Nasopharyngeal carcinoma in children and adolescent: A 10-year review in Sarawak General Hospital, Malaysia

Abstract

Background: The current standard of care for locoregionally advanced nasopharyngeal carcinoma (LANPC) is concurrent chemoradiation (CRT). Unfortunately, 20% patients with LANPC still experience distant failure after CRT. Recently published prospective clinical trials and two meta-analyses have shown that, addition of induction chemotherapy (IC) before CRT, could potentially improve oncological outcomes in comparison to CRT alone. Although it remains unclear, which is the best IC regimen to be offered and for how many cycles. Unfortunately till date, there is no published data from India, regarding the outcomes of various commonly used IC regimens before CRT, in LANPC. Methods: Patients diagnosed with LANPC from January 2012 to March 2018, who received 3 cycles IC before definitive CRT, were reviewed. The inclusion criteria were: age 18 years, pathologically proven NPC, ECOG PS 2; and adequate organ functions. Major exclusion criteria were: previous therapy for NPC and evidence of meta-static disease. Patients received IC with either paclitaxel and cisplatin (TP) or paclitaxel/docetaxel, cisplatin and 5-FU (TPF) at standard doses, every 3 weeks along with primary G-CSF prophylaxis. Results: Total 38 pts received either TP (n ¼ 20) or TPF (n ¼ 18) as IC. The median age was 36 years (range, 18-62); 66% males; WHO histological type 1/2/3, 0%/18%/82%; TNM stage III/IVA/IVB, 37%/34%/29%. The ORR after 3 cycles of TP and TPF IC were 70% and 78% respectively; and the corresponding rates were 85% and 94%, after CRT. At a median follow-up of 26 months, 2-year failure-free survival and OS for TP arm were 80% and 90%; and the corresponding rates for TPF arm were 89% and 94% respectively. All Grade III-IV toxicities were numerically higher with TPF than TP: neutropenia (33% vs 15%), febrile neutropenia (17% vs 5%), mucositis (11% vs 0%) and diarrhea (11% vs 0%). Conclusions: In this retrospective analysis, there was no significant difference between taxane-based doublet and triplet IC regimens in patients with LANPC, in terms of survival outcomes; although grade III-IV toxicities were non-significantly higher with TPF. Clearly, these hypothesis generating findings should be tested in a prospective randomized setting. Legal entity responsible for the study: Background: Nasopharyngeal carcinoma (NPC) is commonly found in Southeast Asia, Hong Kong and Southern part of China. NPC is mainly an adult malignancy, with low incidence in children. It was reported that most children with NPC presented with later stage at presentation. It has a bimodal peak distribution, and commonly presented at adolescent. However NPC is rarely encountered in children less than 10 years old. Methods: All histologically proven cases of NPC, age less than 21 years old treated from 2010-2016 in Sarawak General Hospital were identified through hospital medical record. Analysis was done based on patient's demographic, family history of first-degree relative with NPC, disease staging, treatment and their follow up. Results: There were 33 NPC cases identified, with 26 males and 7 females. Majority of patient presented at 16-20 years old (23 patients) and none below 10 years old. Most of the NPC patients are from Iban ethnicity (60.6%), followed by Malay and Bidayuh (12.1%) respectively, and Chinese (9.1%). Only 2 cases identified to have family history of NPC. About two third (66.7%) of the patients presented with stage IV disease, 6 (18.2%) stage III, 4 (12.1%) stage II and 1 (3.0%) stage I. Most stage IV patients were locally advanced, with 40.9% at stage IVa and 45.5% at Stage IVb. There is no differences noted in term of stage at presentation between the children/adolescent NPC cases with NPC in the general population of Sarawak during the same period; where 85.1% of patients presented at stage III and IV. This is still higher than Malaysia's average, where about 60-63% of patients presented with stage III and IV disease. Conclusions: Native Iban in Sarawak has the highest presentation of children/adoles-cent NPC and predominantly seen in male. Majority of patients were locally advanced at presentation. Comparison between children/adolescent and general population of Sarawak showed that they mostly present at stage III and IV in term of stage at presentation. Thus, measures to improve early detection of NPC for better cure rate is urgently needed in this high risk population.