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  Table of Contents  
ORIGINAL ARTICLE
Year : 2016  |  Volume : 53  |  Issue : 3  |  Page : 377-380
 

Pediatric nasopharyngeal carcinoma: Experience from a tertiary cancer center in India


1 Department of Medical and Pediatric Oncology, Cancer Institute (W.I.A.), Chennai, Tamil Nadu, India
2 Department of Radiotherapy, Cancer Institute (W.I.A.), Chennai, Tamil Nadu, India

Date of Web Publication24-Feb-2017

Correspondence Address:
V Radhakrishnan
Department of Medical and Pediatric Oncology, Cancer Institute (W.I.A.), Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-509X.200663

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 » Abstract 

BACKGROUND: Pediatric nasopharyngeal carcinomas (NPCs) are rare tumors. There is paucity of data on outcomes in pediatric NPC from developing countries. AIM: The present study was conducted to ascertain the outcomes of children with NPC at our center. SETTINGS AND DESIGN: A retrospective analysis of case records of pediatric NPC patients treated at our hospital was performed. PATIENTS AND METHODS: We analyzed the outcomes of 37 consecutive patients <18 years of age with pediatric NPC treated between 2000 and 2015. Patients were treated with concurrent chemoradiotherapy (CTRT) with cisplatin and 5-fluorouracil (5-FU) or CTRT with cisplatin, followed by adjuvant chemotherapy with cisplatin and 5-FU. STATISTICAL ANALYSIS: Survival was analyzed using Kaplan–Meier method, and log rank test was used to compare variables. RESULTS: The median duration of follow-up was 36.6 months. The median age of the patients was 15 years, and 22/37 (59%) patients were male. The most common presenting complaint was neck mass (70%), followed by nasal bleeding (16%). The distribution of Stage I, II, III, and IV patients was 1/37 (3%), 2/37 (6%), 13/37 (35%), and 21/37 (57%), respectively. Distant metastasis at presentation was seen in 3/37 patients. Complete response was seen in 32/37 (86%) patients. The 3-year event-free survival (EFS) for the entire cohort of patients was 60.1%. No significant difference in EFS was observed with age, gender, stage, use of 5-FU with CTRT, and nutritional status. CONCLUSION: Majority of patients with pediatric NPC present with advanced stage disease at our center. No difference in outcomes was seen with the two schedules of CTRT. Local control could be achieved in majority of patients; however, distant metastasis was the most common reason for relapse.


Keywords: Chemotherapy, nasopharyngeal carcinoma, survivalKey Words: Clefting artifact, histopathological examination, malignant melanoma, melanocytes and melanophages, pigmented basal cell carcinoma


How to cite this article:
Radhakrishnan V, Kumar P, Totadri S, Ganesan P, Selvaluxmy G, Ganesan T, Dhanushkodi M, Sagar T. Pediatric nasopharyngeal carcinoma: Experience from a tertiary cancer center in India. Indian J Cancer 2016;53:377-80

How to cite this URL:
Radhakrishnan V, Kumar P, Totadri S, Ganesan P, Selvaluxmy G, Ganesan T, Dhanushkodi M, Sagar T. Pediatric nasopharyngeal carcinoma: Experience from a tertiary cancer center in India. Indian J Cancer [serial online] 2016 [cited 2017 Jul 27];53:377-80. Available from: http://www.indianjcancer.com/text.asp?2016/53/3/377/200663



 » Introduction Top


Pediatric nasopharyngeal carcinomas (NPCs) are rare tumors.[1],[2] They are more common in East Asian countries such as China and Korea. There is paucity of data on outcomes in pediatric NPC from India. The present study was conducted to identify survival outcome in patients with pediatric NPC at our center.


 » Patients and Methods Top


Case records of all consecutive pediatric NPC patients <18 years of age treated at our center between January 1, 2000, and December 31, 2015, were analyzed. Diagnosis of NPC was confirmed by nasopharyngoscopy and biopsy of the tumor. Cervical nodes were evaluated by biopsy or fine-needle aspiration cytology (FNAC) if required. Immunohistochemistry was used to distinguish NPC from other common pathologies such as lymphoma. The World Health Organization (WHO) system was used for subtyping the tumor as Type 1 (keratinized), Type 2 (nonkeratinized), and Type 3 (poorly differentiated).[2] Staging was performed using contrast-enhanced computed tomography scan of the head and neck region, chest X-ray, ultrasound of the abdomen and pelvis, and technetium-99 bone scan. Patients were staged according to the American Joint Committee on Cancer staging system.[1]

Planned treatment included concurrent chemoradiotherapy (CTRT) with infusional cisplatin 70 mg/m 2 on day 1 with or without bolus 5-fluorouracil (5-FU) 325 mg/m 2/day from day 1 to 3 repeated every 3 weeks for three cycles. CTRT was followed by adjuvant chemotherapy with infusional cisplatin 70 mg/m 2 on day 1 and bolus 5-FU 325 mg/m 2/day from day 1 to 3 repeated every 3 weeks for three cycles. CTRT with cisplatin and 5-FU was practiced in our institute till the year 2008 after which patients received CTRT with cisplatin alone omitting 5-FU. External beam radiotherapy was given at a dose of 60–66 Gy at 2 Gy/day fraction over 7 weeks by 4–6 MV photons to the nasopharynx, parapharyngeal areas, oropharynx, and base of the skull by three-dimensional conformal radiotherapy. Any intracranial extension was irradiated with 1-cm margins superiorly while nasal extension was treated by a three-field technique including an anterior field. Pituitary was shielded from the field of radiation. All patients received 45 Gy to the entire cervical nodal areas by 4 MV photons, followed by 10 Gy by electrons sparing the spinal cord. Bulky or residual cervical nodes received a boost of 5–10 Gy by electrons of appropriate energy.

Reassessment was done after 4–6 weeks of completion of adjuvant chemotherapy and included clinical examination, nasopharyngoscopy, and biopsy or FNAC of any residual neck node. Patients with residual neck nodes after completion of treatment were offered neck dissection if the FNAC or biopsy of the node showed disease. Follow-up included visits to the hospital for a clinical examination including nasopharyngoscopy every 3 months for the first 3 years and then every 6 months. All other investigations were indicated only if patient became symptomatic.

Event was defined as death due to any cause or relapse or progression of disease. Patients were censored on the date of the last follow-up. Event-free survival (EFS) was calculated from date of initiation of treatment to date of relapse or documented progression or death. EFS was estimated using Kaplan–Meier method, and variables were compared using the log rank test. P < 0.05 was considered statistically significant. Statistical analysis was done using SPSS software (IBM, SPSS for Windows, Version 17.0. Chicago, SPSS Inc).


 » Results Top


We analyzed the data of 37 patients who were treated during the study period. The median age of the patients was 15 years (range 8–18 years), and 22/37 (59%) patients were male. The median duration of symptoms was 4 months. The most common presenting complaint was neck mass (70%) followed by nasal bleeding (16%). The distribution of Stage I, II, III and IV patients was 1/37 (3%), 2/37 (6%), 13/37 (35%), and 21/37 (57%), respectively. Distant metastasis (Stage IV C) was present in 3/37 (8%) patients at presentation, all three patients had bone metastasis and in addition one patient had liver metastasis. WHO histology subtype 3 was observed in 35/37 (95%) patients and subtype 2 in 2/37 (5%) patients. [Table 1] provides the demographic and clinical details of the patients.
Table 1: Demographic and clinical features of the study patients (n=37)

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Treatment and response

CTRT with cisplatin and 5-FU was given in 25/37 (68%) patients, and CTRT with cisplatin was given in 10/37 (27%) patients. Radiotherapy without concurrent chemotherapy was given in 2/37 patients due to their poor performance status; both these patients had metastatic disease at presentation and did not receive adjuvant chemotherapy due to disease progression. The planned three cycles of CTRT could be completed in 18/25 (72%) patients who received CTRT with cisplatin and 5-FU and 8/10 (80%) patients who received CTRT with cisplatin. Adjuvant chemotherapy was given in 35/37 patients, and the mean and median cycles of adjuvant chemotherapy given were 2.4 and three cycles, respectively (range 1–5). Complete response (CR) was seen in 32/37 (86%) patients, partial response (PR) in 3/37 (8%) patients, and progressive disease seen in 2/37 (8%) patients after the completion of adjuvant chemotherapy. Neck dissection was performed in all three patients with PR response.

Events

There were 15 events in the 37 study patients. Event was due to disease progression in 3/15 patients and disease relapse in 12/15 patients. The mean and median duration of relapse were 18.4 months and 15.7 months, respectively (range 7.03–45.3 months). Distant relapse was observed in 14/15 patients, while 1/15 patients had locoregional relapse in the cervical lymph node. The most common site of relapse was bones seen in 12/15 patients, followed by liver in 2/15 patients and lung in 1/15 patients. Palliative metronomic chemotherapy was offered to 3/14 relapsed patients, 1/14 relapsed patient underwent neck dissection, and palliative radiotherapy to control bone pain was given to 4/14 patients.

Survival

The mean and median duration of follow-up were 48.4 months and 36.6 months, respectively (range: 2.9–160 months). The 3-year EFS for the entire cohort of patients was 60.1%, and the 3-year EFS for patients who received CTRT was 64% [Figure 1]. The 3-year EFS for Stages I, II, III, IV A, IV B, and IV C patients was 0%, 100%, 45.5%, 71.4%, 48.2%, and 33.3%, respectively. No significant difference in EFS was seen with gender, between Stages III and IV disease, tumor size, nodal status, and the chemotherapy schedule used in CTRT [Table 2].
Figure 1: Kaplan–Meier curve depicting event-free survival

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Table 2: Prognostic factors for event-free survival for patients who received concurrent chemoradiotherapy (n=35)

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Toxicity

The common Grade 3 or 4 toxicity observed during treatment were mucositis in 12/37 patients, neutropenia in 5/37 patients, emesis in 4/37 patients, and skin reaction in 1/37 patient.

Lost to follow-up and abandonment

Eighteen of the 37 patients have been lost to follow-up, of which 11/18 patients who had relapsed and were sent home on supportive care and 7/18 patients who had completed adjuvant chemotherapy and were in CR.


 » Discussion Top


Pediatric NPC predominantly occur in the adolescent age group and this was also seen in our cohort where the median age was 15 years.[2] Compared to adults, pediatric NPC are more poorly differentiated (WHO Type 3), but this subtype is more responsive to CTRT.[2] Although we could achieve a CR rate of 86% in our study population, this did not translate to improvement in EFS. Majority of the relapses in NPC are distant as was seen in our study. This indicates the systemic nature of the illness and the importance of chemotherapy for controlling and preventing metastasis.

CTRT with cisplatin and 5-FU did not improve EFS compared to using cisplatin alone in our study; however, this comparison was not randomized [Figure 2]. We use a lower dose of cisplatin and 5-FU at our center compared to what is reported in the literature because majority of our patients tend to be malnourished and in poor physical condition due to advanced disease and therefore are at a greater risk of treatment-related morbidity and mortality. There was a trend toward improved survival in patients who were younger than 12 years of age or were not malnourished or had N1 nodal involvement.
Figure 2: Event-free survival for concurrent chemoradiotherapy with cisplatin and 5-fluorouracil versus cisplatin

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Chemoradiotherapy was well tolerated in our patients and there were no treatment-related deaths. The most common toxicities were mucositis and neutropenia. We had a higher rate of lost to follow-up in our study because patient who had relapsed were referred back home on supportive care. Currently available chemotherapy options for relapsed NPC are limited and are palliative in nature.[1]

[Table 3] provides the comparison between various studies reported from India. The limitations of our study include that it is retrospective in nature, and overall survival could not be calculated as a large proportion of patients were lost to follow-up and inadequate information on treatment morbidity.
Table 3: Studies on pediatric nasopharyngeal carcinomas from India

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 » Conclusion Top


Majority of patients with pediatric NPC at our center present with advanced disease. Distant relapses are common despite achieving excellent local control. Our survival outcomes are comparable to what has been reported in literature from other centers.[3][4][5]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
 » References Top

1.
Lee AW, Ma BB, Ng WT, Chan AT. Management of nasopharyngeal carcinoma: Current practice and future perspective. J Clin Oncol 2015;33:3356-64.  Back to cited text no. 1
    
2.
Ayan I, Kaytan E, Ayan N. Childhood nasopharyngeal carcinoma: From biology to treatment. Lancet Oncol 2003;4:13-21.  Back to cited text no. 2
    
3.
Venkitaraman R, Ramanan SG, Sagar TG. Nasopharyngeal cancer of childhood and adolescence: A single institution experience. Pediatr Hematol Oncol 2007;24:493-502.  Back to cited text no. 3
    
4.
Laskar S, Sanghavi V, Muckaden MA, Ghosh S, Bhalla V, Banavali S, et al. Nasopharyngeal carcinoma in children: Ten years' experience at the Tata Memorial Hospital, Mumbai. Int J Radiat Oncol Biol Phys 2004;58:189-95.  Back to cited text no. 4
    
5.
Guruprasad B, Tanvir P, Rohan B, Kavitha S, Naik SM, Appaji L. Paediatric nasopharyngeal carcinoma: An 8-year study from a tertiary care cancer centre in South India. Indian J Otolaryngol Head Neck Surg 2013;65 Suppl 1:131-4.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]



 

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