Nasopharyngeal Carcinoma in Children and Adolescents: The European PARTN-ER Project for Consensus Guidelines Development.

Background and Aims: Nasopharyngeal carcinoma (NPC) is a rare pediatric tumor. Several collaborative studies performed over the last decades showed improved results compared to historical data, but standardized guidelines for diagnosis and management of pediatric NPC are still unavailable. Methods: We present European consensus guidelines developed by the Cooperative Study group for Pediatric Rare Tumors (EXPeRT) according to the ESMO scale, based on the evidence collected from published series, case reports and personal expertise. Results: Induction chemotherapy is recommended to decrease tumor volume, reduce microscopic distant tumor spread and select patients with very good response who could benefit from radiation deescalation [Level III; Grade A]. It could include 3 cycles of cisplatin and 5-flurouracil [Level III; Grade B]. Tumor response should be assessed (MRI± PET-CT) after the third cycle [Level III; Grade B]. Concomitant chemo-radiotherapy to sensitize the tumor to irradiation, especially for high risk tumors (poor response to initial chemotherapy or metastatic tumors) taking into account the risk of delay in radiotherapy is recommended [Level I (adult studies), III (pediatric); Grade C]. It includes cisplatin either in 21 days cycles or weekly schedules [Level III; Grade B]. Maintenance treatmentwith IFN-β is an option, especially for high risk situations [Level III; Grade C]. Intensity modulated radiotherapy is recommended as a standard of care [Level II (adult), IV (pediatric); Grade A]. Adapted dosages protocols with de-escalation after favorable tumor response following induction chemotherapy is recommended [Level III; Grade A]. Optimal dose of radiotherapy in NPC has still to be defined (from 45 to 59.6Gy PTV1; 54 to 66.6Gy PTV2 on primary and involved nodes) depending on tumor response and 45Gy for PTV-N0 with 1.8 Gy daily fractions [Level III; Grade B]. Conclusions: This European PARTN-ER project leads to a consensus strategy based on international experiences regarding the treatment of young patients with NPC.