Laryngotracheobronchial papillomatosis: findings on computed tomography scans of the chest*

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1 Cse Series Lryngotrcheoronchil ppillomtosis: findings on computed tomogrphy scns of the chest* Ppilomtose lringotrqueorônquic: spectos em tomogrfi computdorizd de tórx Edson Mrchiori 1, Cesr de Arujo Neto 2, Gustvo Souz Portes Meirelles 3, Klus Loureiro Irion 4, Gláuci Znetti 5, Isrel Missrie 6, Julin Sto 7 Astrct Ojective: To present the findings of computed tomogrphy (CT) scns of the chest in ptients with lryngotrcheoronchil ppillomtosis. Methods: We retrospectively nlyzed CT scns of eight ptients, five mles nd three femles, rnging from 5 to 18 yers of ge with men ge of 10.5 yers. Imges were independently reviewed y two rdiologists. In discrepnt cses, consensus ws reched. Results: The most common CT findings were intrtrchel polypoid lesions nd pulmonry nodules, mny of which were cvitted. Conclusions: In ptients with lryngotrcheoronchil ppillomtosis, the most common tomogrphic finding ws the comintion of intrtrchel polypoid lesions nd multiple pulmonry nodules, mny of which were cvitted. Keywords: Tomogrphy, X-ry computed; Ppillom; Trchel neoplsms. Resumo Ojetivo: Apresentr os chdos em tomogrfi computdorizd (TC) de tórx em pcientes com ppilomtose lringotrqueorônquic. Métodos: Form estudds, retrospectivmente, s TCs de oito pcientes, cinco msculinos e três femininos, com iddes vrindo de 5 18 nos, com médi de 10,5 nos. Os exmes form nlisdos por dois rdiologists, de form independente, e s decisões finis form otids por consenso. Resultdos: Os chdos mis comuns ns TCs form s lesões nodulres d trquéi e os nódulos pulmonres, muitos com escvção. Conclusões: O specto tomográfico mis freqüentemente oservdo nos csos de ppilomtose lringotrqueorônquic foi ssocição de lesões polipóides de trquéi com múltiplos nódulos pulmonres, vários deles escvdos. Descritores: Tomogrfi computdorizd por rios X; Ppilom; Neoplsis d trquéi. Introduction Recurrent respirtory ppillomtosis, previously known s childhood lryngel ppillomtosis is typiclly enign nd self-limiting disese. (1) It is cused y infection of the upper respirtory trct with the humn ppillomvirus (HPV), (2,3) resulting in the formtion of ppilloms, which re the most common enign tumors occurring in the respirtory trct during childhood. (4,5) Although the disese is more common in children, it cn lso occur in dults. (3) It is typiclly restricted to the lrynx ut cn lso invde the trcheoronchil tree nd the pulmonry prenchym. (5,6) Centrl irwy involvement is seen in 2% to 5% of ptients with lryngel ppilloms, wheres smll irwy or lveolr involvement occurs in less thn 1%. (3,7) The disseminted form is lso known s lryngotrcheoronchil ppillomtosis. The definitive dignosis of lryngotrcheoronchil ppillomtosis is mde sed on the results of trnsronchil iopsy (ronchoscopy) of lryngel or trchel lesions, lthough the * Study crried out in the Rdiology Deprtments of the Fluminense Federl University, Niterói, Brzil, the Federl University of Rio de Jneiro, Rio de Jneiro, Brzil, the Federl University of Bhi, Slvdor, Brzil, nd the Federl University of São Pulo, São Pulo, Brzil, s well s of the Royl Liverpool nd Brodgreen University Hospitls, Liverpool, Englnd. 1. Full Professor. Rdiology Deprtment. Fluminense Federl University, Niterói, Brzil. 2. Assistnt Professor of Rdiology. Federl University of Bhi, Slvdor, Brzil. 3. Federl University of São Pulo, São Pulo, Brzil. 4. Consulting Thorcic Rdiologist. The Royl Liverpool nd Brodgreen University Hospitls, Liverpool, Englnd. 5. Professor of Clinicl Medicine. Petrópolis School of Medicine, Petrópolis, Brzil. 6. Physicin. Deprtment of Dignostic Imging. Federl University of São Pulo, Pulist School of Medicine, São Pulo, Brzil. 7. Resident. Deprtment of Otorhinolryngology nd Hed nd Neck Surgery. Federl University of São Pulo, São Pulo, Brzil. Correspondence to: Edson Mrchiori. Ru Thomz Cmeron, 438, Vlpriso, CEP , Petrópolis, RJ, Brsil. Tel E-mil: edmrchiori@gmil.com Finncil support: None. Sumitted: 23 Mrch Accepted, fter review: 25 April 2008.

2 Lryngotrcheoronchil ppillomtosis: findings on computed tomogrphy scns of the chest 1085 dignosis cn e suspected on the sis of computed tomogrphy (CT) findings. Here, we present the tomogrphic spects oserved in eight ptients with lryngotrcheoronchil ppillomtosis. Methods This ws retrospective study nlyzing CT scns of the chest relted to eight ptients dignosed with lryngotrcheoronchil ppillomtosis t five medicl fcilities in three Brzilin sttes (Rio de Jneiro, São Pulo nd Bhi). Five ptients were mle, nd 3 were femle. Ages rnged from 5 to 18 yers (men, 10.5 yers). In ll of the cses, the dignosis hd een mde some yers prior sed on findings of ppilloms in the upper irwys. Five of the eight ptients hd een less thn two yers old t the time of dignosis. All of the ptients hd undergone multiple previous resections In the four cses in which the resections hd een quntified, the numer rnged from 15 to 40 (men, 25). In one ptient, mlignnt degenertion, to squmous cell crcinom, ws reported. The most common clinicl complint ws horseness, which occurred in six cses. Other findings c Figure 1 - ) Slice t the level of the trche. Trchel wll with irregulr orders nd polypoid formtion on the left. Homogeneous nodule in the left upper loe. ) Slice t the level of the min ronchi. Multiple, thickwlled cvitted nodules in the right lung. Figure 2 - ) Slice t the level of the upper loes. Trche with irregulr wlls. Voluminous cystic formtion in the right upper loe. ) Slice t slightly lower level thn tht shown in. Note the etter view of the cystic lesion, which hs irregulr internl wlls. c) Slice t the level of the min ronchi, showing thin-wlled cystic lesion in the lingul.

3 1086 Mrchiori E, Arujo Neto C, Meirelles GSP, Irion KL, Znetti G, Missrie I et l. included ronchospsm (in two), dyspne (in two), recurrent respirtory infections (in two), respirtory filure (in two), dry cough (in one) nd purulent expectortion (in one). Seven of the eight ptients hd een sumitted to trcheostomy, t some point during the evolution of the disese, to remove the ostruction. In ll cses, the CT scns were performed when the ptients presented involvement of the upper irwys, especilly of the lrynx. Seven ptients hd presented trchel nd pulmonry involvement; one hd presented trchel involvement lone. The CT scns of the chest were performed using different vrious tomogrphy scnners, xil slices rnging from 5 to 10 mm in thickness, in 10-mm increments, during deep inhltion, from the pices to the lung ses. Some ptients were lso sumitted to high-resolution CT, with slices of 1 mm or 2 mm in thickness. Tests were performed using prenchyml window, with c Figure 3 - ) Slice with medistinl window, upper lung fields. Polypoid lesion on the posterior wll of the trche. nd c) Slices t the level of the lower loes. Multiple, multiloulted nodulr lesions, mny cvitted, some with thick, irregulr wlls nd others with thin wlls. Note the signs of ir trpping. Figure 4 - ) Multiple res of flow impirment ffecting the entire circumference of the trche. ) Slice t the level of the ronchil ifurction. Vrious cvitted nodulr lesions in oth lungs, with tendency towrd confluence on the left. Note the mss with irregulr orders in the right lung, s well s the nodulr formtion in the right min ronchus.

4 Lryngotrcheoronchil ppillomtosis: findings on computed tomogrphy scns of the chest 1087 ptients presented involvement of the pulmonry prenchym. In ll eight cses, there were cvitted polypoid lesions, with irregulr internl orders nd wlls of vrious thicknesses, multiloulted in six cses nd presenting confluence of the lesions in five (Figures 1 through 5). Solid nodules were seen in six ptients. An ir-fluid level ws oserved in only one ptient. The lesions were predominntly in the lung ses in three ptients nd in the middle thirds in two, wheres they were evenly distriuted in two. In one ptient, the lesions were ccompnied y mss, nd, in nother ptient, they were ccompnied y consolidtion. In the ltter cse (Figure 5), there ws mlignnt degenertion in multiple lesions. Signs of ir trpping were lso oserved in one cse. There were no identified instnces of lymph node enlrgement or pleurl effusion. Discussion Figure 5 - ) Slice t the level of the ronchil ifurction, showing multiple, multiloulted cystic lesions with tendency towrd confluence. ) Slice t lower level, showing prenchymtous consolidtion on the left. In this ptient, some lesions susequently presented mlignnt degenertion. width of 1,000 1,500 Hounsfield units (HU) nd center etween 500 nd 750 HU. Tests were lso performed using medistinl window, with width of HU nd center etween 40 nd 60 HU. The HRCT nlysis ws performed y two independent oservers, nd discordnt results were resolved y consensus. The study included the ssessment of lesions in the trche or min ronchi, s well s of ccompnying prenchyml lesions. Results In ll eight cses, the CT scns reveled polypoid formtions in the trche. In one cse, injury to one of the min ronchi ws identified. Seven Lryngotrcheoronchil ppillomtosis is rre, enign disese in children nd is even more rrely seen in dults. The histologicl presenttion is enign squmous epithelil strtifiction, nd the disese is typiclly restricted to the lrynx. However, it cn occsionlly ecome ggressive, resulting in persistent or recurrent involvement of the nsophrynx, lrynx nd trcheoronchil tree. Dissemintion to the pulmonry prenchym occurs in less tht 1% of cses. Such dissemintion cn occur yers fter the dignosis of lryngel ppillom. (4) There hve een no reports of the disese ffecting the trcheoronchil tree without prior upper irwy involvement. (7) All of the ptients evluted in the present study hd een dignosed yers erlier through trcheoronchil iopsy (vi ronchoscopy). The etiologicl gent of lryngotrcheoronchil ppillomtosis is HPV, (1,4-6) which is virus tht presents gret genetic vrition, vrious types nd sutypes hving een geneticlly defined. (4) The types most often ssocited with lryngotrcheoronchil ppillomtosis re HPV-6 nd HPV-11, followed y HPV-16 nd HPV-18. These lst two types hve more often een ssocited with mlignnt trnsformtion, prticulrly with trnsformtion to squmous cell crcinom. (4-7) Infection occurs most frequently t irth nd is relted to pssge through the irth cnl in mothers with vulvr ppillomtosis lesions. (1-4,7) However, infection cn lso occur lter

5 1088 Mrchiori E, Arujo Neto C, Meirelles GSP, Irion KL, Znetti G, Missrie I et l. in life, possily y sexul trnsmission (orl contct with infected externl genitli). (3,7) The forms of lryngotrcheoronchil ppillomtosis re clssified s childhood or dult. The childhood form occurs in ptients less thn 20 yers of ge, presenting multiple lesions tht re unpredictle in terms of their response to tretment, nd the rtes of recurrence tend to e high. In this popultion, solitry ppilloms re rre. Among dults, (ptients over 20 yers of ge t dignosis), the disese is more common in men, nd the ppilloms re typiclly solitry, present high degree of inflmmtory rectivity, do not usully disseminte nd recur less frequently thn those seen in the childhood form. (6) Vrious hypotheses hve een formulted in order to explin distl dissemintion of lryngel ppillomtosis: contiguous spred; diffuse virl contmintion; multicentric origin of the ppilloms; nd cnliculr dissemintion of frgments. (2,3,6) Itrogenic fctors, such s lryngoscopy, ronchoscopy, trcheostomy nd surgicl mnipultion cn lso cuse dissemintion of ppilloms to the distl portion of the trcheoronchil tree. (6,7) In our study, seven ptients hd undergone trcheostomy, t some point during the evolution of the disese, in order to remove n ostruction. In children, the clinicl profile chrcteristic of lryngel ppillomtosis is the trid of progressive horseness, stridor nd difficulty in rething. In dults, horseness is the most common finding. (6) In the trche, the most common symptoms frequently mimic ostructive pulmonry diseses such s sthm nd chronic pulmonry ostructive disese, which cn impede the dignosis nd led to errors in the tretment strtegy. (6,8) The physicl exmintion cn revel wheezing, stridor, tchypne nd ccessory muscle recruitment during respirtion. (8) Peripherl dissemintion cn led to recurrent pneumoni, s well s ostructive telectsis nd mlignnt degenertion. Clinicl symptoms cn include fever, cough, hemoptysis nd progressive dyspne. (2,8) In our smple, the most common symptom ws horseness. The idel method for dignosing lesions in the centrl irwys is ronchoscopy, which mkes it possile to otin iopsy smples of such lesions in order to perform histopthologicl evlution nd to pln therpeutic interventions. (8) In most cses, lryngotrcheoronchil ppillomtosis is esily dignosed, since the pulmonry presenttion is typiclly preceded y rich clinicl history nd previous dignosis of lryngel ppillomtosis. (4,6) In the ronchoscopy, the ppilloms re seen s whitish polypoid lesions in the lrynx, trche or ronchi. (3) Bronchoscopy remins the dignostic method of choice, since it is oth dignostic nd therpeutic, llowing the resection of the lesions, which cn then e sumitted to ntomopthologicl study. Chest X-rys cn occsionlly produce findings suggestive of the disese, such s the comintion of solid or cvitted pulmonry nodules nd vegettive nodulr lesions in the trche or in the min ronchi. Unlike the pulmonry nodules, which re often identified on simple chest X-rys, intrlumen ppilloms in the trche or ronchi re rrely visile on X-rys. (4) In prctice, therefore, the disese is rrely dignosed on the sis of chest X-ry findings. The nodules re typiclly multiple, well defined, of vrious dimensions, cvitted nd thick-wlled, eing more numerous in the sl nd posterior lung regions. (3,4) The cvitted nodules cn e ir-filled or, when infected, cn present n irfluid level. (4) Chest X-rys re inferior to CT scns of the chest, especilly those performed using spirl CT (volumetric cquisition), in the initil phses of pulmonry dissemintion, due to the fct tht CT etter chrcterizes nd llows etter visuliztion of nodulr trcheoronchil vegettion, Tomogrphic findings include focl or diffuse irwy nrrowing cused y the nodules. The nodules form on the mucosl surfce, nd their invsion into the lumen is est evluted using CT. (3) These ltertions re esily correlted with the disese when there is clinicl history of ppillomtosis. (4) Other findings relted to irwy ostruction nd ccompnying infections re telectsis, consolidtions, ir trpping nd ronchiectsis. (2-4) In our smple, polypoid formtions in the trche were oserved on ll of the CT scns Seven ptients presented involvement of the pulmonry prenchym, chrcterized y cvitted polypoid lesions with irregulr internl orders nd wlls of vrious thicknesses, with multiloulted spect nd tendency towrd confluence. Solid nodules were seen in six ptients. None of the ptients presented lymph node enlrgement or pleurl effusion. Ppilloms pper s msses or nodules (single or multiple) tht re exophytic (sessile or pedunculted), soft nd frile, most often seen on the vocl

6 Lryngotrcheoronchil ppillomtosis: findings on computed tomogrphy scns of the chest 1089 cords, ventriculr folds, suglottis nd lryngel surfce of the epiglottis. (6) Histologiclly, ppilloms present s projections of the strtified squmous epithelium with firovsculr centers. Hyperplsi of sl cells nd lrge vcuolted epithelil cells with cler cytoplsm re typicl findings. (6,7) When the lesion invdes the trcheoronchil tree, the epithelium cn e squmous or cilited nd cylindricl. (6) The pulmonry lesions re foci of squmous epithelium tht grow circumferentilly within the lveoli, depending on them for vsculriztion. Ner the centrl portion of solid or cvitted lesion, there re res necrosis nd regenertion, contining frgments. In the periphery, squmous cells invde the djcent lveoli y direct extension of of the principl cell mss, lymphocytes nd mcrophges eing identified in the lveolr content. These cells grow, colesce nd destroy the prenchym, forming cvities. (4) Mlignnt degenertion to squmous cell crcinom is reported in 1% to 10% of ll cses of lryngotrcheoronchil ppillomtosis, (3,4) typiclly occurring fter rdiotherpy or chemotherpy with leomycin, s well s in ptients with history of smoking. (1,6) It cn occur in childhood or even decdes fter the dignosis of enign ppilloms. (4) In most cses, it occurs in ptients hving previously presented dissemintion of the disese to the trcheoronchil tree. Mlignnt degenertion rrely occurs in ptients with the lryngel form of the disese. (2,5) In one of the cses evluted here, there ws mlignnt degenertion reported sed on iopsy findings in three different lesions. Smll lesions provoking miniml symptoms cn e treted with corticosteroids nd ntiiotics. Lrger, symptomtic lesions extirpted through ronchoscopic procedures such s curettge, lser ltion, electrocutery nd cryosurgery, surgicl intervention (thorcotomy or sternotomy) rrely eing necessry. (8) Rdiotherpy, utogenic vccintion nd chemotherpy hve proven unsuccessful. (3,6) Antivirl therpy hs lso een employed. (3) None of the vrious tretment protocols hs een shown to e truly efficcious. (1,4) Recurrence of ppilloms is common, regrdless of the tretment given. In prctice, it is necessry to sumit such ptients to frequent ronchoscopic exmintions. This disese tkes on greter importnce due to the severity of its evolution nd the fct tht it ffects children nd dolescents, resulting in considerle moridity nd hving profound impct on ptient qulity of life, requiring multiple surgicl excisions nd trcheostomies; the prognosis is unfvorle, nd the disese cn even evolve to deth. (4) Although the dignosis is typiclly mde in childhood, sed on finding of ppilloms in the upper irwys, susequent finding of intrtrchel polypoid lesions, with or without pulmonry nodules (cvitted or not), should rise the suspicion of the disese. References 1. Rdy PL, Schndig VJ, Weiss RL, Hughes TK, Tyring SK. Mlignnt trnsformtion of recurrent respirtory ppillomtosis ssocited with integrted humn ppillomvirus type 11 DNA nd muttion of p53. Lryngoscope. 1998;108(5): Chng CH, Wng HC, Wu MT, Lu JY. Virtul ronchoscopy for dignosis of recurrent respirtory ppillomtosis. J Formos Med Assoc. 2006;105(6): Prince JS, Duhmel DR, Levin DL, Hrrell JH, Friedmn PJ. Nonneoplstic lesions of the trcheoronchil wll: rdiologic findings with ronchoscopic correltion. Rdiogrphics. 2002;22 Spec No:S Errtum in: Rdiogrphics. 2003;23(1): Arújo Neto CA, Cmpos RM, Bstos ML. Ppilomtose respirtóri recorrente com disseminção pulmonr - relto de dois csos. Rdiol Brs. 2002;35(2): Wilde E, Duggn MA, Field SK. Bronchogenic squmous cell crcinom complicting loclized recurrent respirtory ppillomtosis. Chest. 1994;105(6): Rd MH, Alizdeh E, Ilkhnizdeh B. Recurrent lryngel ppillomtosis with ronchopulmonry spred in 70-yer-old mn. Tuerk Torks. 2007;55(3): Frnzmnn MB, Buchwld C, Lrsen P, Blle V. Trcheoronchil involvement of lryngel ppillomtosis t onset. J Lryngol Otol. 1994;108(2): Bldi BG, Fernndes CJ, Slge JM, Tkgki TY. Trchel polyp. J Brs Pneumol. 2007;33(5):