancer in Pediatric and Adolescent Patients 303Table 23.2. Chemotherapy combinations for children andadolescents with NPC aMPFPFBECOr BECPMBMethotrexate: 120 mg/m 2 IVon day 1Cisplatin: 100 mg/m 2 /6 h. IVinfusion on day 25FU: 1,000 mg/m 2 /24 h. IVinfusion on days 1–3 or 5Cisplatin: 100 mg/m 2 /4 h. IVinfusion on day 1Or 20 mg/m 2 /1 h. IV infusionon days 1–55-FU: 1,000 mg/m 2 /24 h. IVinfusion on days 1–5Bleomycin: 15 mg/m 2 IVbolus dose on day 1, and12 mg/m 2 /24 h. IV infusionon days 1–5Epirubicin: 70 mg/m 2 /1 h. IVinfusion on day 1Cisplatin: 100 mg/m 2 /4 h. IVinfusion on day 5Variants with reduced dosesCisplatin: 20 mg/m 2 /1 h. IVinfusion on days 1–5Methotrexate: 50 mg/m 2 IVon days 1, 8, 15Bleomycin: 20U/m 2 IV ondays 1, 8, 15aChemotherapy combinations are given with 3-weekly intervalsfor two to four courses before radiation18 children (Ayan et al. 2000). An updated and combined36 children and 37 adolescents data revealedrapid and high response rates both in the primarytumor and the involved neck nods with the same threecycles combination of bleomycin, epirubicin, and cisplatin.The treatment outcome was relatively high bothfor children and adolescents, with 5- and 10-years OSrates of 86.5% and 71.5% respectively (Altun et al.2008). The same combination with reduced doses ofall the three drugs was used in 22 children (Sahraouiet al. 1999). Although the response rates to preradiatonchemotherapy was not indicated in the study, the5-year OS rate of patients who received chemotherapywas 52% and was significantly better than the 38% OSrate of radiation-only patient population (p < 0.001).Laskar et al. reported 42% CR and 44% PR rates followingtwo cycles of preradiation bleomycin, 5-FU,and cisplatin combination in 57 patients of age lessthan 18 years. Further analyses on prognosis revealedthat patients with CR had signifi cantly better OS (86%vs. 61% vs. 16%, p 0.0001) and DFS (69% vs. 36% vs.25%, p 0.0001) than those with PR and no response(NR) (Laskar et al. 2004).A similar result regarding the prognostic significanceof induction response was reported by Kupeliet al. In this retrospective series, 65 of 84 patients lessthan 17 years of age were given preradiation chemotherapy.According to the time period, noncisplatin andcisplatin-based combinations were used. The assessmentfollowing induction revealed 48.8% CR and 33.3%PR for the entire group; the OS rates (68% vs. 0%, p =0.0003) were significantly better in complete responderswhen compared with NR patients. Although theinduction combinations were heterogeneous in thispediatric series, it was demonstrated that the cisplatinbasedcombinations resulted in a significantly betterOS (80% vs. 63.4% vs. 30.9%, p = 0.001) than noncisplatinregimens and post-RT cyclophasphamide monotherapy,respectively (Kupeli et al. 2005).In the GPOH Study NPC-91, where three cycles ofpreradiation methotrexate, cisplatin, and 5-FU havebeen used, 56 of 58 high-risk patients had good clinicaltumor response (14% CR, 86% PR) (Mertenset al. 2005). In POG study 9486, 16 patients receivedfour courses of methotrexate, 5-FU, and cisplatin. Anoverall response rate of 93.75% with 5 CR (31.25%)and 10 PR (62.5%) were achieved before radiation(Rodriquez-Galindo et al. 2005). Recently, the efficacyof preradiation docetaxel and cisplatin combinationwas investigated in newly diagnosed pediatricand young adult NPC patients. Ten patients receivedfour cycles of cisplatin of 100 mg/m 2 and docetaxel of75 mg/m 2 on day 1 with 3 weekly intervals, followedby 59.4 Gy median dose of radiotherapy. The inductionchemotherapy combination yielded 1 CR, 5 PR,3 stable, and 1 progressive disease. No major chemotherapytoxicity was reported, and the 2-year OS andEFS was 90% and 70%, respectively. It was concludedthat cisplatin and docetaxel combination is safe andefficacious for NPC (Varan et al. 2009). A RCN studyevaluated 165 nonmetastatic NPC patients

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