International Journal of Pediatric Otorhinolaryngology Extra 14 (2016) 20e22

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International Journal of Pediatric

Otorhinolaryngology Extra
journal homepage: http://www.ijporlextra.com/

Case report

Presentation and management of nasopharyngeal carcinoma, a rare childhood

malignancy
Priyesh N. Patel a, b, *, Edward B. Penn a, b
a
Department of Otolaryngology, Division of Pediatric Otolaryngology, Vanderbilt University Medical Center, Nashville, TN, USA
b
Department of Otolaryngology, Doctors' Ofce Tower, 7th Floor, 2200 Children's Way, Nashville, TN 37232-8605, USA

a r t i c l e i n f o a b s t r a c t

Article history: Nasopharyngeal carcinoma (NPC) is a rare malignancy of epidermoid origin, one that is especially un-
Received 2 September 2016 common in children in low risk areas such as the United States. The relative rarity in children makes the
Received in revised form diagnosis of this malignancy difcult, and therefore childhood nasopharyngeal carcinoma is associated
30 October 2016
with advanced locoregional disease and higher prevalence of distant metastasis. A case of pediatric
Accepted 1 November 2016
Available online 4 November 2016
nasopharyngeal carcinoma is described here to highlight a possible presentation of this uncommon
pediatric disease and reasons for delayed diagnosis. This is followed by a brief discussion of epidemi-
ology, diagnosis, treatment, and outcomes for this malignancy in children.
Keywords:
Nasopharyngeal carcinoma
2016 Elsevier Ltd. All rights reserved.
Pediatric

1. Introduction demonstrated a mildly elevated ESR, but otherwise normal LDH,

Nasopharyngeal carcinoma (NPC) is a rare malignancy, with the
incidence in high risk regions as high as 20 per 100,000 person-
years and lower rates seen in children [1]. In the United States,
the incidence for children and adolescents is 0.5 per million person-
years [2]. Given the relative rarity of this disease in the pediatric
population and common, yet nonspecic, presentations including
nasal congestion, lymphadenopathy, and ear symptoms, diagnosis
can be delayed and initially attributed to non-malignant etiologies.
Here we present a case of NPC in a pediatric patient and review the
epidemiology, diagnosis, treatment, and outcomes for this malig-
nancy in children.
2. Case report
A 16 year-old otherwise healthy male presented with a 5-month
duration of right sided otalgia with bilateral painful lymphade-
nopathy. He also complained of diminished hearing on the right
side. He has had no fevers, chills, weight loss, bleeding, voice
changes, breathing complaints, or dysphagia.
He had undergone previous laboratory evaluation, which
* Corresponding author. Department of Otolaryngology, Division of Pediatric
Otolaryngology, Vanderbilt University Medical Center, Nashville, TN, USA.
E-mail addresses: priyesh.patel@vanderbilt.edu (P.N. Patel), Edward.b.penn@
vanderbilt.edu (E.B. Penn).
complete blood count, and basic metabolic panel. Given the pa-
tient's age and clinical presentation, he was previously treated with
antibiotics, both per mouth and in the form of ear drops. Despite
this, the patient had persistent symptoms, prompting a pediatric
otolaryngology referral.
On evaluation, patient was found to have a right ear serous
effusion and right greater than left posterior cervical lymphade-
nopathy. Oropharyngeal exam was unremarkable, however, a nasal
exible endoscopic exam revealed a smooth, hyperemic, non-
ulcerative mass in the nasopharynx occupying roughly 90% of this
space (Fig. 1). Repeat laboratory evaluation was notable for an
elevated LDH (300) and white blood cell count (17.6). On a maxil-
lofacial CT scan with contrast, the enhancing mass obliterated the
right portion of the nasopharynx with extension past midline and
into the prevertebral and parapharyngeal spaces. He had notable
bilateral cervical lymphadenopathy with the greatest right sided
nodal conglomerate measuring 3  2.2 cm. MRI (Fig. 2a and b) and
positron emission tomography (PET) imaging conrmed these
ndings, with no evidence of metastatic disease beyond the neck.
He was subsequently admitted to the hospital and taken to the
operating room 4 days after initial ENT evaluation, with a biopsy of
this mass demonstrating undifferentiated, nonkeratinizing NPC.
The tumor, based on radiographic evidence, is staged as a T2b
(extends to soft tissues of the oropharynx with extension to the
parapharyngeal region), N2 (bilateral nodal involvement <6 cm
maximal diameter above supraclavicular fossa), and M0 (no met-
astatic disease), with overall stage 3 diagnosis.

http://dx.doi.org/10.1016/j.pedex.2016.11.001
1871-4048/ 2016 Elsevier Ltd. All rights reserved.
 P.N. Patel, E.B. Penn / International Journal of Pediatric Otorhinolaryngology Extra 14 (2016) 20e22
Fig. 1. Endoscopic exam revealing a smooth, nonulcerative mass in the nasopharynx.
The patient underwent a Hickman catheter and gastric tube
placement prior to starting treatment. Also, given the patient's
right sided effusion and notable mastoid and middle ear opaci-
cation on imaging, a myringotomy tube was placed. The child was
then treated with induction 5-Fluorouracil and Cisplatin, followed
by concurrent Cisplatin and radiation therapy. Treatment was
complicated by mild xerostomia and gingivostomatitis treated with
valacyclovir. He also developed bilateral mild sensorineural hearing
loss at higher frequencies.
3. Discussion
NPC is a rare malignancy of epidermoid origin. While incidence
in some low risk areas such as the United States is low, in some high
risk regions, incidence is as high as 20 per 100,000 person-years [1].
In the United States, the incidence for children and adolescents is
0.5 per million person-years versus 8.4 in adults [2].
The relative rarity in children makes the diagnosis of this ma-
lignancy difcult, and therefore childhood NPC is associated with
advanced locoregional disease and higher prevalence of distant
metastasis [2]. As in this case, diagnosis was delayed since the
child's presentation with painful adenopathy was suspected to be
of infectious etiology. Moreover, while one sided serous effusion in
Fig. 2. (a) T1 weighted MRI with contrast demonstrates a nasopharyngeal mass with extension into the prevertebral and parapharyngeal spaces (b) T2 weighted MRI demonstrates
bilateral nodal disease and uid within the right mastoid.
21
adults is highly unusual and warrants further evaluation, in
younger children this is not necessarily an atypical nding.
This case demonstrates the potential presentation on an un-
common disease process and highlights the importance of
considering NPC in children presenting with persistent unilateral
ear ndings. Infectious etiologies of ear pain in the setting of
adenopathy in the pediatric patient is far more likely than a
neoplastic cause. In the absence of improvement on antibiotic
treatment, further workup and Otolaryngology referral for the
above symptoms is warranted. Of great importance, children with
persistent unilateral ear ndings should undergo in-ofce exible
beroptic examination of the nasal cavity and nasopharynx. The
above case also highlights that constitutional symptoms (i.e. fevers
or weight loss) may not be present in children with NPC, and that
early laboratory evaluation may be relatively unremarkable.
Amongst children in non-endemic areas, there is a peak inci-
dence in individuals aged 15e19 with a male to female ratio of >2:1
[3]. While early diagnosis of NPC in children can be difcult and
although no guidelines exist in regards to the time-frame of initi-
ating a malignancy workup in children with unilateral ear symp-
toms, a higher degree of suspicion may be warranted in males
within this age group.
Standard treatment of NPC involves concurrent chemoradiation,
with surgical therapy usually reserved for salvage treatment. In-
duction chemotherapy is better tolerated than adjuvant chemo-
therapy, and has been increasingly implemented and studied in the
setting of locally advanced disease. Induction chemotherapy was
used in this case, and some phase III trials have demonstrated in-
duction chemotherapy yields improved recurrence and disease free
survival rates [3]. Conicting evidence exists in regards to its
benet when compared with chemoradiation therapy alone [3,4].
The benet and use of induction chemotherapy, therefore, remains
under investigation.
While side effects of radiation therapy to the head and neck
vary, almost all patients complain of some degree of xerostomia.
Ototoxicity with the use of cisplatin is commonly encountered and
is typically bilateral and irreversible. The risk of ototoxicity in-
creases with concomitant use of high dose radiation [5]. In children,
the loss of hearing at younger ages can impact the speech, cogni-
tive, and social development of the child. Thus, future treatments
that will ameliorate ototoxicity are needed. In addition, decision to
proceed with myringotomy tube placement, as was done in this
case, should be highly considered in children as a persistent effu-
sion may also contribute to hearing and speech deciencies.
22 P.N. Patel, E.B. Penn / International Journal of Pediatric Otorhinolaryngology Extra 14 (2016) 20e22
Children respond better than adults to treatment, and therefore
outcomes tend to be better. The 5-year survival in children under
the age of 18 is 77% 7.3% with a 5-year NPC-specic survival of
83% 3.9% [2,6]. In regards to surveillance after treatment, the risk
of developing a second cancer is signicantly increased in all chil-
dren with NPC [2]. In the pediatric population, early diagnosis of
NPC prior to signicant locoregional disease will hopefully limit the
extent of radiation and associated comorbidities associated with
treatment of this disease.
Conicts of interest
None.
Funding
This research did not receive any specic grant from funding
agencies in the public, commercial, or not-for-prot sectors.
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