Case Report. Carcinoid tumor and pulmonary sequestration* Abstract. Resumo. Introduction. Case report

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1 Cse Report Crcinoid tumor nd pulmonry sequestrtion* Tumor crcinoide e sequestro pulmonr Fernndo Luiz Westphl, Luís Crlos de Lim, José Corrê Lim Netto, Mri do Socorro Lucen Crdoso, Márci dos Sntos d Silv, Dnielle Cristine Westphl Astrct Pulmonry sequestrtion is defined s mss of lung tissue seprted from the trcheoronchil tree nd irrigted y n nomlous systemic rtery. It is rrely seen in conjunction with lung neoplsms. We report the cse of 39-yer-old femle ptient dignosed with crcinoid tumor, locted in the intermedite ronchus nd ccompnied y ronchiectsis in the right lower loe. The ptient underwent thorcotomy for the resection of the ffected re. During surgery, she presented with significnt hemorrhge resulting from the trnsection of the nomlous rtery tht irrigted n intrlor pulmonry sequestrtion, which ws locted in right lower loe nd hd not een identified in pre-opertive exmintions. Keywords: Bronchopulmonry sequestrtion; Hemorrhge; Crcinoid tumor. Resumo O sequestro pulmonr é definido como um mss de tecido pulmonr seprd d árvore trqueorônquic e irrigd por um rtéri sistêmic nôml. Su ssocição com neoplsis pulmonres é rr. Reltmos o cso de um pciente de 39 nos com o dignóstico de tumor crcinoide loclizdo no rônquio intermediário, ssocido lterções crcterizds como ronquiectsis em loo inferior direito. A pciente foi sumetid à torcotomi pr ressecção d áre cometid e, durnte cirurgi, presentou hemorrgi importnte decorrente d trnsecção d rtéri nôml que nutri o sequestro pulmonr intrlor loclizdo em loo inferior direito, não identificdo nos exmes pré-opertórios. Descritores: Sequestro roncopulmonr; Hemorrgi; Tumor Crcinoide. Introduction Pulmonry sequestrtion (PS) is rre congenitl mlformtion chrcterized y mss of nonfunctioning lung tissue tht does not communicte with the trcheoronchil tree nd is vsculrized y n nomlous systemic rtery. It is composed of cystic emryonic tissue nd contins disorgnized non-erted lveoli, s well s ronchi, crtilge, nd respirtory epithelium. (1) In 75% of cses of PS, the lood supply is derived from the thorcic or dominl ort, nd venous dringe is vi the systemic veins or vi the pulmonry vein. Accidentl trnsection of n nomlous systemic rtery cn cuse mssive hemorrhge with ftl consequences, nd it is of prmount importnce tht nomlous vessels e identified in the preopertive period. (2) The ojective of the present study ws to report n intropertive finding of PS following hemorrhge resulting from the trnsection of n nomlous pulmonry vessel during resection of crcinoid tumor. Cse report A 39-yer-old femle ptient presented with history of recurrent pneumoni, together with spordic episodes of productive cough nd fever. In the two preceding yers, the ptient hd hd four episodes of moderte volume * Study crried out in the Deprtment of Clinicl Surgery, Federl University of Amzons School of Medicine, Mnus, Brzil. Correspondence to: Fernndo Luiz Westphl. Hospitl Universitário Getúlio Vrgs, Coordenção de Ensino e Pesquis. Avenid Aripunã, 4, Prç 14 de Jneiro, CEP , Mnus, AM, Brsil. Tel E-mil: f.l.westphl@uol.com.r Finncil support: None. Sumitted: 4 August Accepted, fter review: 31 August 2010.

2 134 Westphl FL, Lim LC, Netto JCL, Crdoso MSL, Silv MS, Westphl DC mlformtion, PS, congenitl lor emphysem, ronchogenic cysts, nd pulmonry rteriovenous mlformtions. The reported nnul incidence of these mlformtions rnges from 30 to 42 cses per 100,000 popultion, PS ccounting for % of ll cses. (3,4) Depending on its pleurl covering, PS is clssiclly divided into intrlor nd extrlor. An intrlor PS (ILPS) shre the pleurl covering with the rest of the lung, wheres n extrlor PS (ELPS) is completely covered y its own viscerl pleur. (5) Although the extrlor form is well defined s congenitl normlity, the intrlor form hs controversil pthogenesis, with some evidence indicting tht, in mny cses, it is n cquired disese. (6,7) Accounting for pproximtely 75% of ll cses of PS, ILPS is more common in the lower loes nd on the left, involving the posterior sl segment. (5,8) Its rteril supply is nerly lwys derived from the ort or from one of its rnches, typiclly one of lrge dimeter. Venous dringe is vi the pulmonry veins into the left trium, creting left-to-left shunt. In minority of cses, dringe is vi the inferior ven cv or vi the zygos system. (9) In the cse presented here, the PS ws locted in the right lower loe nd the lood supply cme from n nomlous rnch derived from the thorcic ort. Cses of ILPS typiclly occur in dolescents nd young dults with history of recurrent infections of the respirtory trct, hemoptysis, nd dyspne. Some ILPS ptients develop crdic symptoms, which re consequence of the lefthemoptysis. In the preopertive investigtion, CT scn of the chest reveled lesion in the intermedite ronchus (Figure 1), s well s cystic ronchiectsis in the right lower loe (Figure 2). Fieroptic ronchoscopy confirmed the tomogrphic finding of wine-colored, exophytic endoronchil lesion t the root of the intermedite ronchus, nd iopsy of the lesion reveled typicl crcinoid tumor. Clinicl resoning sed on those findings suggested ronchiectsis resulting from chronic ronchil ostruction. The ptient underwent right posterolterl thorcotomy with iloectomy (middle nd lower loes). During the intropertive period, sectioning of the pulmonry ligment resulted in hemorrhgic process originting from n nomlous rteril vessel tht hd irrigted the prenchym of the PS (Figure 3). Hemostsis ws chieved y ligtion of the vessel. Histopthology results were consistent with the finding of PS. The postopertive course ws stisfctory, without ny complictions, nd the ptient ws dischrged on postopertive dy 5. Discussion Despite eing defined s distinct entities, lung mlformtions constitute spectrum of normlities, with quite similr clinicl presenttions, rising from flws in the development of the primitive intestine nd its differentition into respirtory system during the emryonic period. (2) Chief mong the most common mlformtions re cystic denomtoid Figure 1 - Presence of lesion in the intermedite ronchus, lmost completely ostructing the ronchil lumen.

3 Crcinoid tumor nd pulmonry sequestrtion 135 Figure 2 - In A, CT scn of the chest reveling cystic res permeted y firotic tissue in the right lower loe. In B, cystic res in the posterior segment of the right lower loe. to-left shunt nd of the high flow diverted to the nomlous vessel.(10) The recurrent episodes of infection cuse firosis nd viscerl pleurl thickening. The lung prenchym shows res of consolidtion nd firosis contining multiple cystic formtions, which cn e mistken for ronchiectsis,(5) s in the cse reported here. In generl, n ELPS is locted in the posterior costodiphrgmtic recess, etween the lower loe nd the left hemidiphrgm. More rrely, it cn occur in the medistinum or in the dominl region. An ELPS is typiclly irrigted y n rtery originting directly from the ort, nd, in 80% of cses, venous dringe is systemic, occurring vi the zygos-hemizygos system or vi the superior ven cv, which cretes left-toright shunt.(2,5) Unlike in cses of ILPS, clinicl mnifesttions pper in the first six months of life in cses of ELPS. Neontl sphyxi, dyspne, cynosis, nd feeding difficulties re common. More rrely, recurrent episodes of respirtory trct infection or gstrointestinl symptoms cn occur.(2,11) Cses of ELPS cn e ccompnied y pulmonry, crdic, nd verterl mlformtions, s well s y mlformtions of the chest wll nd gstrointestinl trct, eing most commonly ccompnied y diphrgmtic herni.(12) On chest X-rys, PS cn e initilly identified s mss of homogeneous opcity. Dignostic confirmtion is mde through chest CT, mgnetic resonnce imging, or rteriogrphy. Chest CT is more useful in detecting normlities in the lung prenchym nd only in some cses cn it identify PS nomlous irrigtion. Arteriogrphy is the est test for dignostic confirmtion of PS, ecuse it detects the nomlous rtery irrigting the PS with precision. More recently, reconstruction with multichnnel CT scnners Figure 3 - In A, systemic rtery, which rnched from the thorcic ort, irrigting the pulmonry sequestrtion. In B, intermedite ronchus contining n exophytic lesion.

4 136 Westphl FL, Lim LC, Netto JCL, Crdoso MSL, Silv MS, Westphl DC nd venous contrst hs llowed definitive dignosis ecuse of its enhnced cpcity to depict communiction etween the nomlous rtery nd the PS. (10,13) The tretment for PS is surgicl resection of the sequestered loe or segment y thorcotomy or even y video-ssisted thorcoscopy. In oth cses, the success of the procedure depends on dequte knowledge of the vsculr ntomy of the PS nd on erly ligtion of the rtery irrigting the PS, ecuse, s previously stted, ccidentl trnsection of tht rtery cn led to mssive hemorrhge, with ftl consequences. (4,14) In the cse reported here, there ws significnt hemorrhge, ut it ws contined y mss clmping nd susequent ligtion with continuous sutures. There hve een few reports of PS ccompnied y lung cncer, nd there hs een only one cse in which the histologicl sutype ws crcinoid tumor. (15) In report pulished in 1985, the PS nd the crcinoid tumor were locted in the left lower loe. (16) In the cse presented here, the PS nd the crcinoid tumor were locted in different loes, ut oth were locted in the right lung. A crcinoid tumor is rre type of neuroendocrine tumor, derived from enterochromffin cells, nd occurs primrily in the gstrointestinl trct. When crcinoid tumor occurs in the ronchopulmonry system, it cn mnifest s recurrent episodes of pneumoni, cough, hemoptysis, nd chest pin. (16) In the cse reported here, the mjor symptom experienced y the ptient ws hemoptysis, nd there ws history of recurrent pneumoni. These two chrcteristics re common to PS nd to crcinoid tumors, fct tht mde the preopertive dignosis of PS difficult, ecuse the symptoms of the ptient were ttriuted to the neoplsm nd to chronic ostruction. Despite eing rre, pulmonry mlformtions cn present similrly to more common infectious pulmonry diseses, such s ronchiectsis. Therefore, we emphsize the importnce of ncillry investigtion in ptients with history of recurrent pulmonry infections nd cystic chnges in imging studies, ecuse these prolems cnnot lwys e ttriuted to single, more evident cuse. Since PS is rre pulmonry mlformtion nd hs sutle clinicl mnifesttions, it requires tests tht re more complex, nd, if unsuspected, it might pper only s n intropertive finding following potentilly ftl complictions. References 1. Slmons S. Pulmonry sequestrtion. Neontl Netw. 1995;14(6): Felker RE, Tonkin IL. Imging of pulmonry sequestrtion. AJR Am J Roentgenol. 1990;154(2): Ferreir HP, Fischer GB, Felicetti JC, Cmrgo Jde J, Andrde CF. Surgicl tretment of congenitl lung mlformtions in peditric ptients. J Brs Pneumol. 2010;36(2): Vn Remdonck D, De Boeck K, Devlieger H, Demedts M, Moermn P, Coosemns W, et l. Pulmonry sequestrtion: comprison etween peditric nd dult ptients. Eur J Crdiothorc Surg. 2001;19(4): Aey P, Ds CJ, Pngtey GS, Seith A, Dutt R, Kumr A. Imging in ronchopulmonry sequestrtion. J Med Imging Rdit Oncol. 2009;53(1): Clements BS, Wrner JO. Pulmonry sequestrtion nd relted congenitl ronchopulmonry-vsculr mlformtions: nomenclture nd clssifiction sed on ntomicl nd emryologicl considertions. Thorx. 1987;42(6): Stocker JT, Mlczk HT. A study of pulmonry ligment rteries. Reltionship to intrlor pulmonry sequestrtion. Chest. 1984;86(4): Hlkic N, Cuénoud PF, Corthésy ME, Ksontini R, Boumghr M. Pulmonry sequestrtion: review of 26 cses. Eur J Crdiothorc Surg. 1998;14(2): Ncif MS, Lim Filho HS, Mello RA, Juregui GF, Mirnd BJ, Crmel JM, et l. Seqüestro roncopulmonr intrlor: relto de cso. Rdiol Brs. 2005;38(1): Pêgo-Fernndes PM, Freire CH, Jtene FB, Beyruti R, Suso FV, Oliveir SA. Seqüestro pulmonr: um série de nove csos operdos. J Pneumol. 2002;28(4): Mezzcpp MA, Binchi MO, Furtdo PL, Srgi Neto L, Alvres BR. Derrme pleurl no recém-nscido: um mnifestção incomum de seqüestro pulmonr extrlor. Rev Pul Peditri. 2006;24(1): Sous A, Cost J, Silv LJ. Sequestro pulmonr com dignóstico pré-ntl. Cso clínico. Act Peditr Port. 2007;38(3): Pugliese JG, Bártholo TP, Sntos HT, Sito EH, Cost CH, Rufino R. Usefulness of chest CT in the dignosis of pulmonry sequestrtion. J Brs Pneumol. 2010;36(2): Wn IY, Lee TW, Sihoe AD, Ng CS, Yim AP. Videossisted thorcic surgery loectomy for pulmonry sequestrtion. Ann Thorc Surg. 2002;73(2): Okmoto T, Msuy D, Nkshim T, Ishikw S, Ymmoto Y, Hung CL, et l. Successful tretment for lung cncer ssocited with pulmonry sequestrtion. Ann Thorc Surg. 2005;80(6): Pinchot SN, Holen K, Sippel RS, Chen H. Crcinoid tumors. Oncologist. 2008;13(12):

5 Crcinoid tumor nd pulmonry sequestrtion 137 Aout the uthors Fernndo Luiz Westphl Coordintor of the Teching nd Reserch Center. Getúlio Vrgs University Hospitl, Federl University of Amzons School of Medicine, Mnus, Brzil. Luís Crlos de Lim Physicin-in-Chief. Deprtment of Thorcic Surgery, Getúlio Vrgs University Hospitl, Federl University of Amzons School of Medicine, Mnus, Brzil. José Corrê Lim Netto Attending Physicin. Deprtment of Thorcic Surgery, Getúlio Vrgs University Hospitl, Federl University of Amzons School of Medicine, Mnus, Brzil. Mri do Socorro Lucen Crdoso Professor of Pulmonology. Federl University of Amzons School of Medicine, Mnus, Brzil. Márci dos Sntos d Silv Physicin. Getúlio Vrgs University Hospitl, Federl University of Amzons School of Medicine, Mnus, Brzil. Dnielle Cristine Westphl Medicl Student. Federl University of Amzons School of Medicine, Mnus, Brzil.