KBB ve BBC Dergisi 19 (2):80-3, 2011
Extracranial Meningioma in Maxillary Sinus: Case Report
Maksiller Sinüste Ekstrakranial Meningiom: Olgu Sunumu
* Sinan BAŞOĞLU, MD, * Hale ASLAN, MD, * Ali Ekber İLKNUR, MD, * Yılmaz ÖZKUL, MD, ** Mine TUNAKAN, MD, * Sedat ÖZTÜRKCAN, MD, * Hüseyin KATILMIŞ, MD
* Atatürk Research and Training Hospital, Department of Otorhinolaryngology and Head and Neck Surgery, ** Atatürk Research and Training Hospital, Ministry of Health, Department of Pathology, İzmir
ABSTRACT
Paranasal sinus meningiomas are seen very rarely and called extracranial or ectopic meningiomas, although meningiomas are the second most common tumors of the central nervous system. Most of them are located in the head and neck, whereas paranasal meningiomas are very rare. Total surgical excisi-on is the excisi-only treatment method. However, the locatiexcisi-on and spread of the mass may lead to surgical difficulties. This report presents a 24-year old woman admitted with the complaint of severe pain in the upper teeth and underwent a total excision after being diagnosed with a right maxillary meningioma. The clinical features, diagnosis, differential diagnosis, and therapy of paranasal sinus extracranial meningiomas were discussed with a literature review.
Keywords
Meningioma; maxillary sinus
ÖZET
Paranasal sinus meningiomaları etiyolojisi bilinmeyen ve çok nadir görülen tümörlerdir. Total cerrahi eksizyon, şu anda bilinen tek tedavi yöntemdir. Ancak kitlenin yerleşimi ve yayılımı cerrahi olarak zorluklara yol açabilir. Bu bildiride üst dişlerde şiddetli ağrı yakınması ile başvuran ve yapılan incelemeler so-nucunda sağ maksiller ekstrakranial meningioma tanısı koyularak total eksizyon uygulanan 24 yaşında bayan olgu sunuldu. Bu olgu nedeniyle paranasal sinus ekstrakranial meningiomaları kliniği, tanısı, ayırıcı tanısı ve tedavisi literatür eşliğinde tartışıldı.
Anahtar S zc kler
Menenjiyom; maksiller sinüs
Presented at the 7thTurkish Academy Congress, Antalya, Turkey, April 9-12, 2009.
Çalıșmanın Dergiye Ulaștığı Tarih: 11.05.2010 Çalıșmanın Basıma Kabul Edildiği Tarih: 18.10.2010
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Correspondence Sinan BA O LU, MD Atatürk Research and Training Hospital, Department of Otorhinolaryngology and Head and Neck Surgery,
İzmir, Turkey E-mail: basoglusinan@gmail.com
Extracranial Meningioma in Maxillary Sinus: Case Report 81
Turkiye Klinikleri J Int Med Sci 2008, 4 81
IN TRO DUC TI ON
e nin gi o mas are se cond most com mon be nign tu mors of the cen tral ner vo us system, ma inly af fec ting the in trac ra ni al are a.1The ones
detec ted in the ex trac ra ni al are a are re fer red to as ex trac ra -ni al me -nin gi o mas, which oc cur very ra rely. One to two per cent of all me nin gi o mas af fect the ex trac ra ni al are a and they mostly re si de in the he ad and neck. On the con-trary, pa ra na sal si nus me nin gi o mas are ex tre mely ra re.2,3
Des pi te cer ta in the o ri es, the ir eti o logy re ma ins unk nown. In the di ag no sis, the his to pat ho lo gi cal exa mi na ti ons are ne ces sary and the only ac cep ted tre at ment met hod is the sur gi cal re mo val of the mass.
CA SE RE PORT
Our ca se is a 24-ye ar-old wo man ad mit ted to our cli nic with the comp la ints of swel ling on the right si de of her fa ce, pa in on her up per te eth and na sal bloc ka ge in the right nos tril. Her signs and symptoms had star ted 10 months ago and gra du ally in cre a sed. It was fo und that the re was a swel ling in the right ma xil lary are a, which led to ob vi o us fa ci al asy mmetry as well as right exoph thal mos. The exa mi na ti on re ve a led a sig ni fi cant thin ning in the an te ri or wall and flo or of the ma xil lary si nus. In the en dos co pic na sal exa mi na ti on, it was ob ser-ved that the an te ri or wall of the ma xil lary si nus was pus-hed medially, nar ro wing the na sal ca vity in the ab sen ce of any ve ge ta ti ve mas s. The com pu te ri zed to mog rapy (CT) scan of the pa ra na sal si nu ses re ve a led a 60 x 50 mm isohyper den se mass fil ling the right ma xil lary si
-nus and ob li te ra ting the right na sal ca vity. The tu mor con ta i ned cal ci fi ca ti ons in its in ter nal struc tu re, in di ca -ting con trast me di um re ten ti on and des tro yed bo ne an-te ri orly and in fe ri orly (Fi gu re 1a, 1b). A trans na sal en dos co pic bi opsy sho wed that the mass was a me nin -got he li al me nin gi o ma in di ca ting os se o us me tap la si a (Fi-gu res 2a, 2b, 3). In the ope ra ti on per for med thro ugh the ca ni ne fos sa using the en dos co pic ins tru ments, the mass was fre ed from the an te ri or, pos te ri or and la te ral walls and ex ci sed to tally with its cap su le. The CT scan sho-wed no re cur ren ce on the post-ope ra ti ve 12thmonth
fol-low-up (Fi gu re 4). DIS CUS SI ON
Me nin gi o mas are the most fre qu ent be nign tu mors de tec ted in the cen tral ner vo us system, ac co un ting for 13-26% of pri mary in trac ra ni al tu mors.4Ho we ver, ex
t-rac ra ni al me nin gi o mas are very ra re and only 1-2% of all me nin gi o mas are ex trac ra ni al. They are cli ni cally clas si fi ed in to two types as pri mary and se con dary, ba -sed on the ana to mi cal re la ti on bet we en the in trac ra ni al and ex trac ra ni al are as.5Our ca se’s tu mor was con si de
-red as a pri mary me nin gi o ma.6,7
Fo ur the o ri es are pro po sed re la ti ed to the ori gin of pa ra na sal si nus me nin gi o mas:
1) Arach no id cell re si du es, get ting out of the cen-tral ner vo us system du ring clo su re of ne u ral tu bes in the emb ryo lo gi cal pe ri od, 2) Ec to pic arach no id gra nu la ti -ons and arach no id cell clumps ob ser ved ac ross the cra-ni al ner ves I, II, V, IX, and XI and at du ral pe net ra ti on po ints, 3) Me tap las tic trans for ma ti ons of Schwann cells,
Figure 1a, b. The CT scan shows the iso-hyperdense mass, which occupies the right maxillary sinus and obliterates the right nasal cavity, containing calcifi-cations in its internal structure, indicating contrast medium retention. The mass causes destruction on the anterior and inferior bony structures.
KBB ve BBC Dergisi 19 (2):80-3, 2011 82
and 4) Dif fe ren ti a ti ons of mul ti-po tent me sench ymal cells.2,3,6,7
Ex trac ra ni al me nin gi o mas are di ag no sed with his -to pat ho lo gi cal exa mi na ti ons display fo ur pat terns as me nin got he li al, fib rob las tic, tran si ti o nal, and an gi ob -las tic.8,9Most pa ra na sal si nus me nin gi o mas are me nin
-got he li al.10The his to pat ho lo gi cal exa mi na ti on of our
ca se al so iden ti fi ed a me nin got he li al me nin gi o ma. The me nin gi o mas are usu ally so lid, cir cu lar, and well-de fi ned lo bu lar tu mors. The symptoms ap pe ar de pen ding on its pres su re on the re ta i ned si nus and anato mic for ma ti ons aro und the si nus. The se are slowgro -wing tu mors and the ti me bet we en de tec ti on of the first symptom and di ag no sis is rat her long and can ex tend to as long as 15 ye ars.6In the pre sent ca se, a so lid, wellde
-fi ned, lo bu lar, and he morr ha gic tu mor was de tec ted. The pa ti ent was ad mit ted with the swel ling on the right si de of her fa ce, fa ci al asy mmetry and right exoph thal mos. Be si des, her signs and symptoms had star ted and gra-du ally in cre a sed 10 months be fo re the di ag no sis.
The dif fe ren ti al di ag no sis of pa ra na sal si nus me-nin gi o mas sho uld inc lu de fib ro us dyspla si a, si nus mu-co ce les or mu mu-cop yo ce les, and si nus car ci no mas.10
Furt her mo re, it is a well known fact that ne u ro fib ro ma -to sis (NF), a ge ne tic di se a se, oc curs -to get her with the me nin gi o mas in so me ca ses. Ne u ro fib ro ma to sis is clas-si fi ed in to two subt ypes and NF-2 typi cally oc curs with ex trac ra ni al me nin gi o mas, aco us tic ne u ro ma, and ot her cen tral ner vo us system tu mors.11The re we re no signs
and symptoms re la ted to NF in our ca se.
The only tre at ment of the ex trac ra ni al me nin gi o -ma is to tal sur gi cal ex ci si on of the tu mor. The sur gi cal ap pro ach must be de ter mi ned ta king in to ac co unt the
lo-ca ti on of the tu mor, the in vol ved si nus, and the ex tent of the tu mor. Ex trac ra ni al me nin gi o mas are re por ted to be ra di ore sis tant; the re fo re, ra di ot he rapy sho uld be con si -Figure 2. a) Meningothelial meningioma on the left and respiratory epithelium on the right side, b) Tumor cells are largely uniform with oval nuclei forming whorls (Hematoxylin and eosin, X200)
Figure 3. Immunoreactivity for epithelial membrane antigen (EMA) in tumor cells and respiratory epithelium (Immunohistochemical staining for EMA, X200).
Extracranial Meningioma in Maxillary Sinus: Case Report 83
Turkiye Klinikleri J Int Med Sci 2008, 4 83
de red only for the pal li a ti ve tre at ment in non-to tally ex-ci sed and re cur rent tu mors. It has be en shown that the in ci den ce of re cur ren ce in me nin gi o mas va ri es de pen -ding on the his to pat ho lo gi cal type of the tu mor. In the an gi ob las tic types, the re cur ren ce ra te is hig her be ca u -se of the dif fi culty of to tal re -sec ti on of the tu mor.8The
pre ven ti on of the re cur ren ce de pends on the re sec ti on
of the tu mor to tally. The prog no sis of ex trac ra ni al me-nin gi o mas is very go od. Alt ho ugh re cur ren ce is not re-por ted af ter to tal sur gi cal ex ci si on, the ca ses must be kept un der clo se ob ser va ti on. In the pre sent ca se, sur gi -cal ex ci si on co uld be per for med to tally and the re was no re cur ren ce at the 12thmonth fol lowup exa mi na ti
-on.
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2. Kers his nik M, Cal len der DL, Bat sa kis JG. Ex trac ra ni al, ex-tras pi nal me nin gi o mas of the he ad and neck. Ann Otol Rhi nol Lary ngol 1993;102(12):967-70.
3. Shu ang sho ti S, Pan yat ha ni a R. Ec to pic me nin gi o mas. Arc Oto lary ngol 1973;98(2):102-5.
4. Kle i hu es P, Lo u is DN, Sche it ha u er BW, Ror ke LB, Re i fen -ber ger G, Bur ger PC et al. The WHO clas si fi ca ti on of tu mors of the ner vo us system. J Ne u ro pat hol Exp Ne u rol 2002:61(3): 215-25.
5. Lin gen MW, Ra o SM, Hut ten MC, Pel zer HJ. Pri mary ec to pic me nin gi o ma of the ma xil lary si nus: ca se re port and re vi -ew of the li te ra tu re. He ad Neck 1995;17(3):258-62.
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9. Ho KL. Pri mary me nin gi o ma of the na sal ca vity and pa ra na -sal si nu ses. Can cer 1980;46(6):1442–7.
10. Da nes hi A, Asg ha ri A, Bah ramy E. Pri mary me nin gi o ma of the eth mo id si nus: A ca se re port. ENT Jo ur nal 2003;82(4): 310-1. 11. Hol land K, Ka ye AH. Spi nal tu mors in ne u ro fib ro ma to sis-2: ma na ge ment con si de ra ti ons-a re vi ew. J Clin Ne u ros ci 2009;16(2):169-77.
