GonzálezLópezetal.JournalofCardiothoracicSurgery2013,8:22 http://www.cardiothoracicsurgery.org/content/8/1/22 CASE REPORT Open Access Surgical excision with left atrial reconstruction of a primary functioning retrocardiac paraganglioma María Teresa González López1*, Sergio González González1, Esteban Sarria García1, Stella González Romero2 and Julio Gutiérrez de Loma1 Abstract About 2% ofall paragangliomas are located inthe chest, and a few have been described to be found inthe heart. Primary cardiac paragangliomas are extremely uncommon tumors and surgical experience with this neoplasm is limited. Treatment strategies described in theliterature have included simple excision, excision with reconstruction, autotransplantationafter excision of the tumor and even orthotopic cardiac transplantation, dependingon the extent ofdisease.A primary retrocardiac paraganglioma catecholamine-productive was identifiedin an asymptomatic 49–year old female associated to familial pheochromocytoma-paraganglioma syndrome caused by germline mutation of thegen which codifies for the subunit Bofsuccinate dehydrogenase enzyme (SDHB).The neoplasm was surgically excised from theposterior surface of the leftatrium via median sternotomy using cardiopulmonary bypass. Direct ligation offeedingvessels ofthe tumor along with leftatrial reinforcement using a pericardial patch was performed.The post-operative course was uneventful, withnormalization of catecholamine secretion and no recurrence atthree-month follow-up. We review thecurrent literature about this exceptional cardiac tumor, pathophysiological conditions and options for surgical management. Keywords: Paraganglioma,Pheochromocytoma, Cardiac tumor, Left atrium, Cardiopulmonary bypass Background carotid and vagal bodies), urinary bladder (1%) and media- Pheochromocytomas are catecholamine-producing neu- stinalcompartments(lessthan1%)[2]. roendocrinetumorsarisefrom the chromaffincellsofthe Thoracic paragangliomas are the most uncommon loca- embryonic neural crest. It is estimated that the annual tionandtheycanbedividedintotwogroups:thoselocated incidence is approximately 0.8 per 100.000 persons and in the anterior mediastinum (arise from parasympathetic they occur most often in the fourth to fifth decade [1]. paraganglia) and located in the posterior mediastinum They are highly vascularised but usually benign tumors, (sympathetic chain). Primary cardiac pheochromocytomas although about 10% of pheochromocytomas are found to (paragangliomas) are extremely rare, occurring in only be malignant at the time of the primary tumor is 0.001%–0.003%,withfemalepredominance[3]. discovered. In general terms, the clinical manifestations of these About 85–90% of them develop in the abdomen (they tumors are varied, not particularly specific and itdepends are usually found within one or both adrenal glands) and on the secretion of excessive amounts of catecholamines, only 10% originates from extra-adrenal sites. Extra-adrenal such as noradrenaline (norepinephrine) and adrenaline pheochromocytomas (often described as extra-adrenal (epinephrine). Although these tumors may be a cause of paragangliomas)originateinthegangliaofthesympathetic hypertension, they are the underlying cause of only about nervous system and most are located within the abdomen 0.01%ofcasesofhighbloodpressure. (celiac,superiormesenteric,inferiormesentericgangliaand About75%ofpheochromocytomasaresporadicandthe organ of Zuckerkandl), head and neck (3%, including remaining 25% are hereditary. Mutations of the genes which codify for the subunits D, A, C y B of succinate dehydrogenase enzyme (SDHD, SDHA, SDHC and *Correspondence:[email protected] 1CardiovascularSurgeryDepartment,CarlosHayaRegionalHospital,Carlos SDHB) involved in the Krebs cycle can lead to tumor- HayaAvenue,s/n.29010,Málaga,Spain ogenesis in chromaffin cells and they have been Fulllistofauthorinformationisavailableattheendofthearticle ©2013GonzálezLópezetal.;licenseeBioMedCentralLtd.ThisisanOpenAccessarticledistributedunderthetermsofthe CreativeCommonsAttributionLicense(http://creativecommons.org/licenses/by/2.0),whichpermitsunrestricteduse, distribution,andreproductioninanymedium,providedtheoriginalworkisproperlycited. GonzálezLópezetal.JournalofCardiothoracicSurgery2013,8:22 Page2of8 http://www.cardiothoracicsurgery.org/content/8/1/22 identified as causing familial adrenal pheochromocy- adrenal localizations (such as those paragangliomas origi- toma and extra-adrenal paraganglioma; moreover, these nated in cardiac structures) involve an uncommon and tumorsmayalsooccurinthemultipleendocrineneoplasia complexsurgicalapproachforacompleteremoval. (MEN) syndrome type 2A and 2B, von Hippel-Lindau dis- We describe a case of surgical excision of a primary easeorvonRecklinghausen’sneurofibromatosis[4]. functioning retrocardiac paraganglioma associated with The diagnosis of these tumors can be established by hereditarysyndromeandwereviewtherelevantliterature measuringcatecholaminesandmetanephrinesinplasmaor about the peculiar features and surgical strategies for 24-hoururinealongwithimagingtechniquessuchascom- removalofthiscardiacneoplasm. puted tomography (CT), magnetic resonance imaging, iodine-123-marked metaiodobenzylguanidine (MIBG) scan Case presentation andpositronemissiontomography(PET)scan[5]. We report a case of an asymptomatic 49-year old Treatment of pheochromocytomas involves blocking Caucasian female, with hereditary type 4 pheochromocy- the effect of catecholamines with an alpha adrenergic toma-paraganglioma syndrome caused by germline blocker and after, it then becomes safe to surgically mutation (alanine–43–proline) of the gen which codifies remove the tumor. Although surgical resection is the for the subunit B of succinate dehydrogenase enzyme treatment of first choice in most part of cases, extra- (SDHB) of the mitochondrial complex II. She had a Figure1A.Computedtomographyimagingreconstructionoftheheart.Thetumorisshowed.Thecollateralvessel(whitearrow)fromthe circumflexartery(CxA)isobserved.B.18-FDOPAPETshoweda16×25×36mmextracardiacmassprotrudingwithintheLA.Presenceof metabolicactivitywasdetected.C.Computedtomography(axialview)showedthepresenceofthelargemass(redarrow).D.Coronary angiography.Thecoronarybloodsupplyofthesuperioraspectoftheparaganglioma(T)emergedfromalargecollateralvessel(whitearrow) fromtherightcoronaryartery(RCA).E.Coronaryangiography.ProximalanddistalcollateralsfromCxAwerethefeedingvessels(whitearrow)of theinferioraspectofthetumor(T). GonzálezLópezetal.JournalofCardiothoracicSurgery2013,8:22 Page3of8 http://www.cardiothoracicsurgery.org/content/8/1/22 family history of extra-adrenal paraganglioma: genetic femoral artery and venous cannulation (right femoral testing had revealed the gen SDHB mutation for eight vein and superior vein cava) (Figure 2A-B) because of first- and second-degree relatives and carotid body para- surgical excision could be technically difficult by the ganglioma had been resected in three of them. Urinary position of the tumor. Moderate hypothermia (31°C) and plasma levels of catecholamines (noradrenaline and was instaured and the heart was arrested using ante- adrenaline) and their metabolites (normetanephrine) grade cold blood cardioplegia. The tumor was found as were elevated and computed tomography of the abdo- an “imprisoned” mass between the four pulmonary menruledoutadrenalglandinvolvement. veins: it extended from the right to the left superior Total body MIBG scintigraphy scan and 18F-dihydroxy- pulmonary vein, and caudally, between the inferior phenyl-alanine (18F-DOPA) PET scan showed a primary pulmonary veins. The lesion was confined to this retro- retrocardiac mass with metabolic activity. Metastasic cardiac area as a firm adherent mass, although it was disease was not found and no other intrathoracic masses not adherent toadjacent structures. wereidentified.Transesophagealechocardiography(TOE) Direct ligation of feeding vessels was performed for and TC imaging of the heart showed the large mass control of bleeding: the collaterals vessels from the right (40×20mm),extrinsictotheheart,ontheposteriorwall coronary artery (RCA) and circumflex artery (CxA) were of the left atrium (LA) (Figure 1A-C). Coronary angio- ligated (Figure 2C-D). TOE was used to confirm success- graphy and aortography were performed: the tumor ful interruption of blood flow to the tumor and no elec- received dual blood supply from both right and left coro- tricalchangeswerenoted. nary arteries (Figure 1D-E). No feeding vessels from the Following excision of the superior pericardial reflection aortic arch or thoracic descending aorta (bronchial (Figure 3A), the left posterior atrial wall was exposed and arteries)weredemonstrated. the superior aspect of the tumor was excised. Then, the Alpha-adrenergic pharmacologic therapy (phenoxy- heart was suspended for removal of the inferior aspect of benzamine) was instituted before operation for the the tumor. There was no a plane of cleavage between the catecholamine-secreting tumor. She underwent surgical LA and the tumor, and atrial myocardium was also removal of the retrocardiac tumor via median sterno- removed (Figure 3B-C). The division of great vessels and tomy. As part of our initial surgical planning, cardio- LAwasnotnecessarytoaccessthetumoranditwascom- pulmonary bypass (CPB) was started through right pletely excised en bloc (Figure 3D). Coronary sinus, Figure2A.Peripheralperfusionthroughrightfemoralarteryandvein.B.Superiorveincavae(SVC)drainagethroughPacificocannula. C.ThelargecollateralvesselfromtheRCAwasdissectedandligated.RA:Rightatrium.Ao:Aorta.D.ThetwocollateralvesselsfromCxA (whitearrow)werealsoligatedforanoptimalcontrolofbleedingduringtheexcisionofthetumor.CS:Coronarysinus.LPVs:Leftpulmonaryveins. GonzálezLópezetal.JournalofCardiothoracicSurgery2013,8:22 Page4of8 http://www.cardiothoracicsurgery.org/content/8/1/22 Figure3A.Surgeon’sview.Superioraspectofthetumor(blackarrow)isshowed.Neovascularizationofthesmoothsurfaceisobserved. B.EndocardiumoftheLAwasexposed(blackarrow)duringsurgicalexcision(suspendedheart).C.Surgeon’sview.Dissectionofthesuperior aspectofthetumor(T).D.Paraganglioma(T)aftertotallyexcisionfromtheLA.IVC:Inferiorveincava.LIPV:Leftinferiorveincava. Figure4A.LAafterremovalofthetumor.Theoperativephotographshowsthedistalcollateralvessel(whitearrow)fromCxA(afterligation). EndocardiumoftheLAremainedinsitu.LAA:Leftatrialappendage.OS:Obliquesinus.B.Bovinepericardialpatch(blackarrow)wasusedforleft atrialreconstruction.Thesuturewasstartedfromtheleftatrialroof.C-D.PericardialpatchwasplacedontheposteriorwalloftheLAusinga polypropylenerunningsuture. GonzálezLópezetal.JournalofCardiothoracicSurgery2013,8:22 Page5of8 http://www.cardiothoracicsurgery.org/content/8/1/22 pulmonary veins and left atrial appendage were not Discussion involved. Adequate disease-free margins were achieved Cardiac paraganglionic tumors are rare neoplasms and the denuded LA was reconstructed using a bovine derived from the neural crest in the mediastinum, asso- pericardial patch (Figures 4A-D and 5A) and it was rein- ciated with the autonomic nervous system, which forcedwithsurgicalsealant. account for 0.3% of all mediastinal neoplasms and less CPB time was 143 minutes and aortic cross-clamping than 1% of all primary cardiac tumors [6]. They arise time was 105 minutes. Intraoperative TOE revealed a from either the branchiomeric paraganglia (coronary or completeexcisionofthetumorandnoperioperativehyper- aortopulmonary) or the visceral-autonomic paraganglia tensivecrisisonpalpationofthetumorweredemonstrated. (atrium or interatrial septum) and less than 50% are Thepatientwasthenweanedfrombypasssuccessfully. functional; the majority of functional tumors are intra- Histological examination confirmed the paraganglioma adrenal pheochromocytomas, being uncommon at the and malignant changes were not found (Figure 5B-C). heart. Chromogranin and synaptophysin werepositiveat immu- Edwin Besterman (St. Mary’s Hospital, London) was the nohistochemistry,andsustentacularcellswerepositivefor first author to report a successful resection on CPB of a S–100proteinstaining(Figure5D). cardiac pheochromocytoma in the LA in 1974 [7] and to The post-operative course was uneventful, no bleeding date,fewerthan50caseshavebeenreportedinthemedical was detected, sinus rhythm was maintained and antihy- literature [8,9] and they are typically located adjacent to or pertensivetherapywasnot necessary. Shewasdischarged involving the LA [10], although these tumors have been at 8th post-operative day and short-term anticoagulation described to be found in the interatrial septum [11], right was started for prevention of LA thrombus formation atrium,inferiorveincava[12],rootofthegreatarteries[13] due to transient endothelial dysfunction. At 3–month or proximal part of coronary arteries [14], possibly follow-up, she remains asymptomatic and biochemical explainedbycloseproximityofparaganglioniccellnest. evaluations have confirmed the normalization of levels of Clinical presentation depends on the functional status catecholamines. of the paraganglioma and the location at the heart: Figure5A.PosteriorLAafterpericardialreplacement.B.Macroscopicexamination(6×3×1.5cmfragment).Awell-demarcated homogeneous,smoothsurfacedandhighlyvascularizedtumor(2.8×2.5×1.5cm)wasfound,withclearsurgicalmargins.C.Histological examination.Haematoxylinandeosinstaining(H&E,40xmagnification)showingthe“zellballen”packetingofcells,withoutatypia,surrounded bycapillarynetwork(bluearrow).D.Immunohistochemicalanalysis(40xmagnification)revealedthespecificneuroendocrinemarkers (chromograninandsynaptophysin,C&S).S-100proteinstainingtechnique(100xmagnification)revealedthesustentacularcellnetwork (blackarrow). GonzálezLópezetal.JournalofCardiothoracicSurgery2013,8:22 Page6of8 http://www.cardiothoracicsurgery.org/content/8/1/22 paroxysmal hypertension due to secretion of catechola- the location in the mediastium, but some technical mines, chest pain [15], symptoms secondary to pericar- details mustbemade. dial involvement or invasion of the conduction system Because they are highly vascular, some groups advocate [16] or inespecified symptoms such as fever or malaise. the embolization before surgery to reduce perioperative In cases of extension to valves, other primary cardiac bleeding[25]whensurgicalexcisionofabulkytumorata tumors such as mixoma or angiosarcoma must be difficult location is planned. Although preoperative excluded. More unusual presentations have been embolization has been widely described in the treatment reported such as acute myocardial infarction and stroke of paragangliomas of the neck and carotid body, it has [17], although in some cases, the tumor can be an inci- been reported in a few cases of mediastinal paraganglio- dental finding. Acurious feature of the tumor in our pa- mas. For these situations, superselective angiography and tient was the absence of symptoms despite to be a subsequent embolization must be carried out 1 to 6 days functioningparaganglioma. preoperatively for a complete disappearance of the tumor In a short number of patients, cardiac paragangliomas blushandasafesurgicalexcision. can be associated to hereditary syndromes such as the Nevertheless, in our patient, for prevention of a ca- Carney triad (pulmonary chondromas, gastric stromal tastrophichaemorrhageduringthetimeoftheoperation,a tumors and extra-adrenal paragangliomas) [18] or fami- direct ligation of feeding vessels of the tumor was lial pheochromocytoma-paraganglioma syndromes. In performed. To date, isolated cases of direct ligation at the reported case, the familial pheochromocytoma- surgery have been described [26] and no comparative paraganglioma syndrome type 4 had been previously resultsbetweenthetwooptionsareavailable. detected. These syndromes involve a group of rare auto- As these tumors are of locally invasive nature and they somaldominantdisordersaffectingsympatheticandpara- have a less well-defined capsule than adrenal pheochro- sympatheticparaganglia, causedby germlinemutationsof mocytomas, a complete resection can be technically dif- the genes SDHB, SDHC and SDHD, although some of ficult by the position of the tumor and should be these mutations can also be detected for patients with performed on CPB and cardioplegic arrest in the most apparentlysporadicpheochromocytomas[19]. part of cases in order to achieve disease-free excision Most cardiac paragangliomas are benign, but local margins. When the interatrial septum is involved, the invasion can occur and metastases have been reported anterior aspect of the septum must be preserved to avoid in isolated cases [20,21]. Location in the heart is not heart block; in other situations, the tumor invades particularly associated with familial syndromes, although surrounding vascular structures and resection may be malignant predisposition is increased with the SDHB extremelydifficult.Forselectedpatients,extensivecardiac mutation [22]. Because of this reason, long-term follow- reconstructive surgery may therefore be required, with its up is mandatory in our patient due to the implications associated risks. Our patient underwent a successful of this hereditary disorder. However, malignant primary complete resection of the “caged” tumor between the paragangliomas of the heart are exceptional and to date, pulmonaryveinsalongwithreconstructionoftheresected there arenoreportedcasesassociatedtothis mutation. siteandnocomplicationsweredetectedaftersurgery. The preoperative goals for this type of tumors are to Reconstruction of the LA using either autologous or normalize hemodynamic variables such as blood pres- bovine pericardial patch has been previously described sure and avoid surges in catecholamine release with and, when a portion of the pulmonary veins is also infil- tumor manipulation during surgery. Nevertheless, pre- trated and excised, it must be repaired and reconstructed operativealpha-1-adrenoceptor antagonist seemstohave with the patch [27], although these cases are at risk for no benefit in maintaining intraoperative hemodynamic development of pulmonary stenosis at follow-up. Recon- stability in patients with normotensive adrenal pheo- structionofrightventricularoutflowtractandpulmonary chromocytoma [23]. Although our patient showed valve has also been reported when local invasion is normal preoperative levels of blood pressure despite to detected [28] following the removal of the tumor en bloc be a catecholamine-secreting tumor and it is important withadjacentstructuressuchasthymus,pericardium,part to bear in mind these recent considerations, no evidence of pulmonary trunk and anterior leaflet of pulmonary about normotensive cardiac paragangliomas has been valve. reported and we consider the alpha-blockade prepa- Although surgical resection and reconstruction is the ration for thisisolatedcase. gold standard treatment for these tumors, this approach Less than 40 cases about surgical excision of a cardiac can be impossible due to extensive involvement of the paraganglioma have been described in adults [24] and great vessels or other structures of the heart. When total the surgical treatment is similar to that of all other surgicalresection is extremely difficult, cardiac autotrans- cardiac tumors; the approach can be either a median plantationhasbeendescribedand,inselectedcases,when sternotomy or posterolateral thoracotomy depending on a complete removal is not possible, orthotopic cardiac GonzálezLópezetal.JournalofCardiothoracicSurgery2013,8:22 Page7of8 http://www.cardiothoracicsurgery.org/content/8/1/22 transplantation is a feasible technique in those paragan- Consent gliomas that have direct coronary artery involvement, Written informed consent was obtained from the patient extendintotheleftventricleorinvadetheatrioventricular for publication of this Case report and any accompanying groove[29].Ingeneralterms,althoughoperativemortality images. A copy of the written consent is available for and overall survival seems favorable in series of patients reviewbytheEditor-in-Chiefofthisjournal. with primary cardiac tumors and benefits of this tech- nique include improved accessibility and ability to per- Abbreviations SDHB:SubunitBofsuccinatedehydrogenaseenzyme;SDHD:SubunitDof formacompletetumorresection,therearefewreportsof succinatedehydrogenaseenzyme;SDHA:SubunitAofsuccinate transplantation for excision of cardiac paragangliomas in dehydrogenaseenzyme;SDHC:SubunitCofsuccinatedehydrogenase theliteratureandlateresultsareunknown[29]. enzyme;MEN:Multipleendocrineneoplasiasyndrome;CT:Computed tomography;MIBG–scan:Iodine-123-markedmetaiodobenzylguanidine– In the only reported case series including 14 patients scan;PET–scan:Positronemissiontomography–scan;(18F-DOPA)PET over the last 30 years, at Mayo Clinic [30], Brown et al. scan:18F-dihydroxy-phenyl-alaninepositronemissiontomographyscan; have reported a safe resection of these tumors often TOE:Transesophagealechocardiography;LA:Leftatrium; CPB:Cardiopulmonarybypass;RCA:Rightcoronaryartery;CxA:Circumflex under CPB with modest surgical risk, with one intra- artery;SVC:Superiorveincavae;RA:Rightatrium;Ao:Aorta;CS:Coronary operative death due to massive bleeding (in the only sinus;LPVs:Leftpulmonaryveins;IVC:Inferiorveincava;LIPV:Leftinferior tumor of this serie involving the LA) and a total number veincava;LAA:Leftatrialappendage;OS:Obliquesinus;H&E:Haematoxylin andeosin;C&S:Chromograninandsynaptophysin. of 10 patients were alive at follow-up (median follow-up: 2.3 years). The SDHB mutation was detected in two Competinginterests patientsoftheserie. Theauthorsdeclarethattheyhavenocompetinginterests. When a complete removal of the tumor is achieved, long-term prognosis seems to be excellentand survival of Authors’contributions upto14yearshasbeenreportedinapatientwithexcised MTGL:Design,datacollection,photographsandwritingarticle.SGG;ESG; SGR;andJGL:Criticalrevisionandhelpedtodraftthemanuscript.Allauthors mediastinal paraganglioma [31]. To date, surveillance can readandapprovedthefinalmanuscript. be done by following serum catecholamine levels, repea- tingCTscansand/orTOE,andmostrecentlyMIBGscan Acknowledgements and PET imaging. Recent data support the superiority of WeareindebtedtoDrJoaquínCamposFernández,fromAnatomical 18F-DOPA PET-CT over 123–I–MIBG scintigraphy to PathologyDepartment,atCarlosHayaRegionalHospital(Málaga)for performingthehistopathologicalexaminationandphotomicrographs assess disease extension in patients with recurrent para- ofthetumor. ganglioma [32] and it might be the preferred test in this patient follow-up because of the implications of the Authordetails 1CardiovascularSurgeryDepartment,CarlosHayaRegionalHospital,Carlos hereditary syndrome. However, there is no consensus on HayaAvenue,s/n.29010,Málaga,Spain.2EndocrinologyDepartment,Carlos thetreatmentifthetumordoesrecuratfollow-up. HayaRegionalHospital,Málaga,Spain. Our case is relevant in that there were no classic signs Received:1August2012Accepted:11January2013 of catecholamine excess despite to be a functioning Published:29January2013 tumor and it illustrates successful management of a benign left atrial primary paraganglioma associated with References SDHB mutation, including direct ligation of feeding 1. WaltherMM,KeiserHR,LinehanWM:Pheochromocytoma:evaluation, diagnosis,andtreatment.WorldJUrol1999,17:35–39. vessels of the tumor, complete excision and subsequent 2. MangerWM:Insearchofpheochromocytomas.JClinEndocrinolMetab reinforcement of the LA, without evidence of recurrence 2003,88:4080–4082. at short-term follow-up. 3. PachecoN,MarcosG,GarcipérezFJ,PérezC:Intrapericardial paraganglioma.RevEspCardiol2010,63:116–117. 4. ElderEE,ElderG,LarssonC:Pheochromocytomaandfunctional Conclusions paragangliomasyndrome:nolongerthe10%tumor.JSurgOncol2005, 89:193–201. – Primarycardiacparagangliomasare exceptional 5. 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RufiniV,TregliaG,CastaldiP,PerottiG,CalcagniML,CorselloSM: Comparisonof123-I-MIBGSPECT-CTand18F-DOPAPET-CTinthe and take full advantage of: evaluationofpatientswithknownorsuspectedrecurrent paraganglioma.NuclMedCommun2011,32:575–582. • Convenient online submission • Thorough peer review doi:10.1186/1749-8090-8-22 Citethisarticleas:GonzálezLópezetal.:Surgicalexcisionwithleftatrial • No space constraints or color figure charges reconstructionofaprimaryfunctioningretrocardiacparaganglioma. • Immediate publication on acceptance JournalofCardiothoracicSurgery20138:22. • Inclusion in PubMed, CAS, Scopus and Google Scholar • Research which is freely available for redistribution Submit your manuscript at www.biomedcentral.com/submit