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Sebutan lain untuk angiofibroma di dalam literatur antara lain: juvenile angiofibroma,
juvenile nasopharyngeal angiofibroma (JNA), nasal cavity tumor, nasal tumor, benign nasal
tumor, tumor hidung (nose tumor), nasopharyngeal tumor, atau angiofibroma nasofaring
belia. 6
Sumber : Pradhana D. 2009. Juvenile Nasopharyngeal Angiofibroma. Referat Kepaniteraan
Klinik Ilmu Penyakit THT Fakultas Kedokteran Universitas Trisakti. Jakarta.
EPIDEMIOLOGI
Frequency
Juvenile nasopharyngeal angiofibroma (JNA) accounts for 0.05% of all head and neck
tumors. A frequency of 1:5,000-1:60,000 in otolaryngology patients has been reported.
Sex
Onset is most commonly in the second decade; the range is 7-19 years. Juvenile
nasopharyngeal angiofibroma (JNA) is rare in patients older than 25 years.
Umumnya terdapat pada rentang usia 7 s/d 21 tahun dengan insidens terbanyak antara usia
14-18 tahun dan jarang pada usia diatas 25 tahun. Tumor ini merupakan tumor jinak
nasofaring terbanyak5 dan 0,05% dari seluruh tumor kepala dan leher. Dilaporkan insidennya
antara 1 : 5.000 1 : 60.000 pada pasien THT.
JNA banyak dialami terutama remaja putra berusia 14-18 tahun. Jika remaja putri didiagnosis
JNA, maka sebaiknya menjalani pemeriksaan kromosom atau diagnosis JNA akan terus
dipertanyakan. Umumnya JNA terjadi pada dekade kedua kehidupan, tepatnya pada rentang
usia 7-19 tahun. JNA jarang terjadi setelah usia 25 tahun. Insiden JNA adalah 1 dari 500060.000 kasus THT dan dilaporkan 0,5% dari semua tumor kepala dan leher. Dilaporkan
insiden JNA banyak terjadi di Mesir dan India.
Insiden dari angiofibroma tinggi dibeberapa bagian dari belahan dunia, seperti pada Timur
Tengah dan Amerika. Martin, Ehrlich dan Abels (1948) melaporkan rata-rata setiap tahunnya
dari satu atau dua pasien untuk 2000 pasien yang diobati pada Head and Neck Service of The
Memorial Hospital, New York. Di London, Harrison (1976) mencatat status dari satu per
15000 pasien pada Royal National Throat, Nose and Ear Hospital dimana satu kesimpulan
bahwa lebih sedikit angiofibroma di London dibanding di New York.
Pradillo dkk melaporkan bahwa persentasi pasien dengan usia lebih dari 25 tahun hanya 0.7%
dari semua pasien angiofibroma nasofaring sedangkan jumlah kasus di RS M.Djamil
Padangbagian THT-KL, Juli 2008 Desember 2010 berjumlah 9 orang dengan usia antara
13-21 tahun.(4,5)
Sumber :
1. Soepardi, Efiaty Arsyad, dkk. 2007. Buku Ajar Ilmu Kesehatan Telinga, Hidung,
Tenggorok, Kepala & Leher Edisi Ke-enam. Jakarta : FKUI
2. Asrole, Harry. 2002. Angiofibroma Nasofaring Belia. Medan : FKUSU
3. Hajar, Siti. 2005. Angiofibroma Nasofaring Belia. Majalah Kedokteran Nusantara
Vol.38 No.3 September 2005 : p 251-253
4. Pradillo, et al. Nasopharyngeal Angiofibroma in The Elderly: Report of a Case.
Laryngoscope 1974:1063-65.
5. Sukri Rahman, Bestari J Budiman, Surya Azani. Angiofibroma Nasofaring Pada
Dewasa Bagian Telinga Hidung Tenggorokan Bedah Kepala Leher (THT-KL)
Fakultas Kedokteran Universitas Andalas Padang
ETIOLOGI
Penelitian lain menunjukkan adanya faktor pertumbuhan yang memediasi proliferasi agresif
sel stromal dan angiogenesis. Transforming Growth Factor-1 (TGF-1) atau faktor
pertumbuhan pengubah-1 adalah polipeptida yang disekresikan dalam bentuk inaktif, dipecah
untuk menghasilkan bentuk aktif, dan kemudian tidak diaktifkan dalam jaringan. TGF-1
mengaktifkan proliferasi fibroblas dan dikenal sebagai induksi angiogenesis. TGF-1 aktif
diidentifikasi pada sel nukleus stromal dan sitoplasma dan pada endotelium kapiler pada
semua spesimen angiofibroma nasofaring juvenile.
Etiology from nonchromaffin paraganglionic cells of the terminal branches of the maxillary
artery has also been suggested. Comparative genomic hybridization analysis of these tumors
revealed deletions of chromosome 17, including regions for the tumor suppressor gene p53 as
well as the Her-2/neu oncogene.
Sumber :
KLASIFIKASI
Different staging systems exist for nasopharyngeal angiofibroma. The 2 most commonly used
are those of Sessions and Fisch.
Stage IIIA - Erosion of skull base (ie, middle cranial fossa/pterygoid base);
minimal intracranial extension
Stage III - Tumors invading infratemporal fossa, orbit and/or parasellar region
remaining lateral to cavernous sinus
GEJALA KLINIS
Symptoms
Nasal obstruction (80-90%) - Most frequent symptom, especially in initial stages
Epistaxis (45-60%) - Mostly unilateral and recurrent; usually severe epistaxis that
necessitates medical attention; diagnosis of angiofibroma in adolescent males to be
ruled out
Signs
Proptosis (10-15%)
Other signs include serous otitis due to eustachian tube blockage, zygomatic swelling,
and trismus that denote spread of the tumor to the infratemporal fossa, decreasing
vision due to optic nerve tenting (rare)
Gejala
1. Obstruksi nasal (80-90%) dan ingus (rhinorrhea). Ini merupakan gejala yang paling sering,
terutama pada permulaan penyakit.
2. Sering mimisen (epistaxis) atau keluar cairan dari hidung yang berwarna darah (bloodtinged nasal discharge). Mimisen, yang berkisar 45-60% ini, biasanya satu sisi (unilateral)
dan berulang (recurrent).
3. Sakit kepala (25%), khususnya jika sinus paranasal terhalang.
primer di dalam nasofaring dapat meluas ke palatum, rongga hidung, orofaring dan basis
kranii. Gejala klinis yang paling sering dirasakan adalah adanya benjolan di leher.
Keluhan lain dapat berupa epistaksis, hidung tersumbat, otitis media, telinga berdenging
dan tuli. Karsinoma nasofaring merupakan keganasan dengan karakteristik variasi
distribusi geografis dan etnis, terutama di Asia Tenggara. Gambaran radiologi karsinoma
nasofaring adalah asimetri fossa Rosenmuller, hilangnya lapisan lemak di parapharyngeal
space, destruksi tulang dan penebalan preoccipital space.16,17,18
Sumber :
PENATALAKSANAAN
Pembedahan adalah pilihan utama untuk angiofibroma nasofaring. Teknik pembedahan
ditentukan oleh lokasi tumor, perluasan tumor dan kemampuan ahli bedahnya.2 Beberapa
pendekatan operasi yaitu pendekatan transpalatal, transzygomatik, transmandibular,
transhioid, transantral : rinotomi lateral, midfasial degloving, pendekatan nasoendoskopi dan
kraniotomi 2,19 Spector 12 mengemukakan pilihan operasi secara transpalatal yang
dikombinasi dengan rhinotomi lateral pada tumor yang sudah meluas ke etmoid dan
retroorbita. Ahmed seperti yang dikutip oleh Garca5 mengatakan bahwa teknik transpalatal
digunakan untuk tumor yang berada di nasofaring, sphenoid, foramen sphenopalatina dan
nares posterior.
Medical Therapy
Hormonal therapy
The testosterone receptor blocker flutamide was reported to reduce stage I and II tumors to
44%. Despite tumor reduction with hormones, this approach is not routinely used. Schuon et
al reported on the immunohistochemical analysis of growth mechanisms in juvenile
nasopharyngeal angiofibroma.[2] They concluded that juvenile angiofibroma (JNA) growth
and vascularization are driven by factors released from stromal fibroblasts. Therefore,
inhibition of these factors might be beneficial for the therapy of inoperable juvenile
nasopharyngeal angiofibroma (JNA).
Radiotherapy
Some centers have reported 80% cure rates with radiation therapy. However, concerns
regarding potential effects of radiation make radiation therapy a nonuseful modality in most
cases.
Stereotactic radiotherapy (ie, Gamma Knife) delivers a lower dose of radiation to surrounding
tissues. However, most authorities reserve radiotherapy for intracranial disease or recurrent
cases.
Conformal radiotherapy in extensive juvenile nasopharyngeal angiofibroma (JNA) or
intracranial extension provides a good alternative to conventional radiotherapy regarding
disease control and radiation morbidity, even with advanced disease.[3, 4]
Surgical Therapy
A lateral rhinotomy, transpalatal, transmaxillary, or sphenoethmoidal route is used for small
tumors (Fisch stage I or II).
The infratemporal fossa approach is used when the tumor has a large lateral extension.
The midfacial degloving approach, with or without a LeFort osteotomy, improves posterior
access to the tumor.
The facial translocation approach is combined with Weber-Ferguson incision and coronal
extension for a frontotemporal craniotomy with midface osteotomies for access.
An extended anterior subcranial approach facilitates en bloc tumor removal, optic nerve
decompression, and exposure of the cavernous sinus.
Some authors advocate the use of intranasal endoscopic surgery for lesions with limited
extension to the infratemporal fossa. Image-guided, endoscopic, laser-assisted removal has
also recently been used. Hackman et al (2009) reviewed 31 cases of JNA at the University of
Pittsburgh Medical Center from 1995 to 2006.[5] Most tumors were completely excised using
the expanded endonasal approach (EEA) alone or in combination with minor sublabial
incisions, avoiding the morbidity associated with larger open approaches or postoperative
radiation therapy.
Radical removal of a large JNA may be difficult because of its extreme vascularity and
extension to the cavernous sinus, orbit, middle fossa, and anterior fossa. Nevertheless, most
JNAs with intracranial extension can be resected in the first operation with minimal
morbidity through a facial degloving and further combination of expanded endoscopic
endonasal approaches.[6]
In a retrospective review, Battaglia et al (2014) evaluated the use of endoscopic endonasal
surgery in the radical resection of benign or nonmetastatic malignant tumors that have either
developed in or extended to the infratemporal fossa or upper parapharyngeal space.[7]
According to the investigators, the results, derived from 37 patients, including 20 with JNA,
suggested that purely endoscopic endonasal radical resection can be safely used to treat
selected tumors involving these spaces.
In a review article, Cloutier et al (2012) reported on 72 patients operated on over a 10-year
period.[8] They concluded that the progress in skull-base surgery allowed for expansion of the
indications for endoscopic removal of JNA. This approach has a better outcome in terms of
blood loss, hospital stay, and complications. Of course, an external approach should be
considered only for selected cases due to massive intracranial extension or optic nerve or
internal carotid artery entrapment by the tumor.
In a meta-analysis of the endoscopic surgical outcomes of JNA, covering 92 studies and a
total of 821 patients, Khoueir et al (2014) calculated that the mean operative blood loss from
endoscopic JNA surgery was 564.21 mL. Random effect estimates for recurrence,
complications, and residual tumor were 10%, 9.3%, and 7.7%, respectively. The authors
stated that endoscopic treatment is currently considered the treatment of choice for JNA but
also commented that they could find no randomized, controlled studies for their analysis.
They advised that future studies propose a new, endoscopic approach based classification
system.[9]
Yi et al (2013) described a simplified classification system and management option for
juvenile nasopharyngeal angiofibroma, as follows[10] :
Type I includes tumor localized in the nasal cavity, paranasal sinus, nasopharynx, or
pterygopalatine fossa. The transnasal cavity approach with endoscopic guidance is
suitable for this type.
Type II is if the lesion extends into the infratemporal fossa, cheek region, or orbital
cavity, with anterior and/or minimal middle cranial fossa extension but intact dura
mater. The transantral-infratemporal fossa-nasal cavity combined approach is reliable
for type II.
Type III is a calabashlike, massive tumor lobe in the middle cranial fossa. For type III
tumors, the complete removal is challenging. A combined extracranial and intracranial
approach is often needed. Radiotherapy is useful for treating the residual intracranial
part.