Recurrence of cardiac myxoma is a rare condition, observed in about 3% of patients in sporadic cases, although it is more frequent in familial ones. Several mechanisms have been proposed to explain such recurrence, and the importance of increased vascularization as a facilitating feature is the subject of debate. The authors report the case of a non-familial right atrial myxoma, unusual for both its histopathology and recurrence

Abecasis, J

Andrade, MJ

Branco, LM

Carvalho, MS

Gouveia, R

Mendes, M

Neves, JP

Repository of the Hospital Center of Central Lisbon

Compreender a Recorrência dos Mixomas Cardíacos. Um Caso Clínico

Repositório do Centro Hospitalar de Lisboa Central, EPE

Document downRev Port Cardiol. 2013;32(3):239--242Revista Portuguesa deCardiologiaPortuguese Journal of Cardiologywww.revportcardiol.orgCASE REPORTUnderstanding  cardiac  myxoma  recurrence:  A  case  reportMaria Salomé Carvalhoa,∗, Maria João Andradea, João Abecasisa, Rosa Gouveiab,Luísa  Brancoc, José Pedro Nevesd, Miguel Mendesaa Servic¸o  de  Cardiologia,  Hospital  de  Santa  Cruz,  Carnaxide,  Oeiras,  Portugalb Servic¸o  de  Anatomia  Patológica,  Hospital  de  Santa  Cruz,  Carnaxide,  Oeiras,  Portugalc Servic¸o  de  Cardiologia,  Hospital  de  Santa  Marta,  Lisboa,  Portugald Servic¸o  de  Cirurgia  Cardiotorácica,  Hospital  de  Santa  Cruz,  Carnaxide,  Oeiras,  PortugalReceived  18  May  2012;  accepted  27  May  2012Available  online  28  February  2013KEYWORDSRight  atrial  myxoma;Recurrent  cardiacmyxomaAbstract  Recurrence  of  cardiac  myxoma  is  a  rare  condition,  observed  in  about  3%  of  patientsin sporadic  cases,  although  it  is  more  frequent  in  familial  ones.Several mechanisms  have  been  proposed  to  explain  such  recurrence,  and  the  importance  ofincreased  vascularization  as  a  facilitating  feature  is  the  subject  of  debate.The authors  report  the  case  of  a  non-familial  right  atrial  myxoma,  unusual  for  both  itshistopathology  and  recurrence.©  2012  Sociedade  Portuguesa  de  Cardiologia  Published  by  Elsevier  España,  S.L.  All  rightsreserved.PALAVRAS-CHAVEMixoma  do  corac¸ãodireito;Mixoma  recorrenteCompreender  a  recorrência  dos  mixomas  cardíacos  --  Um  caso  clínicoResumo  A  recorrência  de  mixomas  cardíacos  é  rara,  acontecendo  em  cerca  de  3%  dos  doentescom mixomas  esporádicos,  embora  seja  mais  frequente  nos  casos  familiares.Vários mecanismos  foram  propostos  para  explicar  as  recorrências  e  a  importância  daloaded from http://http://www.elsevier.pt, day 03/05/2013. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.vascularizac¸ão aumentada  como  fenómeno  facilitador  é  controversa.Os autores  descrevem  o  caso  de  um  mixoma  esporádico  do  corac¸ão  direito,  peculiar  pelassuas características  histológicas  e  recorrência.© 2012  Sociedade  Portuguesa  de  Cardiologia.  Publicado  por  Elsevier  España,  S.L.  Todos  osdireitos reservados.∗ Corresponding author.E-mail address: mariasalomecarvalho@gmail.com(M.S. Carvalho).IMrﬁl0870-2551/$  –  see  front  matter  ©  2012  Sociedade  Portuguesa  de  Cardiologhttp://dx.doi.org/10.1016/j.repc.2012.05.027ntroductionyxomas  are  the  most  common  primary  cardiac  tumors,epresenting  30--50%  of  cardiac  neoplastic  lesions  identi-ed  in  anatomopathological  series.  Although  preferentiallyocated  in  the  left  atrium,  myxoma  may  also  appear  in  theia  Published  by  Elsevier  España,  S.L.  All  rights  reserved.240  M.S.  Carvalho  et  al.Figure  1  Transthoracic  echocardiography  study  showing  a  right  atrial  mass  located  near  the  inferior  vena  cava  opening  (A  andB costa  atrirreccasCWintCrtmw(irssewrFsunoIctaaﬁtw(rocanahffruDDocument downloaded from http://http://www.elsevier.pt, day 03/05/2013. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited. --  non-conventional  views,  C  --  apical  4-chamber  view,  D  --  subtrium (B).  *:  right  atrial  mass;  IVC:  inferior  vena  cava;  LA:  leftight  atrium  (up  to  20%  of  cases),  ventricular  chambers  and,arely,  on  cardiac  valves.1,2Its  clinical  behavior  may  be  ‘‘malignant’’  withmbolic  events,  valvular  dysfunction  and  hemodynamicompromise.3 Nevertheless  these  benign  tumors  are  usuallyured  by  surgery  and  synchronous  and/or  recurrent  formsre  rarely  described,  mainly  in  the  context  of  familialyndromes.ase reporte  report  the  case  of  a  38-year-old  man  referred  to  ournstitution  for  recurrence  of  a  heart  tumor.  His  history  wasegative  for  other  cardiovascular  problems  or  family  his-ory  of  heart  disease  and  tumors.  There  was  no  evidence  ofarney  complex.Three  years  previously  he  had  undergone  surgicalemoval  of  a  large  right  atrial  myxoma,  detected  on  aransthoracic  echocardiogram  ordered  because  of  fatigue,alaise,  shortness  of  breath  and  chest  tightness.  The  massas  described  as  fully  occupying  the  right  atrial  cavity68  ×  53  mm  in  apical  view),  extending  towards  the  open-ng  of  the  inferior  vena  cava  and  prolapsing  into  theight  ventricle,  causing  functional  tricuspid  valve  steno-is.  Transesophageal  study  located  the  stalk  in  the  atrialeptum.Surgical  excision  was  reported  as  complete,  requiringxtensive  excision  and  reconstruction  of  the  atrial  septumith  autologous  pericardium.Histopathology  conﬁrmed  the  diagnosis  of  a  completelyesected  right  atrial  myxoma  with  free  tumor  margins.ollow-up  transthoracic  echocardiography  after  12  monthshowed  no  right  atrial  mass.T2aal  view)  and  ﬂow  acceleration  at  the  conﬂuence  with  the  rightum;  LV:  left  ventricle;  RA:  right  atrium;  RV:  right  ventricle.Two  years  later,  a  redo  routine  echocardiographic  eval-ation  revealed  the  presence  of  a new  small  sphericalon-mobile  right  atrial  mass  (26  ×  26  mm).  Its  echocardi-graphic  appearance  was  soft,  smooth,  and  homogeneous.t  was  located  in  a  small  recess  near  the  inferior  venaava  opening.  Impaired  venous  return  was  suspected  dueo  the  absence  of  inferior  vena  cava  respiratory  variation,nd  ﬂow  acceleration  at  the  conﬂuence  with  the  righttrium  (Figure  1).  Transesophageal  echocardiography  con-rmed  these  ﬁndings  and  ruled  out  the  presence  of  otherumor  foci.  Both  the  mass  and  the  residual  atrial  septumere  shown  by  Doppler  imaging  to  be  richly  vascularizedFigure  2).The  patient  underwent  excision  of  the  mass,  whichequired  venous  and  atrial  septum  reconstruction  as  a  resultf  the  broad  base  of  tumor  implantation.Histopathological  diagnosis  was  consistent  with  benignardiac  myxoma  (Figure  3A).  A  peculiar  morphologicalspect  attracted  the  pathologist’s  attention:  an  increasedumber  of  vessels  randomly  located  around  the  insertionrea,  with  irregular  contours,  thick  walls,  and  ﬁbrointimalyperplasia  (Figure  3B).  At  this  point,  a  review  of  the  slidesrom  the  ﬁrst  cardiac  myxoma  showed  similar  morphologicaleatures.Twelve  months  after  the  second  operation,  the  patientemains  asymptomatic,  without  abnormal  ﬁndings  on  follow-p  transthoracic  echocardiography.iscussionhe  estimated  recurrence  rate  of  sporadic  myxoma  is--3%.4--6 They  usually  appear  during  the  ﬁrst  four  years,lthough  they  can  emerge  within  a  few  months  to  severalUnderstanding  cardiac  myxoma  recurrence  241Dopp;  RAtosmscpdrabasbtEPdaConﬁdentiality  of  data.  The  authors  declare  that  they  haveDocument downloaded from http://http://www.elsevier.pt, day 03/05/2013. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.Figure  2  Transesophageal  echocardiography  study  with  color  residual atrial  septum  (B).  *:  right  atrial  mass;  AS:  atrial  septumyears  after  surgical  excision.7--9 Unlike  primary  myxoma,which  is  more  frequent  in  women,  tumor  recurrence  appearsto  be  more  frequent  in  men.10The  ﬁrst  case  of  myxoma  recurrence  was  described  byGerbode  et  al.  in  1967,  several  years  after  the  ﬁrst  surgicalremoval  of  such  tumors.11 At  that  time,  incomplete  surgicalresection  was  presumed  to  be  the  cause.  Since  then,  sev-eral  cases  of  tumor  recurrence  have  been  reported,  not  onlyafter  extensive  excisions  but  also  arising  on  the  opposite  sideof  the  heart  from  the  initial  one.12Four  different  possible  mechanisms  have  been  putforward  to  explain  recurrence:  inadequate  resection,totipotent  multicentricity,  inheritance  (familial  type),  andmetastatic  recurrence.1,5,12,13There  is  some  histopathological  evidence  of  a  tendencyfor  increasing  aggressiveness  and  possible  malignant  degen-eration  into  forms  of  myxosarcoma.1,10Even  though  the  mechanism  of  recurrence  is  difﬁcult  toconﬁrm  in  an  individual  case,  the  possibility  of  a famil-ial  syndrome  should  always  be  considered.  The  presenceof  concurrent  cardiac  lesions  and  clinical  features  sugges-tive  of  syndromic  association  (such  as  hyperpigmentation,endocrine  disease,  or  benign  extracardiac  connective  tissuetumors)  should  raise  the  suspicion  of  familial  transmission,14for  which  causative  mutations  have  been  identiﬁed.15The  unusual  nature  of  this  case  lies  in  a  less  commonlocation  of  the  tumor  (in  the  right  atrium),  and  in  its  recur-rence,  considering  this  is  a  case  of  the  sporadic  form  offtsiFigure  3  (A)  Conventional  view  of  a  cardiac  myxoma  (hematoxylinvan Gieson  stain  ×40).ler  showing  the  rich  vascularization  of  the  mass  (A)  and  of  the:  right  atrium.he disease.  Moreover,  both  echocardiography  and  histol-gy  provided  evidence  of  marked  vascularization,  a featureeldom  encountered  in  myxomas,  but  usually  present  inalignant  lesions.  In  fact,  the  presence  of  these  large  ves-els  in  the  tumor  base,  pedicle,  and  surrounding  structuresould  facilitate  development  and/or  recurrence  of  a  neo-lastic  mass,  acting  as  stimuli  for  excessive  growth  andifferentiation.  A  strong  angiogenic  potential  driven  by  theelease  of  VEGF  by  macrophages  and  the  tumor  cells,  as  wells  autocrine  activation  of  VEGF  receptors  on  these  cells,  haseen  shown  in  cardiac  myxoma,  thereby  perpetuating  thengiogenic  response  within  these  tumors.16 Abnormal  bloodupply  might  thus  be  an  explanation  for  the  oncogenic-likeehavior  of  otherwise  benign  quiescent  multicentric  totipo-ent  cells.thical disclosuresrotection  of  human  and  animal  subjects.  The  authorseclare  that  no  experiments  were  performed  on  humans  ornimals  for  this  study.ollowed  the  protocols  of  their  work  center  on  the  publica-ion  of  patient  data  and  that  all  the  patients  included  in  thetudy  received  sufﬁcient  information  and  gave  their  writtennformed  consent  to  participate  in  the  study. and  eosin  stain  ×100).  (B)  Anomalous  vascularization  (elastic2RosiCTR1111111Document downloaded from http://http://www.elsevier.pt, day 03/05/2013. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.42  ight  to  privacy  and  informed  consent.  The  authors  havebtained  the  written  informed  consent  of  the  patients  orubjects  mentioned  in  the  article.  The  corresponding  authors  in  possession  of  this  document.onﬂicts of interesthe  authors  have  no  conﬂicts  of  interest  to  declare.eferences1. Reynen K. Cardiac myxomas. N Engl J Med. 1995;333:1610--7.2. Oliveira R, Branco L, Galrinho A, et al. Cardiac myxoma:a 13-year experience in echocardiographic diagnosis. Rev PortCardiol. 2010;29:1087--100.3. Manfroi W, Vieira SR, Saadi EK, et al. Multiple recurrences ofcardiac myxomas with acute tumoral pulmonary embolism. ArqBras Cardiol. 2001;77:161--6.4. Murphy MC, Sweeny MS, Putnam JB, et al. Surgical treatmentof cardiac tumors: a 25-year experience. Ann Thorac Surg.1990;49:612--8.5. Shinfeld A, Katsumata T, Westaby S. Recurrent cardiac myx-oma: seeding or multifocal disease? Ann Thorac Surg. 1998;66:285--8.6. Amano J, Kono T, Wada Y, et al. Cardiac myxoma: its origin andtumor characteristics. Ann Thorac Cardiovasc Surg. 2003;9.M.S.  Carvalho  et  al.7. Samanidis G, Perreas K, Kalogris P, et al. Surgical treatment ofprimary intracardiac myxoma: 19 years of experience. Interac-tive Cardiovasc Thorac Surg. 2011;13:597--600.8. Goswami KC, Shrivastava S, Bahl VK, et al. Cardiac myx-omas: clinical and echocardiographic proﬁle. Int J Cardiol.1998;63:251--9.9. Yavuz S, Eris C, Sezen M, et al. Recurrent multiple cardiac myx-omas. Bratisl Lek Listy. 2010;111:549--51.0. Gray I, Williams W.  Recurring cardiac myxoma. Br Heart J.1985;53:645--9.1. Gerbode F, Kerth WJ, Hill JD. Surgical management of tumorsof the heart. Surgery. 1967;61:94--101.2. Aroca A, Mesa JM, Dominguez F, et al. Multiple recurrence of a‘‘sporadic’’ (non-familial) cardiac myxoma. Eur J CardiothoracSurg. 1996;10:919--21.3. Reber D, Birnbaum DE. Recurrent cardiac myxoma: why itoccurs. A case report with literature review. J Cardiovasc Surg(Torino). 2001;42:345--8.4. Kirschner LS, Yin Z. The CNC gene PRKAR1A plays an essen-tial role in cardiac development and myxomagenesis. TrendsCardiovasc Med. 2009;19:44--9.5. Vezzosi D, Vignaux O, Dupin N, et al. Carney complex:clinical and genetic 2010 update. Ann Endocrinol (Paris).2010;71:486--93.6. Gaumann A, Strubel G, Bode-Lesniewska B, et al. The roleof tumor vascularisation in benign and malignant cardio-vascular neoplasms: a comparison of cardiac myxoma andsarcomas of the pulmonary artery. Oncol Rep. 2008;20:309--18.

http://repositorio.chlc.min-saude.pt/bitstream/10400.17/1240/1/RPC%202013%20239.pdf

Compreender a Recorrência dos Mixomas Cardíacos. Um Caso Clínico

Abstract

Similar works

Full text

Available Versions

Repository of the Hospital Center of Central Lisbon

Repositório do Centro Hospitalar de Lisboa Central, EPE