183

Primary Cardiac Tumors: Experience at the University of Minnesota 1. E. M olina, J. E. Edwards, and H. B. Ward

Su mmary A review was made of a ll primary card iac tum ors see n at the University of Minn esota or entered in the Regist ry or Ca rd tova scular Disease, a ffiliated with the Un iversity of Minnesota. between 1959 and 1989 . Of a total of 124 primary cardiac tum ors. 10 3 (83 %) wer e benign and 21 (17 %) were malignant. Atr ia l myxomas (51 cases) accounted for 42 % of all tumors and 50 % of beni gn neoplasias. The remain ing 51 benign tumors were, in or der of frequency : rhabdom yomas, 14 (13 %), papillomas, 12 (1 1%), fibr omas, 9 (9%), hamartomas, (4 %), ter atomas (2%), lipo mas (2%), mes otheliom a s (2%), flbr oelastoma s, hemangiomas, gloma ngiomas, and a few others. The 21 maligna nt tum ors, a ll sarcomas , were, in order offrequency: rhabdomyosarcomas, 6 (29 %). angiosarcomas. 4 (19 %), myxosarcomas , 3 (14 %). fibrosarcomas, 2 (10%), and one eac h leiomyosarcoma , reticulum cell sarcoma, and liposarcoma. There were 3 ca ses of spind le cell sa rcoma (14 %) ori ginating in th e gr ea t vessels: 1 in the pulmonary artery a nd 2 in the aorta. Th e pr ogno sis of the resected benig n tu mo rs was good , with no com plications of recurrenc es for up to 15 years of follow-up . Tumors that could not be excised included mostly rhabdomyomas, in newborns , an d cavernous hemangiomas involving mo st of the heart . with poor prognosis. Malignant tumors of the heart had a very poor prognosis. even wh en operated on promptly after d iagnosis . The mean survival time of these patien ts wa s 5 months a fte r rese ction , even wh en follow ed with chemot hera py a nd/o r rad iation ; 26 %ofthe tumo rs had already met astasized at the time of operation, Extensive resections in malignant tumors did not render sa tisfacto ry results.

Pr tma re Hcrzt umoren . Erfahrungen an der Unfversn at von Minn e so ta Dlese Arbeit fall t aile prtmarcn Heratumoren zu samme n. die zwischen 1959 und 1989 a n der Un ivers itat von Minn esot a behandelt wurden od er Eingang in das de r Universitat a ngegttederte Register flir kardlovaskulare Erk rank ungen fanden . In diesem Zeitraum wurden insgesamt 124 primare l lerztumoren er fab t, von denen sich 103 (83%) als gut artig und 21 (17 %) als mal igne erwiesen. In 51 Fallen ha ndeltees stch urn Myxom e . die 42 %aller Herztumo re n bzw . 50 %dergutartigen Neub ildu nge n stellten . 4 5 % Myxom e fand en sich im linke n vcrhof 4 wa ren im rechten Vorhof lokalisiert und zwet im rechtcn v entrikel. Die Alters vert eilung ergab eine Bevorzugu ng fli t da s 4 . und 5. Lcbensjahrzehnt. d agegen war ke in signilika nter Untersc hied zwisch en den Geschlechtern fest zustellen. 36 Patie nten mit na chg ewiesen em Myxom konnten operativ behandelt werden . Nur bei einem Pat ient en wurde 4 Jahre nach de m EingrifTein Hezidivtumor beobachtet. Als haufigste Neoplas ie des Herzens bei Sa uglingen und Kindem gilt da s Rhab domyom, da s in 14 Fallen (13 % aller gutart igen Herztumoren ) nachgewiesen werde n konnte. 10 Kind er waren jtinger als zwei Woch en , da s

Thora e. ca rdiovasc. Surgeon 38 (1990) (Special lssuel 183- 191 © Georg Thieme Verlag Stutt gart · New York

mittler e Alter betrug 6 Tage . Die GroBe des tiberall im lIerzen vorkommenden Tumors ka nn von wentgen Millimeter n bis zu ein e r ext re men Ausdeh nu ng mit multlzer urtscher l.oka lisation varlieren: die Ergeb niss e dcr operauven Behandlu ng gerade von expan siv wa chsenden ode r ruultilok ula r vor kommend en Rhabd omyomen si nd da he r enuausc hend. Bel dem Pa pillom des He rzcns handelt es stch urn etn en kleinen Tu mo r, der in typi scher weise im Klappengewebe pr oliferiert und 'lo r allem im hoheren l.ebensalter vorkommt. ln dleser Zusammenstellun g \vurden Pa pillome bet 12 Pa ucnten (11 % dcr bcn tgnen Tum oren l nachgewlesen. deren Alter sich von 57 bis 84 Ja hr en er streckte . BetrolTen wa r 'lor a llem die Mitralklappe. Neu n unsercr Pattcntcn wtcsen etn Fibro m (9 % der be nig nen TUllIOre n) auf. da s wie das Rha bdomyom im Myokard lokalisiert ist und mu ltizentr isch auftr ltt. Auch d iese r Tumor findet sich haufig bet kleinen Kindem , Die von den Fibr omen verursacbten Stc rungc n han gen von threr Grolle so wie von ihrer Beelm rs chugung d er Herzfunktion ab o w ese ntltch selte ne r Ia nde n sich unter den guta rttgen Ilerztumoren Tera tome (2%), Ila ma rtome (4 %). Ltpomc (2%1. Mesoth eltomc (2 %) sowlc Ptbroelastome. Hamangtcrn e und Gloma ngiom e. Bel allen 21 pri ma ren maltgnen Herz tumoren h a ndelte es sich urn Sa rka me. 19 der 2 1 Neubildu ngen hau en th ren Ursprung in de n Vorhofe n. vorwtegend im rec hten. De s Pattente na lter erstreckte sich von 4 ~6 8 Ja hren. Ma nner u nd Frauen w aren gleichermaBen betroffcn. Be l de r Dtagnoscstellung halte n bcretts 26 % dcr Tumoren die l.unge metastastert. Bel 9 Patienten wu rd e dcr vcrsuch el ncr Tumorrcsektton unterno mmen . Die d urchschni ttliche Oberle bensrate von 8 Patie nten nach den chiru rgtsche n EingrilTen betrug 6 Monate. Mit 6 Fallen wa r das Hhab domyosarkom der am hauflgston vorgefund ene ma ligne Herztumor . Bei zwei Patien ten ging dcr Tum or von den Ventrikeln aus. Eine operative Behandlung wa r bet kein em de r Pattenten mcgltch . Aus schlieJ31ich im rech ten Vorh of lokalistertc Angiosarkome dta gnosuztcnen wlr be l vier Pattenten . von den en n ur einer ele ktiv operie rt w urde . Trotz radikaler Hesek tton rezidivierte der Tumor nach dr ei Monaten . der Pati ent vcrstarb etn h albe s Jahr nac h de m Eing rifT. Von drei Pattente n mit Myxos a rkom en kormtc in zw ei Fa llen etne Hosekuo n dur chg efUhrt werd en. Tumorreztdlve trate n nach einem Ja h r bzw . nach 5 Ja b ren auf, Der erste Pa tie nt. bei de m ein erne uter Eingr ilT mit Tetlresektton vorgenommen werd en konn te. st a rb 3,5 Ja h re nach der Zwe itoperation. Bet zwei Patteruen mit Fib rosa rkomen konnte erst postmort al die cxa kte Diagn ose gest ellt wo rde n. weuerbtn beob achteten wir Elnzelmile von Leiomyosa rko me n , Retikulozellsarkomen , U posa rkome n un d malign en Jlist iozytomen. Versllche. be i zwei d iese r Patienten den Tum or zu resezieren , blieben er folglos . Wahrend Patienten na ch Entfernung von be nign !'n lI en:tumo ren ei ne a usge ze ich net e Progn ose ohne wesentliche Komplikations- oder Hezidivrate be i einer Verlau fsbeobachtung von 15 Jahren au fweise n, wa rte n nichl-r eseziert e Tumor en wie Hha bd om yome bei Neugebo re nen oder ka ve rn 6scn l lam an gio-

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University of Minnesota, Departments of Surgery an d Pathology, the Minnesota Hea rt and Lung Institute, Minneapolis. Minnesota. and the Registry for Cardiovascular Diseases. St. Paul, Minnesota

J. E. Molina. 1. E. Eduords. II. lJ. l1ard

Thome . rardiol'Use. Surgn3Sf!f)f)())

me mil einer ungu nsnge n Prognose auf. Die Ausslcbt en fur Patientcn mit mahgn en Herzrumoren sind trotz umge hende r chirurgischer Interventioncn nec h Diagnosestellung exrrem scblecht. Xach Resektion betr agt die mittlere Dbe rlebenszen dieser Paue nten 5 \ Ionate, selbst we nn eine adjuvame Chemothe rapte und/oder Badtothera ple erfclgt . 26 % diese r Tumoren hanen bereits zum Zr itpun kt de r Operation metasrasten . Aus gtebige Bese knon sverfah ren bet mallgne n Herztumoren erbrachtcn ketne befrtedlgendc n Hesultate.

Primary cardiac tumors ar e generally ra re, accounting for 0.002% in aut opsy serie s and slightly higher in clinical se ries (4, 5, 7, 9, 16). The purp ose of this review was to reevalua te long-term survival of patients with benign and malignant primar y cardiac tumors, curre nt methods of diagnosis, thera peutic approaches. a nd excision techniques. ~I a t e ri al

a nd ~l ethod s

We reviewed the last 30 years [May 1959-M a rch 1989) crpnmary card iac tumors diagnosed or treated at the Umvers uy of Minnesota or entered in the Registry for Ca rdiovascular Disea ses. There were a total of 124 primary tumors of the hea rt (103 benign and 21 malig na ntl. Table 1 shows the incidence of benign and malignant tumo rs. as well as the ir types .

Benign Tumors (Ta ble 2) Table 1 Pnmary CardiacTumors In order of frequency Number Myxoma Rhabdomyoma Papilloma Fibroma Rhabdomyosarcoma Angiosarcoma Hamartoma Myxosarcoma Others Total

Table 2

%

Cardiac location was as follows: 45 in the left at rium, 4 in the right atrium, and 2 in the right ventricle. Typically, th e left at rial tumo r had the base of implant towa rd the septum above the mitral valve with a base ofle ss than 1cm 2 . lt was always large compared to the left atrial chamber. varying from 3.5 to 9 cm in diameter (mean s.s.cmr. The tumor had a broad spectru m of distribution am ong all ages. ranging from 13 to 85 yea rs (mean age 47.5 years). The peak incidence by age occurred in the 4th and 5th decades of life (see Ta ble 3). The tumor was equally distributed amo ng sexes: 27 females and 24 males.

Table3

Distribution of Myxoma 151 Cases) by Age

Years of Age

Number

80

8 6 22 12 3

Total

51

42 11 10

9 6

7 5

Dia gnosi s

4 4

3 3

Between 1959 and 1979, diagnosis was by clinical history. cardiac catheterization. and angiogra phy. But since 1980. the echocardiography has become the method of choice (Fig. 1). The diagnosis of left atrial myxoma before 1980 was prolonged an d difficult. Patients usually had sympt oms ofleft-sided A-V valve obstruction and heart failure. Some were hospitalized severa l times before an an giogram was done and the diagnosis mad e. Other symptoms and signs as sociated with a leftatrial tumor were at rial flutter. fibrillation, dyspn ea . cough. and chest pain. One patient suffering from atrial ar rhythmia s had a left atrial tumor removed. but has remai ned arrhythmic throughout 15 yea rs of follow-up. although the tumor did not recur. Another patient, a 62-year- old man with recurr ent pulmona ry embolism and ca rdiac failure. had developed left iliofemoral venous thrombosis. He was trea ted with an inferior vena cava filter but continued to deteriorate an d then died. Autopsy revealed left iliac vein throm bosis, but also a large 5.5 x 5 x 4 em left atrial myxoma . Several cases of unsuspected myxoma were found. One l Svyear-old child experienced multiple infar ctions in the kidneys and spleen. along with total mitral valve occlusion ca used by a myxoma that was never diagnosed. Another patient. who died from congestive heart failure . was found at autopsy to have a la rge right atrial myxoma with tricuspid

3

2

21 124

17

Number

Total

Cardiac tumour -Atrial mvxema - Hha bdomvoma -. xtyxosarcorna- Surgical the rapy .

51 14 12

100

Dlstnbutlon of histologIcally BenignPrimary Cardiac Tumors

Myxoma Rhabdomyoma Papilloma Fibroma Hamartoma Teratoma Glomangioma Others

Ke)" wo rds

51



50

I'

13

12

,

11

9

, 9

2 2

2 2

9

9

103

100

Myxoma As in several reports in the literature (2. 4. 5. 7. 9. 16), myxoma was the most frequently found primary ca rdiac tumor: 51 in all. 50% of benign and 42% of all tumors.

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184

Thorae. eardiOtmse. S urgll38 (1990J

Primary Cardiac Tumors: Experience ot the University of Minnes ota

valve obst ruction and secondary lesions at the tricuspid valve (Fig. 9). These 2 patient s died in 1959 and 1961, respectively. Anothe r 2 patients. who died in 1967 . were found at au topsy to have a myxoma of the left at rium; 1 had an embo lism to the right coronary arte ries and several acute myocardial infa rctions. and the other had a lar ge embolus to the bra in plus multiple myocardial infarctions from myxomatose embo lism . One other patient died in 1970 after a massive coronary emboli sm and cereb ral infarction due to tumor tissue ; autopsy showed a left at rial myxoma . The diagnosis of myxoma was made in ano the r patient with a peri pheral femora l embolism; under the microscope emb olus revea led myxomatous tissue . The patien t was subseq uently operated on and a myxoma remov ed from the left atrium without complications . This is one of the long-term survivors. Among the 51 myxoma s reported in this series. only 1 showed obvious calcification of the tumor tissu e. Another patient. who died from guns hot wounds , was found at autopsy to have a large unsu spected atrial myxoma tha t measured 5.3 x 3 x 4 em. Follow- up

Recurrence after removal of a myxoma is rare (1, 10. 12). Among 36 myxoma s that were diagnosed and resecte d surgically. only 1 tumor recur red - 4 years later at the same site . as shown at au topsy after the patient's death from a malignant tum or in the abdomen . One patient in the followup gro up (who died accidentally from guns hot wounds) was found to have no evidence of recur renc e of a myxoma resected 8 years ear lier. Anothe r patient was operated on 6 years earli er for an atrial septal defect tha t was closed surgically with no evidence of tumor at the time; 6 years later . the patient was diagnosed as having a left atrial myxoma. was operated on. and had the tumor remo ved. The base of implant of this myxoma was in front of the previous sutur e line where the patch had been placed to close the ADS 6 years earlier . In 1977 . a patient expired eight mont hs after resection of a left atria l myxoma due to an acute myocard ial infarction caused by at herosclerotic diseas e. Autopsy showed no evidence of recurrence of the at ria l myxoma .

8) Duringtheatrial systole and ventriculardiastole,the tumor massmigrates acrossthemitral valveinto theventricular chamber.The site of implant is localized to theinteratrial septum, abovethe mitral valve

Anothe r patient, 66 years old. died from a ruptured abd ominal aortic ane urysm 2 months after resection of a left at rial myxoma. No residua l tumor was found at autopsy. One 8year-old patient was opera ted on for an atrial septal defect and pulmonary stenosis . At that time. a primary closure of the ASD was accomplished and a pulmonary valvotomy undertaken. But 6 years later, the patient had arrhythmias and signs of left A·Vvalve obstruction. Cardiac catheterization revealed a large mass in the left at rium; a reope ra tion was undertaken in October 1969 . A large left atrial myxoma was removed . and the patient remained asymptomatic 10 years later. In summa ry. left atrial myxoma is the most common benign tumor of the heart that is now easily diagnosed by echoca rd iograph y (Fig. 1). It should be suspected in patien ts of any age with symptoms of left-sided AV valve obst ruction and signs of card iac congestive failure . Unless the status of the coronary vasculature is of interest for the care ofthe patient, echocardiogra phy is usua lly sufficient to procee d with the surgery. The long-ter m prognosis is quite favorab le; surgery shou ld be performed as soon as the diagnosis is mad e in order to avoid complications, mostly related to embolization by tumo r tissu e (3. 4. 5, 13), Rhabdomyoma This benign tumo r was the most common in newbo rn s and infants (5. 9. 15), In fact. 9 of the 14 tumors reported here were found in infants less than 2 weeks old. "lith a mea n age of 6 days (Table 4). The tumor can be of any size from a few

Age < I S days (mean .. 6) 18 months 3 years 13 years 16 years 26 years Total

9 1 1 1 1

1 14

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Fig. 1 Echocardiographic image obtainedofaleft atrial myxomausingthe tov-c hemt ervew LA '" left atrium, RA .. right atrium, LV.. left ventricle, RV = right ventricle, Mx '" myxoma A) Duringthe ventricularsystolethetumorremainswithin the atrial chamber, abovethe mitral valve,which is free of tumor involvement

185

Thome. cardiol'asc. Surgn38 (1990/

1. E. Molina . .J. Ii. Edwards. lf. B. H/ard

millimeter s in diameter to so extensive and multicentric that the entire chamber of the right or left ventri cles is involved , cau sing significant heart failure in earl y infancy (Fig. 2). If the tumo r involves a substantial portion of the heart, par ticularly the ventricles, the child us ually dies of heart failure within a few da ys. Opera tive experience with th is tumo r is also disappointi ng because it is multicentric, i. e.. involves th e ventricles extensively, precluding adequate resection. This tum or is rarely seen in the adult population ; thu s, only sporadic cas es have been reported. The only ad ult case in our review wa s a 26-year -old with a small rhabdomyoma. Fig. 2

Extensive in·

vclvement of multiple rhabdomyomas of the heart in a s.oav-olo infant

pap illary pattern, either in the valvular tissue or in the pap illary muscles, Only 1 of the 9 fibromas was found in an adult patient, who died suddenly from other causes. It is a tumor of the young, with the same implications as the rh abdomyoma s, de pending on the exten t of invasion or involvement throughou t the heart. Teratom a

Only 2 cases (2%) of this tumor were found in our series. Although histologically benign, it ma y present serious problems due to its size and location (6, 13). One teratoma, in a 9· week-old infan t, caused compression of the right ven tricular outflow tract. A subtota l resection was accom plished including the roof of the left at rium, which was involved without comprom ising the right corona ry artery. The result was quite satisfactory. The patient has been followed up for 5 years with no evidence of recurrence. The second patient was also an infant , 2 vears old, with extension of the tu mor in the anterior mediastinum. This was also res ected with good marg ins, and the patient has done well since. Other Hare Benign Tumors

Papilloma

This is a very sma ll, proliferating tum or that does not rea ch mo re than a few millimeter s in diameter (4, 9). The typical location is in the valve tissue, in the cordae, or in the valve leaflets themselves. In genera l, all valvular tissu e is prone to develop these tumors, which most commonly occur in old age . There were 12 cases (11 % ) in this series in patie nts ranging from 57 to 84 years of age, with a mean age of70.8 (Tables 1 and 2). The location was mostly around the mitral valve, but was also in the pulmon ary or aortic cusps. The tumor , usually a post mor tem finding, does not cause hemodynamic ab normality. It is very importan t to keep this in mind in cases where bacte rial endocarditis is diag nosed or sus pected, and echocardiogra phy is used for follow-up to look for vegetations, because of the thera peu tic implications. Fibrom a s

The fibroma, in our review. was the 4lh most common benign tumor ofthe hea rt (9 cases = 9 %). It is closely related to the rhabdomyoma since it occurs within the myocardium itself. It is sma ll and mult icentric . Lik e the rhabdomyoma, the fibroma may reach large proportions in relationship to the size of the heart, and is most common in youngc hildren. This type of tumor ma y essen tially constitute a vari ant ofthe rhab domyoma in which no obvious est riated muscle tissue can be found , but only a fibrous pattern . Half of the fibromas are located in the endocardial surface a nd may develop a

Among the rare , benign tumors of the heart were 4 hama rtoma s and 2 lipomas (1 of them very large and pediculated externally from the epicardium, with its bas e in th e A-V groove). Another ra re tumor was the mesothelioma, located in the A-V node. There were 2 patients with this type of tumor in 1967 . Curiously enough, it was also small but because of its location in th e A-V node, led to conduction pr oblem (4 ). One of these 2 patients actua lly died suddenly; the post mortem examination revealed the mesothelioma only in the A-V node, The second patient was found to have the type of tum or at the post mortem examination, after a failed attempt to correct a VSDin the presen ce of pulmonary hypertension. Two tumors, classified as glomangioma, (a variety of angioma ), wer e located in th e A-V node. Both patients died sudde nly with no other findings at aut opsy. The location of these tumors, therefore, is very important; we question whether any patient with a suspicious tumor ma ss in that area of the A-V node should undergo implant of a pacemake r system. One case of cavernous hemangioma mentioned ear lier was found at post mortem examinat ion in an infant which had died of heart failure (Fig. 3). The left ventricular wall was extensively involved, des pite the histologic benign characte ristics of the tumor. Ma lign ant Tum ors

\Ve found a total of 21 malignan t tumor s, all sa rcomas . They fit into 8 categories. which constituted 17 % of the total number of tumors. The age ra nge of patients was 4 to 68 yea rs, with a mea n age of3 5.7, which is somewhat younger tha n the mean age found in myxomas . There was equal distribution among sexes: 11 ma les and 10 female s. The distribution of malignancies among all ages of patien ts was abo ut even (see Table 4). At the time the tumo r was diagnosed, 26 % of them had already metastasiz ed to the lungs, brain, or other organs. There were 9 patients who were diagnosed preoperatively an d underwent surgery in an att empt to resect the tumor; 8 patients su rvived the operation and und erw ent chemotherapy in various combina-

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186

Primary Cardiac Tumors: Experience at the Umoersu u ojMirlT/esota

Thome. cardiol'Qsc. S urgn38 (1990)

187

Fig. 4 Angiosarcomain a43·year-old man involvingmostof therightatrium, anterolateralwall,and upper portionof the right ventricle(arrowsl: 80 %resection 01the rightatriumwasundertaken With partial resection of therightventricle [see textl

tions. The mean survival of these patients was 6 months. with a range of 2 to 9 months. Of the 21 tum ors. 19 originated in the atrium. mostly on the right side . Only in 2 patient s with rhabdomyosarcoma did the origin appe ar to be in the vent ricle.

Rhabaomuosarcomu The most common maligna nt tumor was the rha bdomyosa rcoma . The 6 cases represented 29 % of the maligna nt tumors 5 % of the total numbe r of ca rdia c tumo rs; 4 originat ed in the at rium and 2 in the ventricle. Each one of the card iac chambers was involved : 2 in each of the atria and 1 in each of the ventricles. None of these patients underwent su rgical resection because of the adva nced stage of the disease at the time of diagnosis. Chemothera py in 3 patients was completely ineffective: they died in less than 4 months. Angiosa rcoma

This was the 2nd most common type of tumor found in the series. Four patient s. all diagnosed pre operati vely. had angiosar coma 09 %1. The tum or was located in the right at rium in all cases . Only 1 patient un derwent elective resection 181. \vhich involved excising all of the entire right atrium an d part of the supe rior aspect of the right ventricl e. but preserving the tricusp id valve appa ratus (Fig. 4). The right corona ry arte ry was bypassed with saphenous vein graft; no obvious metastase s were seen at the time of surgery. The patient. however. had recurrence within 3 months and died 6 months later despite chemotherapy. He was the first one. diagnosed with only echocardiography. an d operate d on without previous angiog rap hy 181. The othe r 3 patients were dia gnosed but not operated on because of the advance d stag e of the disease . which had alread y metastasized to the lungs. perica rdiu m. inferior vena cava . and the brain. My x os arcoma

This tumo r. the malignant version of the myxoma . was found in 3 instan ces (15% of malignant tumors and 2.4% of the total number ofcardiac tumors). The ages ofthe patients were 16. 22. and 65 yea rs . Two of them underwent su rgical

resection. With the Ib-ye ar-old, seen before the echoca rdiograph y. 2 month s elapsed before the diagnosis was made. At the time of surgery. the tumor was thought to be a benign myxoma. and there was no res ection done of the interatrial septum in continuity with the tumo r. Within 1 year, however . the patient returned \..-ith recurrence of the tum or and underwent reoperation. However. by this time the tumor had already extensively invaded the pulmonary veins. the pericardium. and the right ventricular outflow tract in continuity with the root of the aorta (Fig. 5). The patient was treated with chemothera py after pa rtial resection of the tumor. He developed distal metastasis, and further chemotherapy was ineffective at that stage ; he died 3-112 years after the second operatio n. The second patie nt underwent resection of a myxosarcoma at age 17 with wide excision, rem oving the entire inter atri al septum and replacing it with synthetic patch mat erial. Neverthe less . 5 yea rs later, the patient returned with recurrence of the tum or this time with complete obstruction of the right pulmonary veins and also invasion of the root of the aorta . the outflow tra ct of the right ventricle, and the mitral valve. With evidence of metastatic tumor in the lungs. the patient was placed on chemotherapy. but died 3 months later . The last patient. 65 years old. was diagnosed with extens ive involvement of the ca rdiac chambers: no surgical resection was recommended. li e survived only 2 months . Fibrosarcoma

There were 2 cases of this tumor (10 %). both originating in the right atrium . One of the patients was 67 years old and showed ea rly signs of congestive failure ortho pnea and decompensation with hemoptysis. but the diagnosis of the tumor was made postmortem. It origina ted in the right atrium an d in the area of the left atrial appendage. filled the enti re at rial chamber. invaded the pulmonary veins and left ventricle. and metastasized to the lungs. The other patient was 64 vears old. The tumor had actuallv caused the acute ru pture"of the left atrial appendage an d caused sudden death from hemorrhage . Autopsy showed invasion of the upper left ventricular wall. the root ofthe aorta . the pulmonary veins. and the per icardium .

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Fig.3 Histologically benignhemangIOma showing extensiveinvo/llement of theleft ventncutar wall and septum at emeversot the ventncles

1. E. Molina. J. H. Edumrds. /I. 11. H-ard

71lOmc. ca rdiol'asc. Sllr,qn38 (1 990)

Fig. Sa

Fig. 5b

Fig. 5 Autopsy specimenofthemyxosarcoma that recurred withinvasion ofthe pulmonaryveins, lett atrial wall, andmitral valve

Sp indl e Cell Sarcoma There were 3 cases (14 % ) of this peculiar tumor tha t originates in the gre at vessels. Two of these tumors originat ed in the ao rta itself and 1 in the pulmonary artery. All 3 were operated on with the intentio n of resectin g th e involved segments. However . the tumor of pulmona ry artery origin showed exten sive invasion along the vesse ls of the right as well as the left pulmona ry arteries. causing a conside ra ble amount of obst ruction and pulmo na ry hypertension. The site of origin seemed to have been the bifur cation of the pulmonary arte ry trun k. The patient was given radiation thera py. but died 2 months late r. In the other 2 cases, sp ind le cellsa rcoma originated in the root of the ao rta and exte nded along the ascend ing aorta and into the a rch. Both patien ts underwent tentative resection that was not acco mplished satisfactorily. and residual tum or was left beh ind. Within 2 month s. the ma lignanc y recurred and both patients died despit e chemothe rapy. Other Tumors In addition. we found single cas es of leiomyosar coma , reticulum cell sa rcoma. liposar coma. and malignant histiocytoma. Two of these patients underwent tent ative, but uns uccessful. resec tion. Surgical Approach Technically. the resection of cardiac tumor s depends on the type of tumur and its location (4. 5.13 ). In the case of atrial myxoma , the sta nda rd approach is either via a longitudinal at rloto my in the interatrial groove (4, 5. 13l with the patient on tota l ca rdiop ulmonary bypas s an the hea rt arrested. or via a transseptal app roach with a right atr iotomy. The most common is the left at riotomy along the interatrial groove, becau se it places the operator close to the septum \vhere the tum or origina tes (Fig. 61. One should be ca reful technically not to ope n the atri um until the hea rt has been arrested in orde r to preven t embo lism by tumor tissue or by air. Part of the sept um should always be rese cted in contin uity with the base of the tumor to prevent any possible recurrences

(Fig. 7). \Vhen in doubt , the enti re interatrial septum should be resected an d substituted with prosthetic pat ch mate rial. The tumor should be remo ved very gently to pr event disintegrat ion and embolization (5, 9). The operation is very simple and should have a very low mortality rate. In fact, none of these patients in our series had postoperative complications. The second approach is tr anssepta l. wh ich may be more convenient ift he patient has had previous surgery or if the left atrium is not enlarged (Fig. 8). After cannulating both vena cava s and encircling them with tourn iquets , th e right atrium is usually opened parallel to the A-Vgroove (or tra nsversely from the right atrial appendage to the junction of the right pulmonary veins). The incision in the interatrial septum sh ould be made as a nap following the foramen ovale contour posteriorly and anteriorly until the nap is made with the fixed base on the intervalvular ridge (Fig. 81. The septum swings over its bas e between the 2 A-V valves toward the right. In this manne r the entire area of implant of the tumor is clea rly visualized: the tumor is eas y to remove and no important structures are damaged. This exposu re is outsta nding. particularlyin children and patients with small heart s. After the tumor has been removed, the interatrial septum is substituted by a patch of pr osth etic material. if necessary. There is no need for an unusual am ount of retraction to expose the enti re area. In the cases of malignant tumors of'the right at rium the extent of resection depends on the degree of tumor (8). The case shown in Fig. 4 was a 43-year·old ma n with an an giosar coma who underwent resection of nearly 80% of the atrium , where the thickened wall was constituted by the tumor. However. on reaching the A-V groove. the tum or extended into the base of th e ventricle (Fig. 4 ). which has to be resected. But the tricuspid valve ap paratus had to be preserved. The right corona ry a rte ry was sacri fied and bypasse d with a saphenous vein gra ft to the posterior descending vesse l. This par ticular patie nt did quite well for about 6 months but then died despite 2 separate schedules of chemotherapy.

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Primary Cardiac Tumors. Exp erience at the University ofMinnesota Fig. 6a

Thom e. card/atlast'. Surgn 38 (] 990)

189

Fig. 6b

Fig. 6 Standard approach to theleftatriumviaa verticalincision parallelto the interatrial groove; P .. pulmonaryveins, A .. aorta.An atrial myxoma isshown in its typical location A) line of incision BjExposure of thelett atrial chamber

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Fig. 7 Lettatrial myxoma excisedshowingthebase of the tumorwithpart of the interatrialseptumremoved.Thisisthe same tumorshown on theimages in Fig, 1

Fig.8a

Fig.8b

Fig. 8 Interatrial transseptal approach Aj line of incision along thelandmarks of the foramen ovaleareindio cated. Anasteriskindio cateswhere the incisioninthe interatrial septumismadeinitially: arrows indicatethe directionof theincision along theposteriorand anterior limits

Bjlnteratrial septum retracted toward the right, exposingthe left atrial chamber andthe mitralvalve; P = pul· monary veins, A = aorta,T = tricuspid valve, M = mitral valve, S = interatrial septum

190 Thome. eardio/lose. Surgn38 fl990)

1. E. Molina , J. E. Edwa rds. II. fl . Ward

Resection ofRhabdo myomas Infants with large fibromas or rhabdomyo mas represent a serious problem. The tumor often involves half or more of the enti re cardiac wall, including ventricular septum, anterolateral wall, and anterior right ventricular wall (15). These tumors are so extensive that they exert compression in the pulmonary outflow tra ct and also lead to poor contrac ta bility of the ventric ula r chamber. which is reduced to a thi rd or half of the norm . Unless the tumor is small. the chance of an infant su rviving an adequ ate resec tion is low. Other Tumors The resection of pap illomas is probably not indicat ed as a primary operation. but. if in the course of other procedu res papillomas are found. they may be of such a size as to require simultaneous excision . There is no known recurrence rate . In general, these tumors ar e found in olde r pa tients. Discussion Cardiac tumors. at present. are relatively easy to diagnose with echocardiography. Benign tumors are most frequent than ma ligna nt. The diagno sis. treatment . and results of surgery in benign cardiac tumors is very effective. There fore. any patie nt with symptoms of shortness of breath. ar rhythmias . palpitations, or chest pain sho uld und ergo echocardiog rap hy. The incidence ofthe various tumo rs in our serie s pa rallels the wor ld literature and previous reviews (2. 4. 5. 7,9. 16). The importance of our study is tha t it is the experience of only one institution and could serve as a guideline for any future independent study. The system of classification has also improved from the confusing terms used in the 1950s and 1960s. Many of the tumors that were consid ered to be "pseudomyxomas" were not hing but large thrombi in patie nts with severe mitral stenosi s. But in old hos pital records , patients were often classified as having pseudotu mors. We included only tumors that were neoplasias. with definite histologic evidence.

Although myxomas may be found in the right atri um the incidence is rather low. Our study showed only 5 myxomas origina ting in the right atri um vs. 45 in the left (Fig. 9). The outlook for remova l of benign tum ors is quite good and the long-term survival is highly satisfactory. This is not so for malignant tumors , most of which cannot be resected totally. and the long-term prognosis is very poor (4. 5. 11. 14). No intra card iac teratomas were found in our series. but recent reports in the literatur e indicate 4 cases of intracardiac positions worldwide (6). Up to the present, there is no effective chemothe rapy schedule to treat patients with malignan t primary cardia c tumors - either adjuva ntly with surgery or posto peratively. There have been scatte red reports of some success using cobalt and cyclophosphamine (11), but these were earl y reports and the long-term prognosis of these patients has been poor. Ther efore. if a malignant tumo r is diagnosed in its ea rly stage . ca rdiopulmonary transplantat ion might be considered ifno metastases are presen t.

Referen ces 1

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Atfan.A.A. . G. S. Johnson, Z. Masr i, R. Girardet, and A. M. Lansi ngMalignan t clinical behav ior of cardiac myxomas and "Myxoid Imitato rs". Ann. Thor . Surg. 44 (1987) 217-22 2 BurakoL's ky. V. 1.. G. 1. Zucker mann, G. A. Kossatc h. M. A. GotossOL'skaya, and L. A . Jarol'skaya: Surgical treatment of cardi ac myxomas. J . Thorac. Cardiovasc. Surg. 96 (1988) 800-805 Chamberlain. S. W.. J. R. Carter. and R, L. Richards on: Intraoperative coronary artery embolizat ion from left at rial myxoma . Anes thesio logy 47 (1977) 301 Chitwood. W. R.jr.: Cardiac Neoplasms: current diagnosis. pathul ogy, and therapy. J. Cardiac. Surg. 3 (t 988) 119- 154 Uem. J. R.. W. 11. Frist, E. B. Stinso n, et ul.: Prima ry cardi ac neoplasms. J. Tho rac. Card iovasc. Surg. 93 (1987) 502 - 511 Farooki .Z, Q.• C. H. Challg. W. L. Jackson. S. K. Clapp. M . Haki mi. E. Arciniegas. and W. W. Pinsky: Intracardiac terato ma in a newborn Clin. Cardiol. 11 (1988) 642 -644 Fine, G.; Neoplasms of the perica rdium and hea rt. In S. E. Gold: Pathology of the heart and blood vessels. Springfield. I. L., Charles C Thomas . 1968 . pp. 851 -883 Fye. W. B" and J. E. Molil/a: Hil':hl atrial angiosa rcoma : echocardiographic diagnosis and surgical correction. Johns lIopkins Med . J.1 47( 1989) 111-1 16

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Fig.9 Right atrial myxomafillingin the right atrial cavityand protruding into the rightventricle, causing obstruction of the tricuspid valve

Primary Cardiac Tumors: Experience al lh e University cfMinnesota

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l. Ernesto Molina. M. D., Ph. D. Box 182 UMIlC University of Minnesota Hospitals 420 Delaware St. S. E. Minneapolis . M~ 55455/US:\

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. , Goodu·in. L /0:: Sympos ium on ca rd iac tum ors : the spectrum of ca rd iac tum ors . Am. J. Card iol. 21( 1968)307 10 Hannah. 11. , G. Eisemann. R. lIu%Z.czyn sk yi. .\1. U'insky. and L. Cohen: Invasive atrial myxoma: docu mentation of malignant potential of ca rdiac myxomas. Am. Heart J. 104(1982) 104 - 881 11 Hollingsu'Orth. l . H.. and B. C. Sturgill: Trea tment of primary angiosarcoma of th e heart. A. Heart J. 78 (1969) 254 - 258 1% Mart in. L. IY.. A. G. Wasserma nn. 11. Goldstein. J . S. S tei nberg. M Miffs. an d R. J. Katz : Multiple cardiac myxomas with multiple recc urre nces. unusual pre se ntations of a "benign" tum or. Ann. Thor. Surg. H (1987) 77-78 Il Poole. G. Y., R. H. Brey er, R. II. Holfid ay . A . S. Hudspet h, F. R. Jonnenon. A. R. Cordell. and S. A . Mills: Tumors of the heart: surgical considerations. J. Ce rdto vasc . Surg. 25 (1984) 5 I ~ Ross i. N. P., 1. M. Kioschos. C.A. A sch enorener, andJ. L. Ehrenhef t. Pri mary angiosarcoma ofthe heart. Cancer 37,892-894 15 Shober , R. M.. J. Mintzer, M . Farina, U. Alley, and M. Bishop: Clinical presentation of rhabdo myoma of the heart in infancy and childhood . Am. J. Card iol. 30 (1972 ) 95 1& S trauss, R., and R. Mer/iss: Primary tumor of the heart. Arch. Pathol. 39 {1945) 74

Thome. cardiol'ase. S urgn 38 (1990)

Primary cardiac tumors: experience at the University of Minnesota.

A review of all primary cardiac tumors seen at the University of Minnesota or entered in the Registry of Cardiovascular Disease, affiliated with the U...
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