CASE REPORT

NASOPHARYNGEAL CARCINOMA

Presentator : dr. Seno Aji Saputro

Moderator : dr. Agus Surono, Ph.D, M.Sc, Sp.T.H.T.K.L.(K)

Departement of Otorhinolaryngology – Head & Neck Surgery

Faculty of Medicine Gadjah Mada University/ Dr. Sardjito General Hospital
Yogyakarta 2018
INTRODUCTION NPC is still noted in American-born Chinese,
though the incidence is lower than the
Nasopharyngeal carcinoma (NPC) is
Chinese who reside in the southeastern part
a non-lymphomatous,squamous cell
of China.(2) These findings impose an
carcinoma that occurs in the epithelial lining
interaction among geographic, ethnic and
of the nasopharynx. It shows varying degrees
environmental etiologic factors.1,2
of differentiation and is frequently seen at the
In Indonesia, From the intake registry
pharyngeal recess (fossa of Rosenmüller),
in the Ear, Nose, and Throat department at
posteromedial to the medial crura of the
Dr. Cipto Mungunkusumo Hospital, which
Eustachian tube opening. The tumor cell
includes 6000 H&N cancer cases registered
originates from the epithelial lining, and the
between 1995 and 2005, it is studied the
definition of NPC strictly excludes all the
incidence of individual cancer types,
other nasopharyngeal malignancies arising
including 1121 cases diagnosed as NPC. The
from lymphoid tissue or connective tissue,
gender distribution among NPC cases
such as lymphomas or sarcomas.. NPC is a
showed 789 males versus 332 females, with
highly metastatic and invasive malignant
the peak age 50-60years old and less than 1%
tumor and is more metastatic than any other
below 20 years old.2
head and neck carcinomas. Approximately
In Jogjakarta the incidence rate is
90 % of patients show cervical lymph nodes
4,9/100.0000, mean regional incidence.
metastasis as the most frequently found in
Bandung, Malang, Denpasar, Manado, and
nasopharyngeal carcinoma.1 It has a
Surabaya represented high incidence areas,
remarkably distinctive ethnic and geographic
and therefore, early detection of disease in
distribution and most of the cases are
these cities deserves special attention.
reported from China, Southeast Asia, and
Overall, the incidence of 5.66/100 000,
North Africa.1
equaling roughly 1000 new cases per month,
NPC is a unique malignancy with an
reflected a major health problem in
endemic distribution among certain well
Indonesia, particularly because most of these
defined ethnic geographic groups. It is one of
patients were referred to the hospital at a late
the most common head and neck cancers
stage.2
among the Chinese population but is a rare
Infection by the Epstein - Barr virus
cancer among Caucasians in Europe and
is often associated with the incidence of
North America. However, a high incidence of

1
nasopharyngeal carcinoma. The role of
genetic factors in malignancy continues to be
analyzed, the study showed abnormalities in
specific chromosomes. Some studies also
have shown that excessive consumption of
carcinogenic substances (such as
consumption of salted fish) will increase the
incidence of nasopharyngeal carcinoma. The
genetic factor also might have an important
role in the etiology of NPC. Comparative
genomic hybridization studies
demonstrated several genomic spots where
chromosomal losses and gains
identified in NPC. This suggests that genetic,
ethnic, and environmental factors may play a
role in the etiology of the disease.3
Patients suffering from NPC may
present with one or more of the four groups
of symptoms. These groups of symptoms are
related to the location of the primary tumor,
infiltration of structures in the vicinity of the
nasopharynx, or metastasis to cervical lymph
nodes. The presence of tumor mass in the
nasopharynx may lead to the symptoms of
nasal obstruction and discharge. When the
tumor ulcerates, the patient may present with
epistaxis. The amount of bleeding is usually
trivial and the frequent presentation is the
presence of altered blood in the postnasal
drip, especially in the morning.4,5
The tumor bulk in the nasopharynx with or
without posterolateral extension into the
paranasopharyngeal space is frequently
associated with dysfunction of the Eustachian
tube. This may lead to the collection of fluid
the in the middle ear and the patients may
experience unilateral deafness that is
conductive in nature and other otologic
symptoms such as otalgia and tinnitus.
Another symptoms are diplopia, facial pain
have and numbness. In view of the high propensity
of NPC to metastasize to cervical lymph
were nodes, the most frequent presenting symptom
is painless neck masses that frequently
appearing in the upper neck. Unfortunately,
because of the nonspecific nature of the nasal
and aural symptoms and the inconspicuous
nature of the painless cervical lymph node,
the majority of NPC
patients are only diagnosed when their tumor
have reached advanced stages.5
When patients present with
symptoms of NPC, they should be evaluated
clinically for physical signs of NPC such as
the presence of lymph nodes in the neck,
fluid in the middle ear, and cranial nerve
involvement. Other investigations toward the
diagnosis of NPC are the estimation of
antibody levels against EBV, imaging studies
and endoscopic examination of the
nasopharynx and biopsy.5
2
 A clinical staging system for NPC is
essential for treatment planning and
evaluation of thempeutic outcome. Over the
years, a few staging systems were used for
NPC such as the Union International Contre
le Cancer (UICC) system and the American
Joint Committee on Cancer Staging (AJCC).
Both the UICC and the AJCC systems assess
tumor extent in the nasopharynx by
considering the number of tumor-affected
sites within the nasopharynx. In the recent
staging system of 2009, Tl stage included all
tum.on that confine to the nasopharynx or
extend locally such as inferiorly to the
oropharynx or anteriorly to the nasal cavity.
On the other hand, lateral tumor extension to
the paranasopharyngeal space indicates more
advanced disease. T2 stage includes tumor
that has extended to the paranasopharyngeal
space. The T3 disease covers tumor that have
involved the skull base or the paranasal
sinuses. T4 tum on are those that have
extended to the infratemporal fossa, orbit,
hypopharynx. and cranium, or have affected
the cranial nerves. The UICC/AJCC staging
systems recognize the size of the cer:vical
lymph node as an important factor. For other
head and neck cancer, N1 is less than 3 an in
size and N2 is greater than 3 cm. The
difference between N2 and N3 is a nodal size
of 6 cm. The retropharyngeal nodes, which
3
are the first-echelon nodes, are not taken into
account by all staging systems. CT or MRI
now detects the nodes. These factors are all
addressed in the nodal staging section of the
recent UICC/AJCC for nasopharyngeal
cancer staging system. The measurement of 6
cm is the only factor for size. Laterality and
site of involvement such as the
retropharyngeal region and the
supraclavicular fossa are other important
factors in determining the N staging. Under
the current system., Nl referred to unilateral
nodal involvement less than 6 cm in diameter
and not reaching the supraclavicular fossa.
Bilateral retropharyngeal nodes as long as
they are less than 6 cm are still Nl. Bilateral
cervical nodal disease that has not reached
N3 designation, irrespective of size, number,
and location is classified N2. Stage N3
disease referred to lymph nodes larger than 6
cm (N3a), or nodes that had extended to the
supraclavicular fossa (N3b). For M-staging,
M1 represents distant metastases, including
any lymph node involvement below the level
of the clavicle. The current
unified staging system has enabled patients to
be staged more precisely and simply; it is also
considered as a better predictor of prognosis.
5,6
The treatment of NPC already
become one of the challenge nowadays
caused by the complexity of the disease. The
treatment include radiotherapy,
chemotherapy, chemoradiotherapy, and
operatif.7
CASE REPORT
A 64 years old man came into The
ENT clinic of Dr. Sardjito hospital
complained of a lump arise since 1 year ago
on his right neck, and it was increasingly
enlarged. The complained followed by
ringing and decrease of hearing on his right
ear since one year ago. Left nose was
clogged up since about 8 months before
going to a ENT clinic of Dr. Sardjito
Hospital, patients complain of thick yellow
mucus in the right nose but there is no history
about epistaxis or bloody discharge before.
No complaints of pain and discharge from
both ears. Headache, cough, shortness of
breath, throat disorders were denied. Eating
and drinking there is normal and no
complaints of weight loss. Patients were
heavy smokers (1 pack a day). There were no
history of the same complained, diabetes
mellitus, hypertensi, and allergy from the
patient before. There were no history from
his family about having cancer or tumors,
diabetes mellitus, hypertensi, and allergy.
The physical examination of the
patient we got general condition was good,
4
awareness composmentis. Patient’s vital
signs, blood pressure : 110/70 mmHg, breath
frequency : 20 x / min , pulse : 90 x / min , a
temperature of 37.0 °C. Physical examination
of the left and right ear we get his external
ear canals within the normal limits. Both of
tympanic membrane are intac with cone of
light (+). Examination of anterior rhinoscopy
we can see mucopurulent secretions in the
both side of the nasal cavity but there were no
mass present. On posterior rhinoscopy there
were masses from dextra and sinistra of the
nasopharyng. From the oropharing and
Indirect laryngoscopy examination within
normal limits. The examinaton of neck region
showed an enlarged mass of dextra
lymphnodes, size 6 cm x 4 cm x 1 cm and in
the sinistra lymphonodes with diametres of
2cm, both of it without pain. There was no
mass palpable on the axilla.
The supporting examination such as
Nasopharynx CT Scan with contrast showed
malignant of the nasopharing sinistra
extending to the nares posterior with
lymphadenopathy coli medial bilateral with
largest diametres of 4.8cm. From
nasoendoscopy there were masses present
from dextra and sinistra of the nasopharynx.
The result of the biopsy is metastasize
undifferentiated carcinoma (WHO Type III).
From the thorax x-ray, bone survey and
abdominal USG there were no sign and prognosis with 5 years survival rate is 60-80
vision of metastases. % because it is radiosensitive, in contrast
Based on the anamnesis, physical with the NPC WHO type 1 have the worst
examination and supporting examination of prognosis with 5 years survival rate is 20-40
the patients we diagnosed him with % because it is the most less sensitive to
Nasopharyngeal Carcinoma stage III radiation.8 It is important to educate and
(T2aN2M0) on 19th February 2018. The motivate patients to adhere and obey the
patient is planning to get chemoradiotherapy. treatment program well to achieve the
The issue of this case is prognosis. success of the therapy.
Due to nasopharynx anatomical
DISCUSSION proximity to critical structures, the role of
This patient has diagnosed with surgery is limited to biopsy for histologic
Nasopharyngeal Carcinoma stage III confirmation and treatment and prevention of
(T2AN2M0), this case is suitable with the persistent or recurrent disease, respectively.
insidence of the disease, where male patient Therefore, NPC is traditionally treated with
is more dominant (3:1) than female patient, radiotherapy (RT). However, distant failure
there are several factors that influence the leads to a pattern of recurrence after RT
prognosis of this disease, such as tumor size alone, especially among patients with
associated with infiltration into surrounding locoregionally advanced disease. Most
organs, histopathological type of the tumor, patients with distant failure do not survive for
involvement of lymph nodes of the neck, age, > 1 year after the diagnosis. The overall 5-
gender, and therapeutic techniques are given. year survival rates for patients with stages III
Numerous studies have demonstrated by and IV NPC treated with RT in a large series
administering radiotherapy alone, an average were only 45.8% and 29.2% respectively.
of 5 years survival rate for NPC stage I and Therefore, various combinations of RT and
stage II of 85-90 % to 70-80 %. While in the chemotherapy, such as concurrent
further stages ( III - IV ) on average 5 years chemoradiotherapy (CCRT), neoadjuvant
survival rate was 37 % with radiation therapy chemotherapy (NAC), adjuvant
alone, but can be 67 % when given alongside chemotherapy (AC), and alternating
chemotherapy radiotherapy. Nasopharynx chemoradiotherapy (CRT), have been
WHO type III carcinoma has a good investigated.8

5
 Adjuvant chemotherapy has been series involving more than 9500 patients with
employed to treat locoregionally advanced all stages of NPC.9
disease and relapses with a consistently high
rate of initial tumor response, but no study to SUMMARY
date has demonstrated an improvement in It has been reported a 64 years old
overall survival or reduction of distant man diagnosed with Nasopharyngeal
metastases. Cisplatin-based combination Carcinoma stage III (T2aN2M0) at February
chemotherapy has been reported to give a 19th 2018. The patient is planning to get
high response rate in patients with metastatic chemoradiotherapy. It is important to
NPC with a subgroup of the patients educate and motivate patients to adhere and
achieving a long-term disease free survival. obey the treatment program well to achieve
However, the most effective the success of the therapy.
chemotherapeutic agent or regimen, the
optimal timing with radiotherapy or the REFERENCES
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infection, Journal of Zhejiang University
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