Multimodality management of nasopharyngeal carcinoma in paediatric and adolescent patients: updated experience from a regional cancer centre in North India

Abstract

Background and Aims: Nasopharyngeal carcinoma(NPC) is a rare malignant tumour in childhood and adolescence(<1% of paediatric malignancies). Methods: Data pertaining to paediatric and adolescent patients with NPC attending our institute from 2014-21 was abstracted by retrospective chart review. Results: We identified 66 patients with NPC. The median age at presentation was 18 years(range 8-27years). The male to female ratio was 43:23. On clinicoradiological examination 1(1.5%),14(21.2%),11(16.7%),33(50%) and 7(10.6%) patients had AJCC 2010 stage II,III,IVA,IVB and IVC tumours respectively. Majority(63.6%) of the patients had undifferentiated NPC. On immunohistochemistry, 53/59(89.8%) tumours stained positive for EBV-LMP1. 65 patients underwent neoadjuvant chemotherapy(median-3 cycles)- PF(Cisplatin,5fluorouracil) in 19(29.2%), TP(Paclitaxel,Carboplatin) in 31(47.7%), GC(Gemcitabine,Cisplatin) in 12(18.5%) and TPF(Docetaxel,Cisplatin,5fluorouracil) in 3(4.6%). Radiotherapy(RT) to locoregional disease was delivered in 65(98.5%) patients, the techniques being 2D-conventional in 10, fixed-field IMRT in 5 and VMAT in 50 patients. The median RT dose was 65,60,54Gy/30 fractions/6 weeks(simultaneous integrated boost). Weekly concurrent Cisplatin(median-5 cycles) was added in 64(97%) patients. After a median follow-up of 21 months(mean-27.3 months), 27(40.9%) patients had disease progression(PD) and 14(21.2%) patients died(12 due to PD). The common patterns of failure were in the bones(22),lungs(10), distant lymph nodes(10), liver(4), locoregional(3) and local(1). At last follow-up, 31(47%) and 8(12.1%) patients had complete and partial response, 2(3.03%) and 25(37.9%) patients had stable and progressive disease respectively. The median overall survival(OS) and progression free survival(PFS) had not been reached. The actuarial rates of OS and PFS were 82.6% and 56.4% at 2 years and 75% and 50.4% at 3 years respectively. Conclusions: Majority of paediatric and adolescent patients with NPC present in advanced stage in developing countries. Multimodality management (NACT followed by CRT) led to modest clinical outcome in our setting. The most common pattern of failure was distant metastases in the bones. Attention needs to focussed on early detection of NPC, prompt treatment(timely conformal RT) and overcoming barriers like treatment abandonment.