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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,


complete blood count, and basic metabolic panel. Given the pa-
Nasopharyngeal carcinoma (NPC) is a rare malignancy, with the tient's age and clinical presentation, he was previously treated with
incidence in high risk regions as high as 20 per 100,000 person- antibiotics, both per mouth and in the form of ear drops. Despite
years and lower rates seen in children [1]. In the United States, this, the patient had persistent symptoms, prompting a pediatric
the incidence for children and adolescents is 0.5 per million person- otolaryngology referral.
years [2]. Given the relative rarity of this disease in the pediatric On evaluation, patient was found to have a right ear serous
population and common, yet nonspecic, presentations including effusion and right greater than left posterior cervical lymphade-
nasal congestion, lymphadenopathy, and ear symptoms, diagnosis nopathy. Oropharyngeal exam was unremarkable, however, a nasal
can be delayed and initially attributed to non-malignant etiologies. exible endoscopic exam revealed a smooth, hyperemic, non-
Here we present a case of NPC in a pediatric patient and review the ulcerative mass in the nasopharynx occupying roughly 90% of this
epidemiology, diagnosis, treatment, and outcomes for this malig- space (Fig. 1). Repeat laboratory evaluation was notable for an
nancy in children. 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
2. Case report into the prevertebral and parapharyngeal spaces. He had notable
bilateral cervical lymphadenopathy with the greatest right sided
A 16 year-old otherwise healthy male presented with a 5-month nodal conglomerate measuring 3  2.2 cm. MRI (Fig. 2a and b) and
duration of right sided otalgia with bilateral painful lymphade- positron emission tomography (PET) imaging conrmed these
nopathy. He also complained of diminished hearing on the right ndings, with no evidence of metastatic disease beyond the neck.
side. He has had no fevers, chills, weight loss, bleeding, voice He was subsequently admitted to the hospital and taken to the
changes, breathing complaints, or dysphagia. operating room 4 days after initial ENT evaluation, with a biopsy of
He had undergone previous laboratory evaluation, which 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
* Corresponding author. Department of Otolaryngology, Division of Pediatric
Otolaryngology, Vanderbilt University Medical Center, Nashville, TN, USA.
parapharyngeal region), N2 (bilateral nodal involvement <6 cm
E-mail addresses: priyesh.patel@vanderbilt.edu (P.N. Patel), Edward.b.penn@ maximal diameter above supraclavicular fossa), and M0 (no met-
vanderbilt.edu (E.B. Penn). 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 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
Fig. 1. Endoscopic exam revealing a smooth, nonulcerative mass in the nasopharynx. 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
The patient underwent a Hickman catheter and gastric tube within this age group.
placement prior to starting treatment. Also, given the patient's Standard treatment of NPC involves concurrent chemoradiation,
right sided effusion and notable mastoid and middle ear opaci- with surgical therapy usually reserved for salvage treatment. In-
cation on imaging, a myringotomy tube was placed. The child was duction chemotherapy is better tolerated than adjuvant chemo-
then treated with induction 5-Fluorouracil and Cisplatin, followed therapy, and has been increasingly implemented and studied in the
by concurrent Cisplatin and radiation therapy. Treatment was setting of locally advanced disease. Induction chemotherapy was
complicated by mild xerostomia and gingivostomatitis treated with used in this case, and some phase III trials have demonstrated in-
valacyclovir. He also developed bilateral mild sensorineural hearing duction chemotherapy yields improved recurrence and disease free
loss at higher frequencies. survival rates [3]. Conicting evidence exists in regards to its
benet when compared with chemoradiation therapy alone [3,4].
3. Discussion The benet and use of induction chemotherapy, therefore, remains
under investigation.
NPC is a rare malignancy of epidermoid origin. While incidence While side effects of radiation therapy to the head and neck
vary, almost all patients complain of some degree of xerostomia.
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]. Ototoxicity with the use of cisplatin is commonly encountered and
is typically bilateral and irreversible. The risk of ototoxicity in-
In the United States, the incidence for children and adolescents is
0.5 per million person-years versus 8.4 in adults [2]. creases with concomitant use of high dose radiation [5]. In children,
the loss of hearing at younger ages can impact the speech, cogni-
The relative rarity in children makes the diagnosis of this ma-
lignancy difcult, and therefore childhood NPC is associated with tive, and social development of the child. Thus, future treatments
that will ameliorate ototoxicity are needed. In addition, decision to
advanced locoregional disease and higher prevalence of distant
metastasis [2]. As in this case, diagnosis was delayed since the proceed with myringotomy tube placement, as was done in this
case, should be highly considered in children as a persistent effu-
child's presentation with painful adenopathy was suspected to be
of infectious etiology. Moreover, while one sided serous effusion in sion may also contribute to hearing and speech deciencies.

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.
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 References


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Conicts of interest [5] W.C. Chen, A. Jackson, A.S. Budnick, D.G. Pster, D.H. Kraus, M.A. Hunt,
H. Stambuk, S. Levegrun, S.L. Wolden, Sensorineural hearing loss in combined
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None.
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S. Polychronopoulou, F. Akman, S.L. Wolden, S. Sarihan, R.C. Miller, M. Ozsahin,
Funding U. Abaciog lu, M. Martin, M. Caloglu, L. Scandolaro, E. Szutowicz, I.L. Atahan,
Treatment results of 165 pediatric patients with non-metastatic nasopharyn-
geal carcinoma: a rare cancer network study, Radiother. Oncol. 81 (2006)
This research did not receive any specic grant from funding 39e46.
agencies in the public, commercial, or not-for-prot sectors.