Clase acerca de Tumores Cardiacos.

1. Tumores Cardiacos
Abraham Villalobos F RMi

2. Introducción
• Prevalencia en serie de autopsias <0.1%.
• Las metástasis a corazón son 20 veces más
frecuentes.
• Suelen ser asintomáticos.
• Los síntomas comúnmente se confunden con
las patologías cardiacas más frecuentes.
Reynen K. Frequency of primary tumors of the heart. Am J Cardiol 1996; 77:107.

3. Med Assoc J 1934;30:368-373.
TABLE
I
Frequency
of Primary
Heart
Tumors
N
in Autopsy
TU
5. Shelbume
tures which
Series
%
SA. Primary tumors
led to a logical and
1935;9334rk349.
6. Pollia JA, Cog01 W. Some
1936;27:32%333.
8,550
4
0.047
Lymburner4
7. Scott RW, Garvin CF. Tumors
1,200
3
0.25
Shelburnej
17~43 l-436.
Pollia and Gogo
12,000
0
0
8. Benjamin
HG. Primary fibmm
Scott and Garvin’
11,100
0
0
9. Ravid JM, Sachs 1. Tumors
Benjamin*
40,000
12
0.03
“sarcoma
of the left auricle and t
of the mesentety
and alimentary
Ravid and Sachs9
1,888
0.053
10. Straus R, Merliss R. Primary
480,33
1
i3
0.0017
AMA survey
11938-l
942)‘O
11. Leach WB. Primary neoplasm
1,550
3
0.19
Straus and Merliss’O
12. Whotton
CM. primary
malig
Leach’ 1
6,275
0.016
cer lY49;2:245-260.
WhortonJ2
20,337
;
0.015
13. Amsterdam
HJ, Grayzel DM
Amsterdam
et alI3
5,000
I
0.02
heart. Am Heart J 1949;37:291-300.
Prichard14
4,200
4
0.095
14. Richard
RW. Tumors of t
Saphir’5
7,889
2
0.025
hundred
and fifty cases. Arch P
15. Saphir 0. ticoplasms
of the
18,328
23
0.125
FineI
ogy of the Heart. Springfield,
IL
3,914
1
0.026
Heath”
16. Fine G. Primary tumors of
Baumann
and Clavadetscher’*
2,070
4
0.19
JE. eds. Cardiovasc
Clin 5. Clini
Wold
and Lie’9
23,673
41
0.17
Davis 1973:207-238.
10,261
2:
0.049
Fabian
and Rose20
17. Heath D. Pathology
of cardia
26,980
0.078
Schanz
and Schneide?’
18. Baumann
RP. Clavadetsfher
Schwesinger
et aI22
25,855
9
0.035
nanntes myxom, mit embolie in
tibrosarkom.
Schwviz med Wschr
Guang-yingz3
7,423
4
0.054
19. Wold LE. Lie JT. Cardiac m
12,485
7
0.056
Lam et al24
1Y8~101:219-233.
Total
73 1,309
157
0.02 1
20. Fabian JT. Rose AG. Tumou
1982;61:71-77.
Percentage
is based on the total number of autopsies
performed
(N) and
21. Schanz II, Schneider
I. Endo
the number of detected
tumors (TU).
Schwriz med Wschr 19X4;114:850-8
22. Schwesinger
G, Meyer B, v
moren. Z ges inn Med 19pA;3Y:36
23. Guang-ying
L. lncidcnce
an
From the De FrequencyInternal
Medic tumors of the heart. Am J Cardiol 1996; 77:107.
II, University
of ErlongenReynen K. rtment of of primaryine
review of the literature.
Thorac
Niirnberg,
t? Ache
Stadtmauerstrasse
29, 9 10.54
Erlangen,
Ger24. lam KY, Dickens
P, Lam

4. Manifestaciones Clínicas
• Determinadas por la localización del tumor.
• Los mecanismos son variados:
•
•
•
Obstrucción de la circulación -> Falla cardiaca.
Interferencia con las válvulas -> regurgitación.
Invasión directa del miocardio -> alteración de la
contractilidad, arritmias, bloqueos, derrame
pericárdico con/sin tamponade.
Salcedo EE, Cohen GI, White RD, Davison MB. Cardiac tumors: diagnosis and management. Curr Probl
Cardiol 1992; 17:73.

5. Manifestaciones Clínicas
•
Invasión al pulmón -> Síntomas pulmonares.
!
•
Embolización -> Sistémica, la más frecuente.
!
•
Síntomas constitucionales o sistémicos.
Salcedo EE, Cohen GI, White RD, Davison MB. Cardiac tumors: diagnosis and management. Curr Probl
Cardiol 1992; 17:73.

6. Evaluación Diagnóstica
• Ecocardiografía.
• TC y RM.
• PET.
• Coronariografía.
• Biopsia transvenosa.
Salcedo EE, Cohen GI, White RD, Davison MB. Cardiac tumors: diagnosis and management. Curr Probl
Cardiol 1992; 17:73.

7. Tumores Benignos
• 75% de los tumores cardiacos.
• Mixomas
•
•
Los más comunes.
•
El mesénquima de origen puede diferenciarse a
tejido neural y/o endotelial.
•
Productores de VEGF.
Histológicamente compuestos de células dispersas
en el estroma mucopolisacárido.
Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997:
671-682.

8. Tumores Benignos
•
Mixoma
Macroscópicamente
RG ■ Volume 20 • Number 4
•
•
Pedunculados y de consistencia gelatinosa.
•
•
Varía en tamaño que va de 1 a 15 cm de diámetro.
La superficie puede ser suave (>70%), vellosa o friable
(35%).
Peso de 15 a 180 gr.
RG Volume 20 • • Number
RG ■ ■ Volume 20 Number 4 4
Grebenc et al
Grebenc et al 10771077
2.
3.
Figures 2–4. Ca
(2) Photograph de
mass with a varieg
atrial myxoma dem
latinous mass. A p
row) was excised e
graph of a left atria
gated, focally hem
surface.
range in size fro
Cut sections of t
appearance, occ
(1,20).
2. 2.
3. 3.
Radiologic Fea
Clifford-Roberts, W. Primary and secondary neoplasms of 4. heart. Am J Cardiol. Volume 80, 1997: 671-682.
the
Figures 2–4. Cardiac myxoma, pathologic features.
Figures 2–4. Cardiac myxoma, pathologic features.
tures of cardiac

9. Tumores Benignos
Mixoma
•
Manifestaciones Clínicas
•
•
•
80% se originan en la AI, el resto de la AD.
•
Pueden simular enfermedades del tejido conectivo.
(34%).
67% presentan datos de estenosis valvular mitral.
29% presentan embolización (20% síntomas
neurológicos, mayor en hombres).
Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive
cases. 2001; 80:159.

10. Tumores Benignos
•
Mixoma
Síndrome de Gorlin:
•
•
•
•
•
Múltiples nevos con Ca basocelular secundario.
Quistes, fibrosarcomas de la mandíbula.
Anomalías esqueléticas.
Milia, quistes epidermoides, comedones.
Asociado a meduloblastoma, meningioma, fibroma/
fibrosarcoma ovárico, fibroma cardiaco, rabdomioma
fetal y quistes linfáticos mesentéricos.
Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive
cases. 2001; 80:159.

11. Tumores Benignos
•
Complejo de Carney
•
•
•
•
Mixoma
Autosómico dominante.
Múltiples tumores: mixomas intra y extracardiacos.
Schwannomas y varios tumores endócrinos.
Anormalidades en la pigmentación: lentiginosis, nevos
azules.
Pinede L, Duhaut P, Loire R. Clinical
presentation of left atrial cardiac
myxoma. A series of 112 consecutive
cases. 2001; 80:159.

12. Tumores Benignos
Mixoma
5.
6a.

13. RG ■ Volume 20 • Number 4
Tumores Benignos
Mixoma
6a.

15. RG ■ Volume 20 • Number 4
Grebenc et al 1081
a.
b.
Figure 8. Left atrial myxoma in a 61-year-old woman with progressively worsening dyspnea. (a) Non-contrast-enhanced chest CT scan (mediastinal window) shows an ovoid left atrial mass that is hypoattenuating with respect to
the surrounding blood. (b) Contrast-enhanced chest CT scan (mediastinal window) shows heterogeneous enhancement of the myxoma, which is attached to the interatrial septum. The contrast material–opacified blood outlines the
margins of the lobular mass. Note the large bilateral pleural effusions and bibasilar atelectasis.
atrioventricular valve during diastole may also be
demonstrated. Doppler echocardiography can be
used to evaluate associated valvular regurgitation
or stenosis (5,19,31).
Contrast material–enhanced chest CT usually
demonstrates a well-defined spherical or ovoid intracavitary mass, typically with lobular (but occasionally smooth) contours. Tumor attenuation is
lower than that of unopacified blood. Intravenous
administration of contrast material helps to better define the lesion as a mass of low attenuation surrounded by the enhancing intracardiac
blood (Fig 8). Heterogeneity is a common feature of myxoma and is thought to reflect hemorrhage, necrosis, cyst formation, fibrosis, calcification, or ossification (Fig 9) (32).
Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No 4:2000:1073-1103.
a.
b.

16. Tumores Benignos
•
Tratamiento y Pronóstico
•
•
Cirugía: mortalidad <5% a 6 años.
•
Mixoma
Recurrencia: 2-5%
Complicaciones: arritmias u otras anomalías en la
conducción (26%).
Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive
cases. 2001; 80:159.

17. Tumores Benignos
1082 July-August 2000
• Fibroelastoma papilar.
•
•
•
•
El segundo en frecuencia.
55% hombres. 60% > años.
2 a 70 mm. Media 9 mm.
a.
Figure 13. Papillary fibroelastoma in a 69-year-old
man with chronic atrial fibrillation. (a) Transthoracic
four-chamber echocardiogram shows a rounded echogenic mass (arrow) attached to the apex of the left ventricle (LV). LA = left atrium, RA = right atrium, LV =
left ventricle. (b) Axial, cine GRE (66/5) MR image
demonstrates a 1-cm mass at the apex of the left ventricle (arrowhead). (c) Photograph of the excised specimen shows a gelatinous, multilobular, papillary mass.
80% en válvulas (aórtica>mitral>tricúpide>pulmonar).
Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997:
Microscopic Features.—Papillary fibroelastomas are avascular papillomas lined by a single
671-682.
b.

18. Tumores Benignos
•
•
•
•
Clínica: embolización: EVC, AIT.
•
Fibroelastoma Papilar
Angina, IAM, IC, síncope,
síntomas pulmonares, muerte
súbita.
30% asintomáticos.
Apariencia de anémona de mar.
Tratamiento
•
•
Expectante.
Cirugía: ≥1 cm, embolización,
afectación valvular.
Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682;
Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No
4:2000:1073-1103.

19. RG ■ Volume 20 • Number 4
Tumores Benignos
RG ■ Volume 20 • Number 4
Grebenc et al 1083
Rabdomioma
•
•
•
Casi exclusivamente en niños <1 año.
80 - 90% relacionados con esclerosis
tuberosa.
a.
Figure 15. Rhabdomyoma in a 3-month-old boy with tach
shows diffuse heterogeneous nodular thickening of the left ve
rows). (b) Photograph of the cut autopsy specimen of the he
throughout the left ventricular myocardium (arrows).
b.
a.
Figure 15. Rhabdomyoma in a 3-month-old boy with tachycardia. (a) Coronal T1-weighted (370/25) MR image
shows diffuse heterogeneous nodular thickening of the left ventricular myocardium and interventricular septum (arrows). (b) Photograph of the cut autopsy specimen of the heart shows multiple, firm, white nodules distributed
throughout the left ventricular myocardium (arrows).
Localizados en paredes ventriculares o
válvulas.
•
La mayoría presentan regresión
espontánea.
•
Cirugía requerida en caso de síntomas.
Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J
671-682.
screening. Affected children may be detected in
utero because of nonimmune fetal hydrops and
fetal death. Other presenting features include
tachyarrhythmias, murmurs, and heart failure.
Because the majority of cardiac rhabdomyomas
regress spontaneously, surgery is not routinely required. However, patients with life-threatening
symptoms, usually those secondary to left ventricular outflow tract obstruction or refractory arrhythmias, respond well to surgical excision
(47,49–52).
Microscopic Features.—Rhabdomyomas are
composed of enlarged, vacuolated cells with
Figure 14. Rhabdomyoma, microscopic features.
sparse cytoplasm that resemble altered myocytes.
High-power photomicrograph (original magnification,
Cells stain stain) demonstrates typical vacuolated
strongly with periodic-acid
Cardiol. Volume 80, 1997: Schiff cells
300; H-E
stains due to theirCytoplasmic streaming is a“Spider
of rhabdomyoma. high glycogen content. typical
cells” are typical and are characterized by a cenartifact that results in “spider cells” (arrows).

20. Tumores Benignos
Fibroma
also endocrine neoplasms. Both the NAME (nevi,
atrial myxoma, neurofibromata, and ephelides) and the
LAMB (lentigines, atrial myxoma, and balloon nevi)
syndromes are associated with the familial variety of
cardiac myxoma. Chromosomal abnormalities for
atrial myxoma on chromosome 2 (Carney’s) and chromosome 12 (Kiras-genes) have been described.
Myxomas probably present the most varied clinical
picture of all primary cardiac neoplasms.6,8,9 Several
major syndromes have been observed including presentation with signs of emboli, obstruction to blood
flow, and various constitutional syndromes. Fragments of tumor located in the right side of the heart
may embolize to the lungs and those in the left side of
the heart, of course, to various systemic organs. Diagnosis may be made on occasion by finding typical
myxomatous endothelial-like cells which are elongated and spindle shaped with round or oval nuclei
and prominent nucleoli in operatively removed emboli. Obstruction to blood flow may occur at the
orifice of any valve, most commonly, of course, the
mitral valve. Interference with flow through the mitral
orifice may mimic signs of mitral stenosis, including
signs of pulmonary congestion, diastolic apical rumble, opening snap, and accentuated first heart sounds.
A murmur of mitral regurgitation may also be present
as a result of chronic damage to the valve leaflets or to
interference with proper closure of the valve by tumor.
Differentiation between left atrial tumor and primary
mitral stenosis is suggested by the influence of position on symptoms and on the intensity of the precordial murmurs and the opening snap.
The constitutional symptoms associated with atrial
myxomas are protean and include fever, weight loss,
Raynaud’s phenomenon, digital clubbing, anemia, elevated erythrocyte sedimentation rate, elevated leuko-
•
•
RG ■ Volume 20 • Number 4
FIGURE 4. Diagram of an intramyocardial fibroma seen at necropsy in a 5-month-old boy (A-07-92) who was found dead in
his crib. The neoplasm had been diagnosed by biopsy during the
child’s first few days of life. It was considered nonresectable.
mately 10% of all patients with myxomas and the
transmission is an autosomal dominant one. The familial myxomas occur in younger patients (mean age
25) than the nonfamilial ones and they have less
female gender predominance. The myxomas in the
familial syndrome are much more liable (50% of the
time) to be multiple and to have a ventricular cavity
location (13% as compared with about 2% in the
nonfamilial or sporadic patients with myxomas).
These patients typically have exterior facial freckling;
they have noncardiac myxomas (breast or skin) and
Raros. El segundo en frecuencia en
niños.
Músculo ventricular
(Izquierdo>Derecho).
•
Clínica: disfunción miocárdica, falla
cardiaca.
•
•
Cirugía: pacientes sintomáticos.
Trasplante.
Clifford-Roberts, W. Primary features. HighFigure 16. Fibroma, microscopic and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682;
Gerenc et al. Primary cardiac and pericardial
power photomicrograph (original magnification, 150; neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No
4:2000:1073-1103.
H-E stain) demonstrates dense collagen bundles in a

21. F
re
te
g
a
(
e
Tumores Benignos
Teratoma
•
•
•
a.
b.
Figure 22. Cardiac paraganglioma in a 13-year-old boy with a heart murmur and hypertension. (a) Axial T1weighted (400/20) MR image demonstrates a round, lobular mass of intermediate signal intensity arising from the
interatrial septum and protruding into both atria. (b) Axial proton density–weighted (2,000/20) MR image shows an
increase in tumor signal intensity. Intraoperative palpation of this paraganglioma resulted in immediate, severe hypertension and tachycardia.
Se originan dentro del
pericardio.
Therapy and Prognosis.—Surgical
Consecuencias mecánicasexcision of
cardiac paragangliomas is usually successful and
provides complete symptomatic relief (73,75).
importantes.
particular to cardiac paraganglioSurgical risks
mas include hypertensive crisis from intraoperative manipulation of the tumor and fatal hemorrhage due to the high vascularity of these lesions.
Cardiopulmonary bypass isolates the heart from
the systemic circulation and allows safe manipulation and dissection of the tumor. Because paragangliomas may be infiltrative lesions, extensive
resection of the atrial wall may be required for
complete excision (80,81).
Tratamiento: cirugía in utero o
Cesárea y cirugía inmediata
programadas.
c.
Microscopic Features.—Microscopically, cardiac teratomas are similar to benign extrapericardial teratomas. Typically, they contain derivatives of all three germ layers, with mature endodermal, mesodermal, and ectodermal elements
(Fig 23) (1). Tissues discovered in intrapericardial teratomas are diverse and include neuroglia, cartilage, skeletal muscle, liver, intestine,
pancreas, and glandular tissue.
Pathologic Features.—Pericardial teratomas
are usually right-sided masses, which typically
connect to one of the great vessels via a pedicle.
Clinical secondary neoplasms of teratoma is a
Clifford-Roberts, W. Primary and Features.—Pericardial the heart. Am J Cardiol. Figure 23. 1997: 671-682; microscopic features.
Volume 80, Mature
Most teratomas of theteratoma, within the periheart 20,
benign germ cell neoplasm that typically - Pathologic correlation. AFIP Archives. Vollie No
Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologicaffects
Low-power photomicrograph (original magnification,
cardial sac; rarely, they can be intramyocardial.
infants and children, who present with respira4:2000:1073-1103.
75; Masson trichrome stain) demonstrates neuroretinal
Teratoma
m
f
s
T
w
R
f
c
p
w
a
m
t
c
c
i
a
e
M
o
m
t
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22. Tumores Benignos
Hamartoma/células de Purkinje
•
•
•
•
•
•
Pequeños. Células aplanadas.
Usualmente en VI, superficies endocárdicas o epicárdicas.
Indetectables con técnicas radiológicas.
Niños. Taquicardia ventricular incesante.
EKG: BRDHH.
Estudios electrofisiológicos.
Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682;
Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No
4:2000:1073-1103.

23. Tumores Benignos
•
•
Adipocitos.
Lipoma
>50% surgen en la región
subendocárdica, el resto en válvulas.
•
b.
a.
b.
Figure 20. Intracardiac lipoma in a 45-year-old woman
with palpitations. (a, b) Axial (a) and coronal (b) proton
density–weighted (1,091/20) MR images demonstrate a
smooth, round, intracavitary right atrial mass with a signal
intensity characteristic of fat. (c) Photograph of the specimen demonstrates a well-circumscribed, spherical, yellow
mass that was excised from the right atrium.
Tamaño pequeño aunque pueden
llegar a pesar hasta 4.8 kg.
•
Clínica: arritmias, anomalías en la
conducción.
•
Diagnóstico por ecocardiografía o
RM.
•
Tto. Cirugía.
Radiologic Features.—The most frequent radiographic abnormality in patients with cardiac
lipoma is cardiomegaly (68,70,71). A globularshaped heart has also been described (69). Echocardiography typically demonstrates an echogenic,
nonmobile mass (68,70). CT and MR imaging
are useful for making a tissue-specific diagnosis
based on the findings of fat attenuation (Hounsfield measurement
50) and signal intensity
c. Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682;
c.
characteristics of fat (Fig 20). Lipomas appear as
Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No
predominantly homogeneous masses on CT
4:2000:1073-1103.
scans but may display internal soft-tissue septa or

24. Tumores Benignos
Potencialmente Malignos
• Paranglioma
•
•
Tumores neuroendócrinos. Hormonalmente activos o inactivos.
Clínica
•
•
•
•
Inactivos: compresión y tamponade.
Activos: producen NE: cefalea, diaforesis, taquicardia, HAS.
Usualmente en pericardio.
Secuestran el riego sanguíneo del corazón y son difíciles de
remover.
Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682;
Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No
4:2000:1073-1103.

25. Tumores Benignos
Potencialmente Malignos
RG ■ Volume 20 • Number 4
• Paranglioma
1090 July-August 2000
1090 July-August 2000
RG Volume 20 • • Number
RG ■■ Volume 20 Number 4 4
Figure 21. Paraganglioma, microscopic features.
High-power photomicrograph (original magnification,
300; H-E stain) demonstrates the classic nesting (zellballen) appearance of the paraganglial cells.
which are normally predominantly located within
the atria. Most lesions have been reported in
adult patients with an age range of 18–85 years
(mean age, 40 years) (73). The majority of reported cardiac paragangliomas have been catecholamine-producing tumors, and affected patients present with arterial hypertension, headache, palpitations and flushing, (symptoms typical
of pheochromocytoma) (73,74). The biochemical
laboratory abnormalities that lead to the diagnoa.
b.
sis of a paraganglioma include elevated levels of
a.
b.
Figure 22. Cardiac paraganglioma in a 13-year-old boy with heart murmur andAm J Cardiol. Axial T1- urinary norepinephrine, vanillylmandelic acid, and
Clifford-Roberts, paraganglioma in 13-year-old neoplasms of the heart. hypertension. (a) Axial T1Figure 22. Cardiac W. Primary andasecondaryboy with aaheart murmur and hypertension. (a) Volume 80, 1997: 671-682;
weighted (400/20) MR image demonstrates round, lobular mass ofRadiologic -signal intensitycorrelation. the
Gerenc et al. Primary cardiac and pericardial neoplasm: intermediate Pathologic arising from AFIP Archives. Vol elevated
weighted (400/20) MR image demonstrates aaround, lobular mass of intermediate signal intensity arising from the total metanephrine or20, No levels of plasma
interatrial septum and protruding into both atria. (b) Axial proton density–weighted (2,000/20) MR image shows
norepinephrine and epinephrine (74). Up to 20%
4:2000:1073-1103.
interatrial septum and protruding into both atria. (b) Axial proton density–weighted (2,000/20) MR image shows anan
increase in tumor signal intensity. Intraoperative palpation of this paraganglioma resulted in immediate, severe hy-of patients with cardiac paragangliomas have as-

26. Tumores Benignos Potencialmente Malignos
Mesotelioma
RG ■ Volume 20 • Number 4
Grebenc et al 1097
•
•
•
Grebenc et al 1097
when examined w
histochemical tech
tures include necr
morphism (1). Cy
dial fluid is freque
the difficulties in d
sothelioma cells fr
Pathologic Featu
mesotheliomas typ
pericardial masses
space and constric
masses are firm, w
though there may
epicardium, signif
rare (Fig 32) (1).
Figure 31. Mesothelioma, microscopic features.
High-power photomicrograph (original magnification,
400; H-E stain) demonstrates tubulopapillary structures (arrow) characteristic of epithelial mesothelioma.
Origen: pleura o pericardio.
Constricción.
survivals of 6 months to 1 year after diagnosis
(1,107).
Radiologic Featu
patients with peric
demonstrates card
pericardial effusion
or diffuse mediast
Chest CT demons
dial thickening and
(107,108). MR im
strates cardiac enc
cardial mass, as w
effusion (109).
Cualquier técnica de imagen y/o
pericardiocentesis.
Microscopic Features.—Malignant mesothelioma is typically a biphasic tumor composed of
epithelial areas, which resemble a carcinoma, and
a.
b.
spindled areas similar to a sarcoma. The epitheFigure 32. Pericardial mesothelioma in a 36-year-old woman with fatigue, malaise, and dyspnea. (a) Axial conlial areas form tubulopapillary structures (Fig 31).
trast-enhanced chest CT scan (mediastinal window) demonstrates a lobular, heterogeneous left pericardial mass
The spindled areas usually demonstrate some
with a large pericardial and bilateral pleural effusions. Note the large areas of low attenuationevidence oflikely repre(arrow), mesotheliomatous differentiation
•
Raro: origen en nodo AV con
bloqueo.
when examined with ultrastructural or immuno-
senting necrosis, within the tumor. (b) Photograph of the cut specimen of the heart obtained at autopsy shows a diffuse nodular pericardial mass that encases the heart. Scale is in centimeters.
histochemical techniques. Other microscopic features include necrosis and extensive cellular pleomorphism (1). Cytologic examination of pericardial fluid is frequently nondiagnostic because of
the difficulties in differentiating malignant mesothelioma cells from reactive cells (107,108).
b.
Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682;
r-old woman with fatigue, malaise, and dyspnea. (a) Axial conGerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No
Pathologic Features.—Malignant pericardial
w) demonstrates a lobular, heterogeneous left pericardial mass
4:2000:1073-1103.
mesotheliomas typically form multiple coalescing
ns. Note the large areas of low attenuation (arrow), likely repre-
•
Tratamiento: resección + Qx y/o Rx.

27. Tumores Malignos
• Constituyen el 15% de los
tumores cardiacos.
• Sarcoma
•
•
a.
b.
Figure 27. Unclassified sarcoma in a 29-year-old man with cough,
transplantation. (a) Coronal T1-weighted (714/12) MR image shows
tensity involving the left side of the heart. (b) Photograph of the excis
left atrial wall and mitral valve.
a.
b.
Figure 28. Angiosarcoma in a 28-year-old woman who presented with mitral valve obstruction. (a) Axial contrastenhanced chest CT scan (mediastinal window) shows a large nodular, homogeneous, soft-tissue mass that invades
the right atrium and encases the heart. (b) Coronal T2-weighted (3,780/57) MR image demonstrates extensive
circumferential cardiac involvement by the nodular, heterogeneous, hyperintense tumor, which invades the right
atrium and encases the heart.
Los más frecuentes.
Angiosarcomas: células malignas que
Radiologic Features.—The most common raenhancing areas typically corresponding to necrodiographic abnormality in patients with
forman canales is cardiomegaly. Other findingscardiac
vasculares.
sarcoma
include
sis (91,97,98).
MR imaging typically demonstrates cardiac
heart failure, pleural effusion, focal cardiac mass,
angiosarcomas as large, heterogeneous, invasive
pulmonary consolidation, and pericardial effuright atrial masses, frequently with extensive perision (2,7,91,94–96). Because of their location,
cardial involvement and hemorrhagic pericardial
the radiographic findings of left atrial sarcomas
effusion. Signal intensity in these tumors is hetcan mimic those of left atrial myxoma.
erogeneous, intermediate on T1-weighted images
CT is helpful in the evaluation of cardiac sarwith higher signal intensity on T2-weighted imFigure 26. Angiosarcoma, microscopic features.
comas as it demonstrates the broad-based tumor
ages (Fig 28). A case report describes the MR
High-power photomicrograph (original magnification,
450; H-E stain) demonstrates irregular anastomosing
attachment; myocardial, pericardial, and mediasimaging appearance of a polypoid right atrial
Clifford-Roberts, W. Primary and secondary well as extension into the greatCardiol.mass with extensive671-682; endothelial cells.and
neoplasms of the heart. Am J
Volumechannels linedpericardial involvement
vascular 80, 1997: by atypical
tinal invasion; as
Gerenc et al. Primary cardiac andand pulmonary metastases, when present
vessels pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No
reveals that after intravenous gadolinium admin4:2000:1073-1103.
(11,12). Angiosarcomas have been described as
istration, the pericardial tumor demonstrated
•
Predominan en VD.
mor at
these s
myxom
Micros
ance of
tually a
sues ha
most c
(Fig 26
(includ
coma)
stroma
nign m
ated sa
tural, o
Pathol
of card
majorit
masses
endoca
large, i

28. Tumores Malignos
Sarcomas
•
•
•
Rabdomiosarcoma: 20%. Sin localización específica.
Fibrosarcoma: tumores de “carne blanca”.
•
•
Compuestos de células gigantes fusiformes.
Áreas extensas de necrosis y hemorragia.
Leiomiosarcoma: células gigantes fusiformes.
•
•
Aurícula derecha.
Metastatizan y recurren frecuentemente.
Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682;
Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No
4:2000:1073-1103.

29. Tumores Malignos
Sarcomas
•
Tratamiento
•
•
•
•
•
•
Cirugía.
Mortalidad secundaria a recurrencia.
Supervivencia media: 6 meses a 1 año.
Quimioterapia y/o Radioterapia.
Trasplante cardiaco.
Autotrasplante cardiaco.
Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682;
Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No
4:2000:1073-1103.

30. qu
be
ent
im
Th
60
yea
po
Tumores Malignos
Linfomas
•
•
Proveniente de miocardio.
Pronóstico pobre.
b.
gure 30. Primary cardiac lymphoma in a 75-year-old woman with
ogressive dyspnea, superior vena cava syndrome, and atrial fibrillan. (a) Coronal T1-weighted (571/12) MR image shows vena caval
vasion (arrow) by a mass. (b) Superior vena cavogram demonstrates
e large intraluminal tumor that obstructs the vena cava. Note the coleral blood flow through the azygos and hemiazygos veins. (c) Photoaph of the specimen of the heart obtained at autopsy shows a firm,
hite, multinodular right atrial tumor with plaquelike pericardial infiltion (arrow) and obstruction of the superior vena cava ( ).
1096 July-August 2000
RG ■ Volume 20 • Number 4
Figure 29. Lymphoma, microscopic features. Highpower photomicrograph (original magnification, 300;
H-E stain) demonstrates lymphoma cells (dark blue
round cells) surrounding and infiltrating residual myocytes (arrow).
(2,40). Aggressive surgery offers significant palliation of symptoms (caused by valvular and vascular obstruction) and improves survival (89,
92,94). Even after complete tumor excision,
however, local recurrence and metastatic disease
occur frequently and early, usually within 1 year
(2,90,94). Heart transplantation has been performed in some patients with unresectable cardiac sarcoma with satisfactory results (92,95,
100). Chemotherapy and radiation therapy have
not proved beneficial for the treatment of affected patients (92). Death in these patients usually results from postoperative complications,
cardiopulmonary failure from progressive tumor
growth, and metastatic disease (2).
adiologic Features.—Chest radiography of
tients with primary cardiac lymphoma usually
monstrates cardiomegaly, pericardial effusion,
d signs of heart failure. Echocardiography typilly demonstrates hypoechoic myocardial masses
the right atrium or ventricle with an associated
a.
b.
Primary Cardiac Lymphoma
ricardial effusion (102–104,106). At CT, carFigure 30. Primary cardiac lymphoma in a 75-year-old woman with
c.
Clifford-Roberts, W. Primary and secondary neoplasms
ac lymphomas are hypoattenuatingsyndrome,neoplasm:of the heart.-Am J Cardiol. Volume 80, 1997: 671-682; 20, No
or iso- and atrial fibrillaprogressive dyspnea, superior vena cava
Gerenc et al. Primary cardiac and pericardial
Radiologic Pathologic correlation.Clinical Features.—Primary cardiac lymphoAFIP Archives. Vol
mas are typically of the non-Hodgkin type. By
tion. (a)relative T1-weighted (571/12) MRand dem- vena caval
Coronal to the myocardium image shows
enuating
4:2000:1073-1103.
definition, these tumors involve only the heart or
pro
car
dro
the
dia
ma
ear
ma
pai
tha
deb
du
Mi
dia
Th
typ
ph
lar
Bu
ph
epi
the
Pa
ma
ma
scr
pea
Th
lym
rig
sep
car
alt
diu
tig

31. Myxo
Rhabdomyo
Synovial
Lipo
Schwannoma
Lymphoma
8
6
4
2
1
7 (5%)
8
2
4
0
1
1
0
4
0
2
0
6
1
3
0
0
0
0
Breast
Mouth & tongue
Thyroid gland
Uterus
Urinary bladder
Stomach
Colon & rectum
Prostate gland
Pancreas
Nose (interior)
Ovary
Pharynx
Miscellaneous
100
100
100
100
100
100
100
100
100
100
100
100
100
Neoplasias secundarias
Totals
144 (100%) 117 (81%) 27 (19%)
11 (8%)
*Modified from: Burke A, Virmani R. Atlas of Tumor Pathology. Tumors of the
Heart and Great Vessels. Washington, DC: Armed Forces Institute of Pathology 1996:231.
•
•
•
•
Metástasis.
•
Clínica: síntomas CV en pacientes ya
conocidos con cáncer.
•
Descartar toxicidad por quimioterapia.
TABLE III Metastatic Neoplasms in the Heart at Necropsy—
Order of Frequency of Cancers Encountered*
Primary Tumor
Total Autopsies
8% de todos los cánceres.
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
21.
22.
23.
Lung
Breast
Lymphoma
Leukemia
Esophagus
Uterus
Melanoma
Stomach
Sarcoma
Oral cavity & tongue
Colon & rectum
Kidney
Thyroid gland
Larynx
Germ cell
Urinary bladder
Liver & biliary tract
Prostate gland
Pancreas
Ovary
Nose (interior)
Pharynx
Miscellaneous
10%
9%
9%
8%
6%
5%
5%
4%
3%
3%
1%
1%
0
*These percentages were obtained by combining studies by McAllister and
Fenoglio and by Mukai et al. (see Table III).
Metastases to Heart
1,037
685
392
202
294
451
69
603
159
235
440
114
97
100
21
128
325
171
185
188
32
67
245
180
70
67
66
37
36
32
28
24
22
22
12
9
9
8
8
7
6
6
2
1
1
0
6,240
653 (10%)
Vía hematógena o invasión directa.
(17%)
(10%)
(17%)
(33%)
(13%)
(8%)
(46%)
(5%)
(15%)
(9%)
(5%)
(11%)
(9%)
(9%)
(38%)
(6%)
(2%)
(4%)
(3%)
(1%)
(3%)
(1%)
Melanoma, pulmón, mama, sarcomas,
riñón, esófago, hígado, tiroides, leucemia y
linfoma.
*Modified from: Burke and Virmani (who combined studies of McAllister HA
and Fenoglio JJ Jr). Tumors of the Cardiovascular System Atlas of Tumor
Pathology. Washington, DC: Armed Forces Institute of Pathology 1978: 111–
119; and Mukai K, Shinkai T, Tominaga K, Shomosato Y. The incidence of
secondary tumors of the heart and pericardium: a 10-year study. Jpn N Clin
Oncol 1988;18:195–201.
FIGURE 1. Diagram showing various locations of cardiac neoplasms involving the heart. LA 5 left atrium; LV 5 left ventricle;
RA 5 right atrium; RV 5 right ventricle.
in the right atrial cavity (Figure 3), and about 2% in a
ventricular cavity.4 –16 On rare occasion,
Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: the tumor is
present in more than 1 cavity.17 Generally, the neo671-682.
plasm when located in the left atrium produces symp-