KBB ve BBC Dergisi 19 (1):51-4, 2011
Turkiye Klinikleri J Int Med Sci 2008, 4 51
Oral Mature Teratoma: Report of a Rare Disease
Oral Matür Teratom: Nadir Bir Hastalık
*Üzeyir GÖK, MD, *İsrafil ORHAN, MD, **Bengü ÇOBANOĞLU, MD, *Emrah SAPMAZ, MD * Fırat University Medical Faculty, Otorhinolaryngology Department,
** Fırat University Medical Faculty, Pathology Department, Elazığ
ABSTRACT
Teratomas arising from the oral cavity are rare. Most cases appear in the midline and paraxial locations; the sacrococcygeal region being the most com-mon site (40% to 60%). Only 10% of teratomas originate from the head, neck and central nervous system. Oropharyngeal teratomas represent 2% of all teratomas. They develop in neonates and are associated with feeding difficulty and airway obstruction. Although some tumors consist of immature elements, teratomas of the head and neck are mature in nature, and surgical excision is the most effective treatment. We present a neonate with a polypoid mass ori-ginating from the anterior hard palate and protruding out of the mouth. The mass, which was completely excised, was histologically identified as a mature teratoma.
Keywords
Teratoma; palate, hard; feeding behavior
ÖZET
Oral kavite teratomları oldukça nadirdir. Hastaların çoğunda orta hatta ve paraksiyal lokalizasyonda olup sakrokoksigeal bölge en sık yerleştiği bölgedir (%40 ile %60). Teratomların sadece %10’u baş, boyun ve santral sinir sisteminden gelişir. Orofarengeal teratomlar tüm teratomların %2’sini oluşturur. Ye-nidoğanlarda beslenme zorluklarına ve hava yolu obstrüksüyonlarına sebep olurlar. Bazıları immatür hücreler içerse de baş ve boyun teratomları matür ka-rakterde olup eksizyon en önemli tedavi yöntemidir. Bu çalışmada sert damağın ön kısmından kaynaklanan ve ağız dışına taşan polipoid kitlesi olan bir yenidoğan olguyu sunuyoruz. Tamamen çıkarılan kitlenin histolojik sonucu matür teratom olarak rapor edildi.
Anahtar Sözcükler
Teratom; sert damak; beslenme davranışı
Çalıșmanın Dergiye Ulaștığı Tarih: 14.03.2009 Çalıșmanın Basıma Kabul Edildiği Tarih: 22.05.2009
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Correspondence İsrafil ORHAN, MD
Fırat Üniversitesi Tıp Fakültesi Hastanesi, KBB Kliniği, 23200, Elazığ, TÜRKİYE
Phone: +90 424. 233 35 55 Fax: +90 424. 238 76 88 E-mail: israfil.orhan@mynet.com
KBB ve BBC Dergisi 19 (1):51-4, 2011
52
IN TRO DUC TI ON
e ra to mas are de ri ved from mul tip le tis su es fo re -ign to the or gan or si te from which they ari se. They are the most com mon ex tra go na dal germ cell tu mors of child ho od and best de fi ned as be nign tu-mors that con ta in all thre e ger mi nal la yers: ec to derm, me so derm and en do derm.1-3Te ra to ma is a ra re le si on
that oc curs in ap pro xi ma tely in 1:4000 births.4Most ca
ses ap pe ar in the mid li ne and pa ra xi al lo ca ti on; the sac -ro cocc yge al re gi on is the most com mon si te (40% to 60%).4Two to 10% of ca ses are fo und in the he ad and
neck re gi on,4,5es pe ci ally at the cer vi cal and na sop hary
-nge al re gi ons. Alt ho ugh the re is a 6:1 fe ma le to ma le pre pon de ran ce in ot her si tes, they occur equal in both genders in the he ad and neck re gi on.6Of the he ad and
neck te ra to mas, pu re oral pre sen ta ti on is ra re.
We re port a ne o na te with a te ra to ma ori gi na ting from the hard pa la te and ca u sing fe e ding prob lems. Lo -cal Et hics Com mit te e ap pro val was ob ta i ned.
CA SE RE PORT
A 3300-gr ma le in fant born of a 32-ye ar-old mot her by va gi nal de li very af ter 37 we eks of ges ta ti on pre sen ted with a pe dun cu la ted mass prot ru ding out of his mo -uth (Fi gu re 1). The mot her had not had ul tra so nog raphic exa ma i na ti ons du ring her preg nancy be ca u se of so ci o -cul tu ral re a sons. On ini ti al exa mi na ti on, the baby was only two ho urs old and the mass was prot ru ding out si de of the mo uth. The baby was not ab le to suck his mot her’s bre ast. The mass was 5x4 cm in di a me ter and ori gi na ted from the an te ri or hard pa la te, ne ar the mid li ne. The mass was at tac hed to the oral ca vity with a pe dunc le 15 mm in di a me ter. It ham pe red fe e ding wit ho ut ca u sing res pi ra -tory dis tress. Na sal and na sop hary nge al en dos copy was nor mal. The re were no as so ci a ted abnormalities. We did not ob ta in fi ne ne ed le as pi ra ti on bi opsy, com pu te ri zed to mog rapy or mag ne tic re so nan ce ima ging. Our pre o pe -ra ti ve di ag no sis was epu lis. An ex ci si o nal bi opsy was plan ned. The mass was to tally ex ci sed and ble e ding was con trol led un der lo cal anest he si a on the first day. Af ter the sur gery, the hard pa la te re ma i ned in tact. We did not use a fe e ding tu be be ca u se the baby star ted to suck his mot her’s bre ast af ter the sur gery and he was disc har ged from the hos pi tal on the se cond postoperative day. His -to pat ho lo gic exa mi na ti on re ve a led a ma tu re te ra -to ma (Fi gu re 2). The pa ti ent sho wed no signs of re cur ren ce in a follow up period of one year.
DIS CUS SI ON
Con ge ni tal te ra to mas, which oc cur in 1 of 4000 li ve births, are di ag no sed pre do mi nantly in fe ma le ne o na tes.4,7
Most ca ses ap pe ar in the mid li ne and pa ra xi al lo ca ti ons, the sac ro cocc yge al re gi on being the most com mon si te (40% to 60%).4Only 10% of te ra to mas ori gi na te in the
he ad, neck, and cen tral ner vo us system.8Orop hary nge al
te ra to mas rep re sent 2% of all te ra to mas.9,10Te ra tomas ari
-sing from the oral ca vity are ra re in the new borns; only 13 ca ses ha ve be en re por ted in the li te ra tu re.11
Te ra to mas usu ally de ve lop from pri mor di al germ cells and are composed of tissues fo re ign to the si te of in-vol ve ment. The re are at le ast thre e hypot he ses for the ori gin of te ra to mas: part he no ge ne sis, in comp le te twin-ning, and to ti po tent so ma tic cell ori gin.12Alt ho ugh a te
r-a to mr-a is cli ni cr-ally de fi ned r-as hr-a ving three embryologic
Figure 1. The neonate with oral teratoma.
Fi gu re 2. His to pat ho lo gic exa mi na ti on sho wed a be nign tu mo ur which inc lu -ded skin, epi der mal ap pen dix, car ti la gi no us tis su e, li po ma to us struc tu re and musc le tis su e (HE X40).
la yers, re cent clas si fi ca ti ons al so inc lu de mo no der mal types. He ad and neck te ra to mas are clas si fi ed in to four types: der mo id which con ta ins epi der mal and me so der -mal ele ments is the most com mon type; te ra to id which con sists of the ec to derm, me so derm, and ec to der mal el-e mel-ents is po orly dif fel-e rel-en ti a tel-ed; tru el-e tel-e ra to ma which con-ta ins all three germ cell la yers dif fe ren ti a tes in to a re cog ni zab le early or gan; and epig nat hus which is highly dif fe ren ti a ted in to re cog ni zab le or gans or limbs is very ra re and as so ci a ted with a high mor ta lity ra te. It is sug-ges ted that the le si on de ve loped by pinc hing off the rem-nants of three ger mi nal la yers con si de red to be mar gi na ted in the nor mal mig ra tory path way as the struc-tu res of the he ad and neck de ve lo ped. Ma lig nant trans-for ma ti on of te ra to ma oc curs in ap pro xi ma tely 20% of ca ses and is mo re com mon in adults. Germ cells in ma-lig nant te ra to ma are bi zar re col lec ti ons of the epit he li al and me sench ymal tis su es. The ma ni fes ta ti on of the se ne op las tic le si ons is ag gres si ve lo cal in fil tra ti on; many me -tas ta si ze.13
The pre na tal di ag no sis of tu mors of the he ad and neck is very im por tant. Early di ag no sis enab les a ca re -fully plan ned de li very du ring which an open air way can be ma in ta i ned to im pro ve pe ri na tal out co me.14It sho uld
be kept in mind that a lar ge obs truc ting mass may be fa -tal, even if it was di ag no sed pre na tally.15Be ca u se air way
obs truc ti on can ca u se se ve re comp li ca ti ons, Ce sa re an sec ti on is the pre fer red met hod of de li very for in fants with an obs truc ting mass of the he ad or neck. Im me di a -tely be fo re the ter mi na ti on of fe to ma ter nal cir cu la ti on, the se in fants sho uld un der go in tu ba ti on by the oral or na -sal ro u te. So me ne o na tes with a hu ge obs truc ting mass may re qu i re trac he os tomy in the de li very ro om.14
Chil dren with pu re oral te ra to mas se em to ha ve less dra ma tic res pi ra tory be ha vi or com pa red to chil dren with ot her he ad and neck pre sen ta ti ons. When oral te ra to mas
grow, they tend to prot ru de out si de of the mo uth, rat her than pos te ri orly to ward the orop harynx. Be ca u se new-borns are ob li ga te na sal bre at hers, obs truc ti on of the oral com part ment is re la ti vely less im por tant and usu ally ca -u ses less -ur gent fe e ding prob lems.12In ad di ti on, chil dren
with oral te ra to mas ha ve be en re por ted to ha ve as so ci a ted abnormalities inc lu ding cleft pa la te, cystic hygro -ma,16and ot her mul ti fo cal te ra to mas.17,18In our ca se, no
as so ci a ted ano ma li es we re fo und.
Alt ho ugh ra di o lo gic ima ging with ul tra so nog raphy, com pu ted to mog raphy, or mag ne tic re so nan ce ima ging and tes ting for the le vel of α-fe top ro te in are help ful for iden tif ying te ra to mas, the di ag no sis must be ba sed on his to lo gic examination. In our ca se, pre o pe ra ti ve di ag -no sis was epu lis, but his to pat ho logy re ve a led a ma tu re te ra to ma. Sur gi cal ex ci si on is the tre at ment of cho i ce for oral te ra to mas. A re vi ew of the li te ra tu re in di ca ted that to tal re sec ti on co uld be per for med in most pa ti ents, and re cur ren ce af ter that pro ce du re was ra re. Con ge ni -tal te ra to mas are ge ne rally ma tu re in na tu re.8
Oral te ra to mas are ex tra go na dal germ cell tu mors that oc cur very ra rely du ring in fancy and child ho od. Chil dren with oral te ra to mas ha ve less se ve re symptoms when com pa red to tho se with orop hary nge al, na sop -hary nge al and cer vi cal te ra to mas. Alt ho ugh all re por ted pu re oral te ra to mas are be nign, there is a risk of ma lig -nant chan ge, and long-term fol low-up is man da tory even if the tu mor is to tally ex ci sed with fre e mar gins. The pre na tal di ag no sis of tu mors of the he ad and neck is very im por tant. Early di ag no sis enab les a ca re fully plan ned de li very du ring which an open air way can be ma in ta i ned to im pro ve pe ri na tal out co me. It sho uld be kept in mind that a lar ge obs truc ting mass may be fa tal, even if it was di ag no sed pre na tally. Therefore, all mot -hers must ha ve ul tra so nog rap hic exa ma i na ti ons du ring the ir preg nancy.
Oral Mature Teratoma: Report of a Rare Disease 53
Turkiye Klinikleri J Int Med Sci 2008, 4 53
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