Table Of ContentGonzálezLópezetal.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
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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).
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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.
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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.
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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).
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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
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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
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32. RufiniV,TregliaG,CastaldiP,PerottiG,CalcagniML,CorselloSM:
Comparisonof123-I-MIBGSPECT-CTand18F-DOPAPET-CTinthe and take full advantage of:
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