KARSINOMA NASOFARING
Merupakan salah satu jenis kanker terbanyak di
Indonesia. Terdiri atas 3 jenis berdasarkan penggolongan WHO.
WHO criteria
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Type 1
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Differentiated squamous cell carcinoma
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Type 2
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Nonkeratinizing carcinoma
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Type 3
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Undifferentiated carcinoma
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ETIOLOGI
a.
Herediter(1), gen HLA-A2,B-17,Bw26(2)
Orang yang keluarganya
pernah mengidap penyakit ini uga lebih rentan.
b.
Virus (EBV)(1), HPV-18 (3)
c.
Lingkungan(4)
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Zat karsinogen
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Radiasi
Faktor
ini terutama faktor eksogen dapat menyebabkan terbentuknya sel tumor lewat
mutasi gen nya seperti Gen yang biasa terganggu adalah (onkogen: bcl2, ras, myt. Tsg: p53, rb,,
SPLUNC1, UBAP1, BRD7, Nor1, NGX6, and LTF (5) kehilangan kromosom heterozigot (6), pengulangan kromosom 3p21 (2)(7).
FAKTOR RESIKO
a.
Alkohol dan rokok(8)
b.
Pria lebih banyak 2-3 kali daripada yang perempuan (4)
c.
Ras kaukasian jarang, sedangkan pada ras mongolian lebih banyak,.
Diketahui nbhwa indonesia merupakan daerah endemic tetapi dengan mortalitas yg
lebih rendah dari china, taiwan dan malaysia. (9) angka mortalitas tahun 2004 yaitu sebesar 127/100.000 jiwa (10)
d.
Perempuan yg pernah melakukan koitus di usia 16-18 tahun menunjukkan
adanya ebv di secret genitalnya(11)(12)(12).
e.
Masyarakat yang sering memakan ikan asin (mengandung nitrosamin)
sebagai karsinogen
f.
Tukang masak (lebih sering terpapar asap (13)
g.
Tempat kerja, orang yang sering terpapar formaldehida lebih rentan (10)(14)
GEJALA DAN MANIFESTASI KLINIS
Manifestasi klinis CNP biasanya dihubungkan dengan
letak anatomiknya terhadap hidung, tuba eustachii dan dasar tengkorak dan adanya jaringan submukosa
luas pembuluhh limfe.
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Epistasis (pendarahan dari hidung)
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Hidung tersumbat
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Pilek yg sudah lebih dari 1 bulan. Terutama pada orang usia diatas
40tahun.
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Lendir berdarah di tenggorokan belakang
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Suara berdengung di telinga (tinusitis)
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Kehilangan pendengaran
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Tidak berfungsinya saraf otak (n V, VI, X)
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Sakit kepala (biasanya dimulai dengan unilateral baru bilateral)(13)
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Gangguan neurologik kelumpuhan gangguan fungsi penglihatan(terganggunya
kiasama optikum)
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Pembengkakan leher (biasanya karena pembengkakan kelenjar limfe
PATOFISIOLOGI
Virus EBV yang masuk akan menyebabkan terjadi nya sel-sel abnormal lewat mutasi gen
nya.
Langkah awal infeksi litik EBV ditandai
dengan aktivitas protein ZEBRA yang disandi oleh gen BZLF1 yang terdapat pada
sel epitel dan limfosit B. Beberapa produk yang berbeda-beda dari gen yang
mempuyai korelasi dengan tahapan siklus replikasi litik dapat diidentifikasi
dan dikategorikan menjadi: Early Membrane Antigen (EMA), Early Intra- Celulair
Atigen (EA), Viral capcid Antigen (VCA),Late Membrane Antigen (LMA). Pada
infeksi latent terjadi ekspresi dari beberapa protein antara lain: Epstein Barr
Nucleus Antigen 2 & 5 (EBNA 2 & 5) yang dapat diteksi 2-5 jam setelah
infeksi, Latent Membrane Protein 1 & 2 (LMP 1&2) yang dapat diteksi 5-7
jam setelah infeksi.
Infeksi laten yang bersifat diam dan tidak memproduksi partikel-partikel virus yang baru, dikaitkan salah satunya dengan KNF. Bentuk laten infeksi EBV pada KNF termasuk tipe II dengan karakteristik terekspresinya protein LMP disamping protein EBER dan EBNA1.
Virus EBV akan menyerang sel epitel nasofaring,
menghasilkan LMP (laten membran protein) yg mencegah sel diapoptosis. Gen LMP ditemukan hampir disebagian besar
penderita CNP
PEMERIKSAAN FISIK DAN PENUNJANG
Pemeriksaan fisik
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Inspeksi
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Palpasi
Pemeriksaan Penunjang
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Endoskopy, rinoskopi (tetapi
akan susah dilihat, soalnya sudah membengkak)
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MRI
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CT scan
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Biopsi aspirasi (pemeriksaan serologic)
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Biopsi nasofaring (periksaan histologi)
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Tes antigen EBV
STADIUM
Table. TNM classification
Primary tumor (T)
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TX
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Primary
tumor cannot be assessed
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T0
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No
evidence of primary tumor
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Tis
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Carcinoma
in situ
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T1
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Tumor
confined to the nasopharynx, or tumor extends to oropharynx and/or nasal
cavity without parapharyngeal extension (eg, without posterolateral
infiltration of tumor)
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T2
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Tumor with
parapharyngeal extension (posterolateral infiltration of tumor)
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T3
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Tumor
involves bony structures of skull base and/or paranasal sinuses
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T4
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Tumor with
intracranial extension and/or involvement of cranial nerves, hypopharynx, or
orbit, or with extension to the infratemporal fossa/masticator space
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Regional lymph nodes (N)
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NX
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Regional
nodes cannot be assessed
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N0
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No
regional lymph node metastasis
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N1
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Unilateral
metastasis in cervical lymph nodes =6cm in greatest dimension, above the
supraclavicular fossa, and/or unilateral or bilateral retropharyngeal lymph
nodes =6 cm in greatest dimension (midline nodes are considered ipsilateral
nodes)
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N2
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Bilateral
metastasis in cervical lymph nodes =6cm in greatest dimension, above the
supraclavicular fossa (midline nodes are considered ipsilateral nodes)
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N3
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Metastasis
in a lymph node >6cm and/or to the supraclavicular fossa (midline nodes
are considered ipsilateral nodes)
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N3a
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>6cm in
dimension
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N3b
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Extension
to the supraclavicular fossa
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Distant metastasis (M)
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M0
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No distant
metastasis
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M1
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Distant
metastasis
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Histologic
grade (G)
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GX
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Grade cannot be assessed
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G1
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Well
differentiated
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G2
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Moderately differentiated
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G3
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Poorly
differentiated
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G4
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Undifferentiated
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Stage
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T
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N
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M
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0
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Tis
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N0
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M0
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I
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T1
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N0
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M0
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II
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T1
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N1
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M0
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T2
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N0
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M0
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T2
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N1
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M0
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III
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T1
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N2
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M0
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T2
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N2
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M0
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T3
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N0
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M0
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T3
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N1
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M0
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T3
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N2
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M0
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IVA
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T4
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N0
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M0
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T4
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N1
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M0
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T4
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N2
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M0
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IVB
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T Any
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N3
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M0
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IVC
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T Any
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N Any
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M1
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TERAPI
The usual treatment for these early stage cancers is radiation therapy aimed at the
nasopharyngeal tumor.
Although the cancer has not yet spread to lymph nodes
in these stages, the nearby lymph nodes in the neck are usually treated with
radiation therapy as well. This is considered preventive (prophylactic)
radiation. Some patients may have cancer cells in these lymph nodes that can’t
be detected. Although there are too few cancer cells in the lymph nodes to
cause them to be enlarged, these cells could continue to grow and spread if not
destroyed by radiation therapy.
Stages II, III, IVA and IVB
These cancers have spread outside of the nasopharynx
and often to lymph nodes in the neck or above the collarbone. Patients with
these stages of NPC usually receive chemoradiation (chemotherapy given along with radiation therapy to the
nasopharynx and neck lymph nodes). The chemotherapy (chemo) drug most often
used is cisplatin, but sometimes another drug is used as well. This is usually
followed by more chemo, most often with cisplatin plus 5-FU. Most studies have
found that chemoradiation helps patients live longer than just radiation
therapy alone. But adding chemo leads to more side effects, which can affect quality
of life. It’s important to understand what the side effects are likely to be
before starting this treatment.
If cancer is still in the lymph nodes after this
treatment, surgery (neck dissection) may be done to
remove the lymph nodes.
Stage IVC
These cancers have spread to distant parts of the body
and can be hard to treat. The usual treatment is chemo, often with cisplatin and one other
drug. If there is no sign of the cancer after chemotherapy, radiation therapy to the
nasopharynx and the lymph nodes in the neck or chemoradiation is given to try
to kill any remaining cancer cells. Another option in some cases is to give
chemoradiation as the first treatment.
If there are still signs of cancer after the initial
chemotherapy, another chemotherapy regimen using different drugs may be tried.
Chemotherapy plus the targeted drug cetuximab (Erbitux) may be another option.
Because these cancers can be hard to treat
effectively, taking part in a clinical trial of newer treatments may be a good
option.
Recurrent nasopharyngeal cancer
Cancer is called recurrent when it come backs
after treatment. Recurrence can be local (in or near the same place it started)
or distant (spread to organs such as the lungs or bone). If your cancer returns
after treatment, the choices available to you depend on the location and extent
of the cancer, which treatments were used the first time around, and your
overall health. It is important to understand the goal of any further treatment
– whether it is to try to cure the cancer, to slow its growth, or to help
relieve symptoms – as well as the likelihood of benefits and risks.
Some tumors that recur in the nasopharynx can be
removed by surgery using an approach through the nose
(called endoscopic skull base surgery). This is a specialized surgery
that should only be done by a surgeon with a great deal of experience in this
procedure, so it’s not available at all medical centers.
Recurrent NPC in regional (neck area) lymph nodes can
sometimes be treated by additional radiation therapy. But if
doctors believe that more radiation would cause serious side effects or if the
initial response to radiation was incomplete, surgery (neck dissection) may be
used instead.
Cancer that recurs in distant sites is usually treated
with chemotherapy. If chemotherapy has been given
already, different chemo drugs may be tried. The targeted drug cetuximab may be
given along with chemo, generally on a clinical trial.
New drug treatments being tested in clinical trials
and new surgical procedures may help some patients with recurrent NPC, as well
as improve knowledge that can help others with NPC in the future.
If the cancer can’t be cured, further treatments may
be aimed at slowing its growth or relieving symptoms caused by the distant
spread of the cancer. For example, if the cancer has spread to the spine,
radiation therapy may be given to the area to relieve pain and reduce the
chances of further complications. Even if a cure is not possible, it is
important to remember that there are many options to
relieve symptoms of advanced cancer. (http://www.cancer.org/cancer/nasopharyngealcancer/detailedguide/nasopharyngeal-cancer-treating-by-stage)
Bisa juga lewat imunoterapi
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