Turkiye Klinikleri J Int Med Sci 2008, 4 119
Malignant Myoepithelioma Arising from
the Submandibular Salivary Gland: A Case Report
Submandibular Tükrük Bezinde Gelişen Malign Miyoepitelyoma:
Olgu Sunumu
*Çağatay Han ÜLKÜ, MD, *Hakan KELEKÇİ, MD **Hasan ESEN, MD
* Selçuk University School of Medicine, Department of Otolaryngology-Head and Neck Surgery ** Selçuk University School of Medicine, Department of Pathology,
Konya ABSTRACT
Myoepithelioma accounts for less than 1% of all salivary gland tumors. It is generally located in the parotid gland, less often in the minor salivary glands of the oral cavity and in the submandibular gland. This tumor can be classified as benign or malignant on the basis of clinical and histological findings. Malignant myoepithelioma is even more rare, representing 0.2-0.45% of all salivary gland tumors. Only 27 cases with malignant myoepithelioma arising from a major salivary glands have previously been reported in the English literature. Diagnosis is based on histological and immunuhistochemical findings. Conservative surgery is the most accepted treatment choice. Radioterapy is only used when surgery is not considered feasible. In this study, a 61-year-old woman with submandibular gland malignant myoepithelioma was reported. Based on the English literature review, this case report represents the sixth ca-se of malignant myoepithelioma of the submandibular salivary gland.
Keywords
Myoepithelioma; carcinoma; submandibular gland
ÖZET
Miyoepitelyoma, tüm tükrük bezi tümörlerinin %1’inden azını oluşturur. Genellikle parotis bezinde, daha nadir olmak üzere oral kavitedeki minör tükrük bezleri ve submandibular tükrük bezinde lokalizedir. Klinik ve histolojik bulgular temelinde, tümör benign ve malign olarak sınıflandırılabilir. Malign mi-yoepitelyona daha da nadirdir ve tüm tükrük bezi tümörlerinin %0.2-0.45’ine karşılık gelir. İngilizce literatürde, major tükrük bezinden kaynaklanan sa-dece 27 malign miyoepitelyoma olgusu daha önce rapor edilmiştir. Tanı histolojik ve immünohistokimyasal bulgular ile konur. Konservatif cerrahi en çok kabul gören tedavi seçeneğidir. Radyoterapi sadece cerrahinin uygun olmadığı durumlarda kullanılır. Bu çalışmada, submandibular bez malign miyoepi-telyomalı 61 yaşında bir bayan tanımlanmıştır. İngilizce literatür gözden geçirildiğinde, sunulan bu olgu altıncı submandibular tükrük bezi malign miyoe-pitelyomasıdır.
Anahtar Sözcükler
Miyoepitelyom; karsinom; submandibular bez
Çalıșmanın Dergiye Ulaștığı Tarih: 17.07.2009 Çalıșmanın Basıma Kabul Edildiği Tarih: 28.12.2009
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Correspondence Çağatay Han ÜLKÜ, MD Selcuk University School of Medicine,
Department of Otolaryngology-Head and Neck Surgery, Konya Phone: + 90 – 332 - 2237250
Fax: + 90 - 332-3232643 E-mail:chanulku@yahoo.com
yo e pit he li o ma of the sa li vary gland was first des cri bed by Shel don in 1943.1Alt ho ugh it
was ini ti ally con si de red as a subt ype of ple -o m-orp hic ade n-o ma, the W-orld He alth Or ga ni za ti -on clas-si fi ed it as a dis tinct en tity in 1991.2
Myo e pit he li o ma ac co unts for less than 1% of all sa li vary gland tu mors. It is ge ne rally lo ca ted in the pa -ro tid gland, less of ten in the mi nor sa li vary gland of the oral ca vity and in the sub man di bu lar gland.2-4This tu
-mor ra rely prog res ses to ma lig nant trans for ma ti on.5
To the best of our know led ge, only fi ve ca ses of ma lig nant myo e pit he li o ma of the sub man di bu lar gland ha ve be en pre vi o usly re por ted in Eng lish li te ra tu re,3,4,6,7
and he re in we re por ted the sixth ca se.
CA SE REPORT
A 61-ye ar-old wo men was ad mit ted to our cli nic with a 48 months his tory of a slow-gro wing mass in the right sub man di bu lar re gi on. Physi cal exa mi na ti on on ini ti al pre sen ta ti on re ve a led a 4 x 5 cm di a me te red hard, ten der and se mimo bi le mass in vol ving the left sub man -di bu lar re gi on. The pa ti ent po in ted out that the vo lu me of the mass in cre a sed mo re ra pidly in the last three months and that her pa in be ca me mo re ap pa rent. The flo or of the oral ca vity and Whar ton duct ope ning we re nor mal. The re ma in der fin dings of the pa ti ent, inc lu ding ne u ro lo gic eva lu a ti on of the sen sory and mo tor func ti -on, we re wit hin nor mal li mits. Her past me di cal his tory was al so un re mar kab le.
A pre o pe ra ti ve fi ne ne ed le as pi ra ti on cyto logy (FNAC) was pe for med. It sho wed class II I cells (con ta i ned so me cells with lar ge and hyperc hro ma tic nuc le -i that -in d-i ca ted pos s-ib le ma l-ig nancy), but no spe c-i f-ic di ag no sis was re por ted. Axi al con trast-en han ced com-pu ted to mog raphy (CT) scan de mons tra ted a 50 x 40 mm, so lid, wellcir cums cri bed mass with nonho mo ge -ne o us en han ce ment in the left sub man di bu lar re gi on, and the man dib le was not in vol ved (Fi gu re 1).
The pa ti ent un der went sur gery. It was seen that the tu mor had in va ded the sur ro un ding nor mal sub man di -bu lar gland and it was ad he rent to the ne igh bo ring struc-tu res. Sa li vary gland was re sec ted with ad ja cent soft tis su es and a few re gi o nal lymph no des. Fro zen sec ti -ons re ve a led tu mor-ne ga ti ve sur gi cal mar gins.
His to pat ho logy re ve a led the mi to ti cally ac ti ve epit he lo id and poly go nal tu mor cells wepith ve si cu ler nuc -le i, dis tinct nuc -le o li, and eo si nop hi lic cytop lasm. Me anw hi le, so me tu mor cells had cle ar cytop lasms. An in va si ve growth pat tern was se en thro ugh he ma toxy lin eo sin sta in (Fi gu re 2 a, b). Tu mor cells we re im mu no -his toc he mi cally sta i ned po si ti vely with Cyto ke ra tin, Des min, EMA (Fi gu re 2c), and S100 (Fi gu re 2d). His -to pat ho logy con fir med the di ag no sis of sub man di bu lar ma lig nant myo e pit he li o ma (MME) and the re was not any lymph no de me tas ta sis.
The pa ti ent had an une vent ful postope ra ti ve pe ri -od. She was disc har ged from the cli nic on the 7ththe
post-ope ra ti ve day. No sign of di se a se was pre sent du ring the fol lowup exa mi na ti on per for med 18 months af -ter the ope ra ti on.
DIS CUS SI ON
Myo e pit he li al cells are ec to der mally de ri ved contrac ti le cells and ex hi bit a smo oth musc le and epit he li -al phe noty pe.3,7Many nor mal tis su es with a sec re tory
func ti on such as sa li vary glands, swe at glands, lac ri mal glands, bre asts and the pros ta te con ta in the se cells.1,7
Alt ho ugh the se cells are one of the most fre qu ent com po nents of many sa li vary gland tu mors, pu re myo -e pit h-e li o mas ar-e ra r-e, ac co un ting for l-ess than 1% of all sa li vary gland tu mors.1,7Myo e pit he li o mas can be
classi fi ed as be nign (BME) or ma lig nant (MME) on the ba -sis of cli ni cal and his to lo gi cal fin dings.3
Figure 1. Axi al con trasten han ced CT scan de mons tra ted a so lid, wellcir cums cri bed mass with nonho mo ge ne o us en han ce ment in the left sub man -di bu lar re gi on.
The tu mor usu ally ap pe ars as an asym pto ma tic mass that slowly in cre a ses in si ze over a pe ri od of se v-e ral months or yv-e ars for BME.8
MME is even mo re ra re, rep re sen ting 0.2-0.45% of all sa li vary gland tu mors. Ap pro xi ma tely 55 MME ca ses ha ve be en re por ted in the Eng lish li te ra tu re.3Only
27 ca ses of ma lig nant myo e pit he li o ma of the ma jor sa -li vary gland and five sub man di bu lar MME ha ve be en pre vi o usly re por ted in the Eng lish li te ra tu re.3,4,6,9
Alt ho ught ma lig nant myo e pit he li o mas are ge ne rally re gar ded as a lowgra de ma lig nant tu mors, they so -me ti -mes show ag gres si ve fe a tu res.10Mac ros co pi cally,
the le si on may in va de the sur ro un ding tis su es and pro-du ce both lo cal and dis tant me tas ta ses. Re cur ren ce is fre qu ent.8Mic ros co pi cally, MME shows vas cu lar and
pe ri ne u ral in va si on, mar ked cel lu lar ple o morp hism,
im-mu no re ac ti vity to p53, and high pro li fe ra ti ve ac ti vity with a po or cli ni cal out co me.3
MME is mo re com monly se en in wo men at the age of 60 (with a ra ti o 2:1 ).11 The me an tu mor si ze for
MME was re por ted as 4.9 cm (ran ge from 2.5-8 cm).3
With re gard to oc cur ren ce, the ma in pri mary si te is the pa ro tid gland and the most com mon in tra-oral si te is the hard pa la te.2,10,12They so me ti mes ari se in the na sal ca
v-ity, na sop harynx, larynx and lung.12
Ma lig nant myo e pit he li o mas may ari se eit her “de no vo ” or de ve lop in a preexis ting ple o mor pic ade no -ma or BME.6,8The prog nos tic imp li ca ti on of the his to
-ge ne sis of MME is con tro ver si al.10 Ac co ur ding to
Na ga o et al.,3the re was no dif fe ren ce in prog no sis in
terms of the ab sen ce or the pre sen ce of a pre-exis ting ple o morp hic ade no ma. Ho we ver, Di Pal ma and Guz zo13
Turkiye Klinikleri J Int Med Sci 2008, 4 121
Fi gu re 2a. In vasi ve growth pa tern is se en (He ma toxy lin Eo sin [HE] x 100).
Fi gu re 2b. Mi to ti cally ac ti ve epit he lo id and poly go nal tu mo ur cells with ve si cu ler nuc le i, dis tinct nuc le o li, and eo si nop hi lic cytop lasm. So me tu mo r cells ha ve cle -ar cytop lasms (He ma toxy lin Eo sin [HE] x 400).
Fi gu re 2c. Tu mor cells are po si tivly sta i ned with EMA, (x 200). Fi gu re 2d. Tu mor cells are po si ti vely sta i ned with S-100, (x 200).
si on, they are smal ler in si ze, ha ve an in si di o us on set, and lo wer ma lig nancy ra te. MME ari sing from a pre-exis ting BME disp lays an in ter me di a te pat tern. Usu ally, de no vo MME and tho se which de ve lo ped from pre-exis ting BME ari se from the mi nor sa li vary glands.14
Ba sed on the cli ni cal co ur se and the his to lo gic fe a tu res, we spe cu la ted that the MME of the pre sen ted ca -se pos sibly aro -se from a pre-exis ting ple o mor pic ade no ma. For the ot her pre vi o usly re por ted fi ve ca ses in the li te ra tu re, MME of the sub man di bu lar gland aro -se de no vo in one, to ok ori gin from a pre-exis ting BME in one, and from a ple o morp hic ade no ma in two ca -ses.3,6,9
Ma lig nant myo e pit he li o ma in the sub man di bu lar gland de mons tra te a well-cir cums cri bed mass with an in ter nal lo bu la ted pat tern and an in ho mo ge ne o us density on con trasten han ced CT as re por ted by Ue ma e to -ma ri et al.9Ade no id cystic car ci no ma, mu co e pi der mo id
car ci no ma and aci nic cell car ci no ma sho uld be inc lu ded in ra di o lo gic (CT) dif fe ren ti al di ag no sis.4In the pre sen
ted ca se, axi al con trasten han ced CT scan analy sis de -mons tra ted a 50 x 40 mm so lid, well-cir cums cri bed mass with non-ho mo ge ne o us en han ce ment in the left sub man di bu lar re gi on.
Arc hi tec tu rally, myo e pit he li o ma of sa li vary gland disp la yed eit her so lid, myxo id, or re ti cu lar growth pat-terns. His to pat ho lo gi cally, cells of myo e pit he li o ma may be plas macy to id, spind le-sha ped, epit he li o id, cle ar or a com bi na ti on of the se.15Spind lesha ped types of myo e
-pit he li o ma ori gi na tes from the pa ro tid gland, and the plas macy to id types ari se from mi nor sa li vary glands of the pa la te.4Our ca se sho wed a com bi ned dif fe ren ti a ti on
(plas macy to id/epit he li o id/spind le-sha ped) and so lid stro ma.
Di ag no sis is ba sed on his to lo gi cal and im mu nu -his toc he mi cal fin dings. S-100 pro te in and vi men tin are not usu ally pre sent in nor mal myo e pit he li um and are
as, cyto lo gic aty pi a, high mi to tic ra te and cel lu lar ple o morp hism. Tu mo ur cells we re im mu no his toc he mi -cally po si tivly sta i ned for EMA, Cyto ke ra tin, Des min, and S-100 in our ca se. Ma lig nant myo e pit he li o ma was di ag no sed his to pat ho lo gi cally.
The ma jor dif fe ren ti al di ag no sis of myo e pit he li o -ma is a ple o morp hic ade no -ma. Myo e pit he li o -mas are com po sed comp le tely, or al most comp le tely of myo e pit he li al cells, ho we ver the amo unt is va ri ab le in the ple -o m-orp hic ade n-o ma, but may re ach le vels c-om pa rab le t-o tho se in myo e pit he li o ma.12
Re gar ding the tre at ment of MME, the re is litt le infor ma ti on ava li ab le at the pre sent ti me. Du e to the li mi -ted num ber of ca ses and the wi des pec trum of the ne op lasm, pub lis hed re sults tend to be conf lic ting.8But
con ser va ti ve sur gery is the most ac cep ted tre at ment cho -i ce.12If the re are cli ni cally ap pa rent me tas ta ses in the
cer vi cal lymph no des, the ra pe u tic neck dis sec ti on is in-di ca ted.8Ra di o te rapy is used only when sur gery is not
con si de red fe a sib le.12 Ac co ur ding to the li te ra tu re,
MME which ari ses from a pre e xis ting ple o mor pic ade-no ma, li ke the ca se pre sen ted he re, has the best cli ni cal out co me, if tre a ted pro perly.13,14
In our ca se, we re sec ted the sub man di bu lar gland with ad ja cent soft tis su es and a few re gi o nal lymph no des. As the re was no cli ni cal and his to lo gi cal lymph no -de me tas ta sis, neck dis sec ti on was not per for med. Du ring most re cent fol low-up exa mi na ti on per for med 18 months af ter the ope ra ti on, our pa ti ent was he althy and the re was no sign of the di se a se.
CONC LU SI ON
We ad vi se early tumor removal and avoidance of incomplete removal for the pre ven ti on of the de ve lop ment of MME from a preexis ting ple o morp hic ade no -ma. Long-term fol low-up is al so re com men ded.
Turkiye Klinikleri J Int Med Sci 2008, 4 123 1. Shel don W. So-cal led mi xed tu mors of the sa li vary glands.
Arch Pat hol 1943;35:1-20.
2. Se i fert G, So bin LH. The world he alth or ga ni za ti on’s his to -lo gi cal clas si fi ca ti on of sa li vary gland tu mors. A com men tary on the se cond edi ti on. Can cer 1992;70(2):379-85.
3. Na ga o T, Su ga no I, Is hi da Y, Ta ji ma Y, Mat su za ki O, Kon no A, et al. Sa li vary gland ma lig nant myo e pit he li o ma: a cli ni co -pat ho lo gic and im mu no his toc he mi cal study of ten ca ses. Can-cer 1998;83(7):1292-96.
4. Mon zen Y, Fu kus hi ma N, Fu ku ha ra T. Myo e pit he li o ma and ma lig nant myo e pit he li o ma ari sing from the sa li vary gland: com pu ted to mog raphy and mag ne tic re so nan ce fin dings. Aus-tra las Ra di ol 2007;51 Suppl:B169-72.
5. Na yak JV, Mo li na JT, Smith JC, Brans tet ter BF 4th, Hunt JL, Snyder man CH. Myo e pit he li al ne op la si a of the sub man di bu -lar gland: ca se re port and the ra pe u tic con si de ra ti ons. Arch Oto lary ngol He ad Neck Surg 2003;129(3):359-62. 6. Lac co ur re ye O, Lac co ur re ye L, Mus ca tel lo L, Cha ri al JP,
Car-not F, Bras nu D. Sub man di bu lar ma lig nant myo e pit he li o ma. Am J Oto lary ngol 1997;18(5):331-4.
7. Bar nes L, Apel BN, Perz H, El-At tar AM. Myo e pit he li o mas of the he ad and neck: ca se re port and re vi ew. J Surg On col 1985;28(1):21-8.
8. Ca rin ci F, Gras so DL, Gran di E, Pe lucc hi S, Pas to re A. Ma-lig nant myo e pit he li o ma of the ton gu e ba se: ca se re port and li te ra tu re re vi ew. J Cra ni o fac Surg 2001;12(6):544-6.
9. Ue ma e to ma ri I, Ta buc hi K, Wa da T. Ha ra A. Ku sa ka ri J. Ii ji -ma T, et al. A ca se of -ma lig nant myo e pit he li o -ma ari sing in the sub man di bu lar gland. Prac ti ca Oto lo gi ca (Kyo to) Pract Otor hi no lary ngol 2003;96(3):237-43.
10. Yos hi za ki T, Hi mi Y, Mi na to H, Oga wa I, Ni kai H, Fu ru ka wa M. Ma lig nant myo e pit he li o ma ari sing from re cur rent ple o -morp hic ade no ma of mi nor sa li vary gland. Au ris Na sus Larynx 2002;29(1):91-4.
11. Fon se ca I, So a res J. Epit he li al-myo e pit he li al car ci no ma of the sa li vary glands. A study of 22 ca ses. Virc hows Arch A Pat hol Anat His to pat hol 1993;422(5):389-96.
12. Fer ri E, Pa von I, Ar ma to E, Ca va le ri S, Ca puz zo P, Ian ni el lo F. Myo e pit he li o ma of a mi nor sa li vary gland of the che ek: ca -se re port. Ac ta Otor hi no lary ngol Ital 2006;26(1):43-6. 13. Di Pal ma S, Guz zo M. Ma lig nant myo e pit he li o ma of sa li vary
glands: Cli ni co pat ho lo gi cal fe a tu res of ten ca ses. Virc hows Arch A Pat hol Anat His to pat hol 1993;423(5):389-96. 14. Alos L, Car de sa A, Bom bi JA, Mal lof re C, Cuc hi A, Tra ser
ra J. Myo e pit he li al tu mors of sa li vary glands: a cli ni co pat -ho lo gic, im mu no his toc he mi cal, ul tras truc tu ral, and flow-cyto met ric study. Se min Di agn Pat hol 1996;13(2): 138-47.
15. Dar dick I, Tho mas MJ, van Nos trand AW. Myo e pit he li o manew con cepts of his to logy and clas si fi ca ti on: a light and elec -tron mic ros co pic study. Ul tras truct Pat hol 1989;13(2-3): 187-224.
