Intracardiac sarcoma diagnosed by left ventricular endomyocardial biopsy

Intracardiac sarcoma diagnosed by left ventricular endomyocardial biopsy. F H Hausheer, R A Josephson, L B Grochow, D Weissman, J A Brinker and H F Weisman Chest 1987;92;177-179 DOI 10.1378/chest.92.1.177 The online version of this article, along with updated information and services can be found online on the World Wide Web at: http://chestjournal.chestpubs.org/content/92/1/177.citation

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clinical evidence suggesting recurrent arterial emabsence of signs of infection, and negative blood Other clinical clues include the presence of a coagulopathy and right heart catheterization.e Echocardiography is a powerful tool which is able to assist in the antemortem diagnosis of the condition. Estevez and Corya’#{176}described the serial M-mode echocardiographic features of NBTE of the mitral valve in a patient in whom the diagnosis was confirmed at surgery. Siegel et al’5 reported on a patient in whom two-dimensional echocardiography demonstrated the presence of a cluster of freely mobile vegetations associated with the tricuspid valve. Although they had strong circumstantial evidence that these lesions repre-

ditis:

murmur,

bolization, cultures.’

sented

NBTE,

there

was

no

direct

autopsy

is the

validation.

The

of

absence

microscopic

hallmark

inflammatory

an

cell

CM,

patients.

Corya

nonbacterial

Chest

1976;

Siege!

RJ,

JF,

Am Howard

a neurologic

of 99

Estevez

11

Toole

endocarditis:

correlations in

VR,

Serial

thrombotic

1976;

92:723-29

Nonbacterial

perspective

Arch

BC.

J

Heart VJ.

throm-

of clinicopathologic

Neuro!

39:95-98

1982;

echocardiographic

abnormalities

endocarditis

of

the

mitral

valve.

69:801-04

LE,

Ginzton

endocarditis:

diagnosis

Flanagan

by

2-D

K,

Criley

JM.

echocardiography.

Marantic

Chest

1981;

1st ed.

Phila-

80: 118-19

12 Weyman delphia:

AE. Lea

Cross-sectional

echocardiography,

& Febiger,

1982:230-31

of the twodirect

with

of these

response,

lesions

thus

distin-

lesions from those accompanying acute and subacute bacterial endocarditis.e The specific features on two-dimensional echocardiography which were seen were a well-defined diffuse thickening of the valve cusps with additional localized nodularity and the absence of significant limitation of cusp opening. The other conditions which can cause this picture include valvular degeneration with or without associated calcium deposition, and cardiac amyloidosis.’2 By contrast, the vegetations of infective endocarditis typically appear “shaggy” and ill-defined, may be mobile, may be massive, and may be associated with evidence of destruction of the valve. Increased awareness of the clinical importance of NBTE, together with a knowledge of the clinical settings in which the disease occurs and the appropriate use of echocardiography, particularly the spatially oriented two-dimensional technique, should permit more frequent diagnoses of the condition during life. This will open the way for studies directed at determining the pathogenesis of the process, and ultimately, effective therapeutic measures.

Intracardiac Sarcoma Diagnosed by Left Ventricular Endomyocardial Biopsy*

these

guishing

ACKNOWLEDGMENTS: ance of Suzanne Ellis

We Linda

and

gratefully Inman.

acknowledge

the

assist-

1 Wooley

CF,

Baba

Clinical

2 Garcia!,

Fainstem

Kaposi’s 3 Cammarosano deficiency

V, Bios

BA,

Heart

White

CL,

Clark

thrombotic Cancer DH,

M,

J 1975;

1985; Lumb

thrombotic

endocarditis

pulmonary

artery

Med

1983;

125:126-28

J, et al.

P, Reuben homosexual

Dock

DS.

autopsy

sample:

a review

K, Matsushita

WE,

in bone

Santos

marrow

GW.

F, Killam with

catheterization.

AP.

severe

Nonbacterial

preeclampsia

J Reprod

Med

and

1985;

S, Yamanouchi

LM,

Fayemi

AO.

Non-bacterial

H.

Nonbacterial

and myocardial thrombotic

throm-

infarcendocar-

often

provides

management

rejection,

information

useful

of various

situations

clinical

transplant

include

myocardial

assessment

anthracycline

of

cardiotoxicity,

and myocarditis,’ Seldom does a biopsy yield an unequivocal diagnosis. We present a case of high grade (undifferentiated) primary cardiac sarcoma diagnosed via percutaneous left ventricular endomyocardial biopsy which illustrates several advantages of this technique. CASE

REPORT

The patient was a 45-year-old white man in excellent health ten months prior to admission when he was found to have stage nodular

sclerosing

Hodgicin’s

course of mantle tion. The cardiac valve.’

He

radiation

therapy

features

of cardiac physical

doxus pitched

tion.

was

the

tamponade

effusion on

a mass

and

left

episode. for

involving

atypical

etiologies

recurrent

Hodgkin’s

marantic

endocarditis,

of the disease,

drainage

yielding

650

Pericardial

fluid

cytology

Baltimore

1) were Echocar-

consistent

Oncology,

with

or vegetaincluded

lymphoma,

sarcoma,

endocarditis.

subsequent

catheter

pericardial

negative The

Oncology Center, and the Division of Cardiology, Medicine, The Johns Hopkins Medical Institutions, tAmerican Cancer Society Regular Clinical Fellow. lMedical Staff Fellow, National Institute of Aging, tutes of Health. Reprint requests: Dr Hausheer, Medical Oncology,

Hospital,

posterior

abnormality

bloody

results were

of Medical

most

parahighA large

(Fig

thrombus,

with

of exudative

study

atrium,

wall

culture-negative

performed

ml

Department

be

myxoma,

intracardiac

or (fastidious)

Admis-

pulsus

evaluation.

non-Hodgkin’s

was

left

free to

for

of with

a mid-diastolic opening snap.

the

ventricular

considered

were and

completion he presented

significant and valve

‘11A5

complicalevel of the

a syncopal

were

initial echocardiographic

features

the

foilowing

following

until

a subsequent

when

Kussmaul’s sign, with a mitral

and

process,

Possible

remission

results

leaflet,

completed

day of admission,

examination

valve

He

radiation therapy without were shielded below the

in clinical

until

of 20 mm Hg, sound compatible

wFmm

disease.

and periaortic structures

Pericardiocentesis

transplant

55:631-35

botic endocarditis as a cause of cerebral tion. Jap Circ J 1984; 48:1000-06 8 Deppisch

Typical

cardiac

neoplastic

of

30:497-500 7 Kuramoto

diseases.

diographic

throm-

MS., M.D.;’t MS., M.D.4 M.D.; David Weissman, M.D.; M.D., and Harlan F. Weisman, M.D.

Endomyocardial biopsy in the diagnosis and

observed

immune 5:703-06

Nonbacterial

AW, Beschorner

associated

A. Brinker,

mitral

90:190-98

Jelovsek

B. Grochow,

pericardial

with

143:1243-44

lesions in acquired Coil Cardiol 1985;

endocarditis PD,

Louise

Jeffrey

sion

endocar-

1970;

in a male

Med

A, Otake

Am

Nonbacterial 6 Chestnut

Intern

thrombotic

M, Manse!!

in a Japanese

cases.

patients.

A, Luna

C, Lewis W. Cardiac syndrome (AIDS). J Am

endocarditis

5 Patchell

Intern

endocarditis

Arch

F, Kodama

eighty

Nonbacterial

Arch

thrombotic sarcoma.

4 Chino

JM.

recognition.

Nonbacterial

botic

N, Ryan

Frederick H. Hausheer, Richard A. Josephson,

mitral

REFERENCES

ditis.

Challa

proof.

Our study is unique in that it is the first report dimensional echocardiographic features of NBTE autopsy

J,

botic 10

correlations.

clinicopathologic

9 Biller

fluid.

for malignant Johns

Hopkins

Department Baltimore. National

Johns

of

Insti-

Hopkins

21205

CHEST

Downloaded from chestjournal.chestpubs.org by guest on July 11, 2011 © 1987 American College of Chest Physicians

I 92

/ 1 I JULY,

1987

177

cells.

Hemodynamic

Diagnostic rate

and

chest,

status

stabilized with

thoracocentesis

biopsy,

and

abdomen,

computerized

and

pelvis

following

pleural

the

biopsy,

bone

tomographic

were

negative

procedure. marrow

aspi-

examination

of the

fur malignancy

or infec-

and

coronary

tion. Left

and

revealed

stenosis,

significant

originating

from

in the

basilar

lesion

seen

both

left

left

of the

pathologic

studies

the

right

2).

Due

percutaneous wall

coronary

arteries to the

to

the

performed.

sarcoma

was

A

biopsy high

documented

endomyocardial

mass

uncertain

catheter-guided

was

left

mitral

neovascularization

corresponding

(Fig

cell

from

and

ventricle,

free

spindle

and

circumflex

left

mass,

ventricular

(undifferentiated)

(Fig

the

insignificant

regurgitation,

of the

selective

constriction,

echocardiography

nature

the

catheterization

pericardial mitral

region on

histologic of

cardiac

right

arteriography

by

biopsy

grade histo-

specimen

3).

Discussion 1. Echocardiogram (long axis view) showing echo (arrow) in the region of the mitral valve and left ventricular (v); ao= aorta; a=left atrium. FIGuRE mass

dense wall

This cult

patient’s

issues.

and

presentation

An

atypically

located

course

raised

primary

diffi-

several

cardiac

tumor

was

considered, though it was believed to be too early for radiation-induced primary sarcoma3 or non-Hodgkin’s lymphoma. Since the cardiac structures had been relatively spared from irradiation, consideration was given to the unusual

possibility

of

of Hodgkin’s

rence formulate

an optimal

of an extensive biopsy

was

“marginal

disease. treatment

evaluation,

successful

miss”

A tissue

intracardiac

diagnosis

approach.

left ventricular

in providing

a tissue

recur-

was needed to After completion endomyocardial diagnosis

without

complication. cardiac malignancies are rare. The reported at autopsy ranges from 0.0017 to 0.25 percent, as to 1 to 22 percent for metastatic cardiac involveSarcomas are the most common primary cardiac malignancies, and usually originate from the right side of the heart or pericardium. Patients with cardiac sarcomas present at an average age of 40 years, but can range from 10 months to 79 years.46 The clinical manifestations are usually subtle, but in some instances there may be dyspnea, elevated jugular venous pressure, edema, embolic phenomena, hypotension, or tamponade from effusion or neoplastic encasement.7 Primary

incidence compared

FIGURE

“puddling” arrowhead).

2. Left of

coronary tumor

angiogram vessels at

the

showing left

neovascularization ventricular

base

and

(small

There

are

no

reported

biopsy

cases

of

left

ventricular

endo-

identification

primary cardiac malignancy.8 may be useful in situations where pathologic of left-sided cardiac abnormalities might signif-

icantly

clinical

myocardial This

method

alter

diagnosing

management.

Echocardiography,

an-

giocardiography, gated CT or magnetic resonance imaging scans are useful in evaluating the presence and extent of cardiac involvement, but none of these can establish a histopathologic

lesions biopsy nosis. than routine

3A. Photomicrographs of patient original nodular ing Hodgkin’s disease. B. Undifferentiated spindle cell cardiac sarcoma obtained by left endomyocardial biopsy. FIGURE

178

sclerosprimary

diagnosis.9

Diagnosis

of

intracardiac

mass

is one of the few contexts in which endomyocardial may be conclusive rather than suggestive of a diagThis procedure offers less morbidity and mortality thoracotomy with approximately the same risk as cardiac

catheterization.’

An

additional

advantage

of

endomyocardial biopsy is that it may permit diagnosis in patients with high operative risk. it is important to note that this method obtains a small sample of tissue, which by virtue of possible sampling error may allow for misinterpretations of the neoplastic process. There is also a risk of systemic embolization of friable tumor tissue with subsequent infarction or metastases. Intracardiac

Downloaded from chestjournal.chestpubs.org by guest on July 11, 2011 © 1987 American College of Chest Physicians

Sarcoma

(Hausheer)

Primary and secondary cardiac malignancies generally portend a poor prognosis, but some metastatic tumors are now curable with specific therapy (eg, testicular cancer and lymphomas) and long-term palliation is achievable with many more. Since survival is improving in many oncologic disorders, the incidence of cardiac malignancy may rise and the use of methods such as echocardiography may increase antemortem recognition of this involvement. In cases with suspected cardiac involvement, it may be difficult to establish the diagnosis. Endomyocardial biopsy may be useful in such instances to diagnose and optimize management without the risk of thoracotomy. The relative diagnostic and treatment influence of this procedure will need clarification by

further

experience.

ACKNOWLEDGMENT: We greatly appreciate the diagnostic sistance and expertise of Dr. Francis Kuhajda regarding interpretation of the patient’s cardiac biopsy histopathology specimens.

as-

amiodarone-induced pulmonary toxicity in a patient with bilateral exudative pleural effusions and toxic involvement of other organs. We review amiodarone-associated pleural reactions reported in the literature. (Cordarone, Wyeth ful antiarrhythmic drug’ that increasing multi-organ toxicity.2 mensch et al3 linking pneumonitis we have learned that amiodarone-related

Laboratories), is a powerhas been associated with Since the report by Rotwith amiodarone therapy,

miodarone

malities

can

be

detected

in many

pulmonary asymptomatic

abnorpatients

and

can progress to significant pulmonary insufficiency.4 Pleural involvement associated with antiodarone treatment, infrequently described as an incidental radiographic finding,7 has not been clearly documented. We describe a patient whose ainiodarone-induced exudative pleural effusions and pleuritis spontaneously resolved after withdrawal of asniodarone treatment.

REFERENCES 1 Mason

JW.

dial biopsy.

Am V, Gardner

2 Page the

technique.

of

the

Radiology EC,

tissue

left ventricular

1970;

Greenberg

Ibilowing

dosimetric

aspects

I. The

of

of

HD.

Sarcoma

Hodgkin’s

of bone

disease.

and

Cancer

soft 1984;

4 Straus

R, Merliss

39:74-78 5 Malaret

CE,

1968;

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of the

tumor

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heart.

disease

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to the

Pathol

1945;

heart.

6 Clancy

DL,

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JB, Roberts

1968;

WE

Clinical

PC,

Shub

WC.

of the

Angiosarcoma

9 Stark

aspects C,

of cardiac

Seward

diagnosed

1983;

tumors.

Am j Cardiol

1968;

DD,

by

Higgins

et al. Magnetic

and

pathologic

CB,

findings.

Lanzer

and Daisy

P, Lipton imaging

Radiology

was

1,600

1,200

he complained

following

ventricular

biopsy.

halo

mg

mg

ten weeks,

vision,

oral

daily Ten of

dry

lethargy, cough,

was

dosage when

was

anorexia,

and

amiodarone

daily

weeks.

to 800 began

a 30-pound

progressive

dyspnea.

began

two

weeks,

for

symptoms

but

Because

reduced

per.

patient

and

amiodarone

vision,

normal.

the

His

starting

night

was

of conven-

dysrhythmias and

after

Nine

infarction

combinations

eight

func-

admission,

ventricular

myocardial

ventricular

he gradually

to

pacemaker.

discontinued

weeks

fuzzy

prior

symptomatic

various

fur

examination

amiodarone

cardiac

were

subsided.

ophthalmologic

years

and with

drugs,

medications

angiographically ventricular

sequential

cardioverted

therapy

with

two

he experienced

antiarrhythmic

by

Intracavity

MJ, Schiller

ther-

of

result

of stable mg

daily

for

experiencing weight He

an

cardiac the

malaise,

loss, took

nausea, no other

N, Crooks

of the pencardium: 1984;

normal

150:469-74

Toxicity

Pulmonary

Exudative

Effuslons* Gonzalez-P.othi,

E.

LE. left

as Bilateral

Presenting RlcardoJ.

Wold

endomyocardial

resonance

Amlodarone

Pleural

JB,

syndrome

with normal

84:195-98

LE,

Stephen

These

status,

melanoma Chest

oral

sisted.

and

atrioventricular

Despite

tional

apy

21:413-19

21:328-43 8 Hanley

He

out.

dysrhythmias

heart.

sinus

smoker

disease

to admission

tachycardia.

followed Morales

sick

prior

treatment

Cancer

22:457-66

artery

a permanent

months

ruled

53:232-36

Am

developed

REPORT

30 pack-year

coronary

requiring

Suit

former

documented tion

mantle

96:609-18

MS.

treatment

and

lymphomas.

malignant

CASE

endomyocar-

A 62-year.old

J Cardiol 1978; 41:887-92 A, Karsurar R. Physical

radiotherapy

3 Halperin

and

fur right

Techniques

Hannan,

M.D.,

M.D.;t

A. Franzlnl,

RC.C.R;

C. Ian

Hood,

MB.,

Ch.B.;

M.D.

Therapy th the antiarrhythinic drug amiodarone has been associated with drug-related side effects. in addition to pulmonary fibrosing alveolitis, anecdotal reports have alluded to incidental pleural involvement associated with amiodarone. We describe an unusual manifestation of *

From

the

Pulmonary

Center, and the sity of Florida, tDr. Hannan is a Lung Association Reprint requests: Medical Center,

Division,

of

Medicine

Fellow

Dc Conzalez-Rothl, Gainesville,

Administration

Veterans

Departments Gainesville. Christmas Seal of Florida.

FL 32602

and supported

lilA

Medical

Pathology,

Unives’.

by the American

Pulmonary

Division,

VA

1. Posterior-anterior view of chest roentgenogram at the patient’s illness, after four months of amiodarone therapy. Pre-therapy film was normal (not shown). There is marked blunting of both costophrenic angles with pleural fluid which layered on lateral decubitus views (not shown). In addition there are bilateral fine interstitial markings in the lower lung fields. FIGURE

height

of the

CHEST

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Intracardiac sarcoma diagnosed by left ventricular endomyocardial biopsy. F H Hausheer, R A Josephson, L B Grochow, D Weissman, J A Brinker and H F Weisman Chest 1987;92; 177-179 DOI 10.1378/chest.92.1.177 This information is current as of July 11, 2011 Updated Information & Services Updated Information and services can be found at: http://chestjournal.chestpubs.org/content/92/1/177.citation Permissions & Licensing Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://www.chestpubs.org/site/misc/reprints.xhtml Reprints Information about ordering reprints can be found online: http://www.chestpubs.org/site/misc/reprints.xhtml Citation Alerts Receive free e-mail alerts when new articles cite this article. To sign up, select the "Services" link to the right of the online article. Images in PowerPoint format Figures that appear in CHEST articles can be downloaded for teaching purposes in PowerPoint slide format. See any online figure for directions.

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