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T H E AMERICAN JOURNAL OF CLINICAL PATHOLOGY
Vol. 39, No. 4, pp. 374-382
April, 1963
Copyright © 1963 by The Williams & Wilkins Co.
Printed in U.S.A.
INTRAMURAL FIBROMA OF THE HEART
JAMES A. FREEMAN, M.D., JACK C. GEEE, M.D., W. S. RANDALL, JK., M.D., AND
WILLIAM G. PALFREY, M.D.
Department of Pathology, Louisiana State University School of Medicine, New Orleans, Louisiana,
and the Departments of Pathology and Pediatrics, Baton Rouge General Hospital, Baton Rouge,
Louisiana
Because of the rarity of primary cardiac
tumors in general, and fibromas in particular, recording their occurrence is desirable
in order to assist in early clinical recognition
and possible surgical treatment of future
cases. Documentation is also necessary to
the study of the biologic behavior of these
tumors. The interventricular cardiac fibromas have histologic features and behavioral characteristics similar to those of
the fibromatoses in general. With this in
mind, a case is added to the short list of
interventricular fibromas (Table 1), and an
attempt is made to relate these fibromas to
the general group of fibromatoses.
R E P O R T OF CASE
Clinical History
The patient was a Caucasian female born
in December 1960 in Baton Rouge, Louisiana. The birth weight was 7 lb. V/i oz.
No perinatal difficulties were encountered.
The infant was blood type B, Rh-positive,
Coombs' test negative. With the exception
of a milk allergy, growth and development
were within normal limits. A complete
physical examination performed March 20,
1962, during an office visit to a pediatrician
for conjunctivitis, was normal. No murmurs
were heard, and the heart rate and rhythm
were normal. On May 8, 1962, the child's
mother noted she was sleeping late and
tried to arouse her without success. Upon
arrival of a pediatrician she was pronounced
dead.
Gross Necropsy Report
The body was that of a normally developed, fairly well nourished, 17-month-old
Caucasian female. With the exception of
the lungs, heart, and liver, there were no
lesions. The liver was moderately congested,
and the lungs were severely congested. Approximately 100 ml. of clear fluid were in
the pericardial sac. The heart was twice the
normal size, and a gray-yellow, lobulated,
soft mass occupied practically all of the
musculature of the anterior left ventricle
and extended into the interventricular septum. There was encroachment of the tumor
upon both ventricular chambers. No endocardial or atrial involvement was found.
There were no valvular defects. The heart
weighed 170 Gm. (wet weight, after 10 per
cent formalin fixation; normal dry heart
weight for age and sex is 48 to 52 Gm.). The
tumor mass measured 8 by 8 by 5% cm.
Received, July 13, 1962; revision received,
August 29; accepted for publication December 5.
Dr. Freeman is Instructor of Pathology, and
Dr. Geer is Associate Professor of Pathology,
Louisiana State University School of Medicine.
Dr. Randall is Pathologist, Baton Rouge General
Hospital, and Clinical Assistant Professor,
Louisiana State University School of Medicine.
Dr. Palfrey is Visiting Pediatrician, Baton Rouge
General Hospital.
This study was supported in part by grants
from the Louisiana Heart Association, the New
Orleans Cancer Association, and the National
Institutes of Health (H-2549).
374
Microscopic Features of Tumor
The tumor was an unencapsulated but
well delineated mass of abundant, dense,
fibrous connective tissue bands interlaced
with bundles of collagen and some elastic
fibrils (Figs. 2 to 5). An interstitial proliferative infiltration of fibrous tissue was evident
in the muscle; it enclosed muscle fibers
(Fig. 4). No "primitive" cardiac muscle
cells were recognized. Groups of young fibroblasts were seen in selected areas. Numerous small capillaries were present (Fig.
4). Masson's trichrome stain revealed the
bundles of collagen, some spiralling through
the mass (Figs. 3 and 4). The characteristic
unit fibrils of collagen with the 640 A pe-
April 1968
INTRAMURAL CARDIAC FIBROMA
375
FIG. 1. Gross photograph of the heart
riodicity were demonstrable by means of
electron microscopy (Fig. 6). The PTAH
stain and trichrome stain demonstrated the
cardiac muscle fibers incarcerated in the
infiltrating fibrous tissue (Fig. 4). There was
a small area of dystrophic calcification. No
prominent xanthomatous element was present. Mitoses, anaplasia, and giant cells were
not present.
DISCUSSION 7
Occurrence and Incidence
Although primary tumors of the heart
are rare, Prichard20 (1951) and Bigelow and
associates2 (1954) have demonstrated that
they are more frequent than is generally
believed. Whereas metastatic tumors represent approximately 3.9 per cent (there
have been 326 metastases in a total of 8414
cases in published reviews) of cardiac tumors (Jernstrom and Cremin," 1959), primary tumors represent approximately 0.15
per cent of 4.80,000 autopsies, i.e., 716 instances of tumor (Straus and Merliss,24
1945). To date, there have been approximately 27 published cases of intramural
fibroma (or fibrous hamartoma) of the heart
(Table 1), excluding those occurring in the
atrium. Although Mahaim 16 (1954) attributes the first recognition of a cardiac fibroma
to Colombo in 1559, the first documented
bona fide case is that of Luschka14 (1885).
Author
Unknown
Sudden death
Cardiomegaly;
harsh m u r m u r
Unknown
None
Left ventricle
Left ventricle
Left ventricle
Left ventricle
Left ventricle
F
F
F
F
F
1 yr.
8 mo.
3 da.
2yr.
9 mo.
23
1940 Vukan
13
1949 Kulka
1954 Bigelow and associates 2
9
1955 Froboese
19
1955 Naeve
cleft
Unknown
None
Diphtheria;
polio
Glomerulonephritis ( ± a m y loidosis)
Unknown
None
None
Unknown
Harelip,
palate
None
None
Sudden d e a t h
Sudden d e a t h
Unknown
Left ventricle
Interventricular
None
septum
Newborn A recapitulation of Mono keberg's case
15 mo.
M Left ventricle (pos- Sudden d e a t h (in
hospital)
terior wall)
Uremia
53 yr.
M Left ventricle
M
M
F
M
None
None
Unknown
Diphtheria
None
Clinical Diagnosis
Sudden d e a t h
Murmur
Unknown
None
Sudden d e a t h
Presenting or Cardiac
Symptoms, or Both
65 yr.
9 mo.
2yr.
3 mo.
Left ventricle
Interventricular
septum
Interventricular
septum and left
ventricle
Interventricular
septum
Left ventricle
Left ventricle
Location of Tumor
1937 Macherey 1 6
1937 Fidler and associates'
1938 Symeonidis and
Linzbaeh 2 6
1927 T e u s c h e r "
4
1930 Brown and Gray
Newborn M
1924 Monckeberg 1 8
F
M
F
Sex
30 yr.
6 yr.
3 mo.
Age
Patient
1880 Zander 3 1
1559 Colombo
14
1855 Luschka
1871 Wagstaffe 30
Year
TABLE 1
Fibroma
Fibroelastic
hamartoma
Fibroma
Fibroma
Fibroma
Incidental
Hamartoma
finding
Incidental finding
(Plastic) surgical d e a t h ;
incidental finding
Description and p h o t o s
compatible
with
fibroma
(No photos in a r t i c l e ) ;
died in hospital
(Cited by M a h a i m )
Incidental finding
Remarks
Hamartoma
Fibroma
Fibrosarcoma
Fibroma
Rhabdomyoma
Fibroma
Fibroma
Fibroma
Fibroma
Fibroma
Pathologic Diagnosis
R E S U M E O F C A S E S O F INTRAMURAL V E N T R I C U L A R CARDIAC F I B R O M A S
CQ
••a
%
CO
-
^
«
Left ventricle
M
4yr.
VA yr.
M
M
F
51 yr.
42 hr.
17 mo.
1960 Boyette and
Foushee 3
1962 Freeman and
Spurlock 8
F
M
1960 Svejda and
Tomasek 2 5
8 mo.
Left ventricle
F
43 yr.
1958 Aiiriol and associates 1
1958 Krueger and
Knoll 1 2
and
1959 Jernstrom
Cremin 1 1
1960 Valledor and associates 2 8
Left ventricle
F
19 mo.
*
Interventricular
septum and left
ventricle
Interventricular
septum
Interventricular
septum and left
ventricle
Interventricular
septum
Left ventricle
Left ventricle
1957 Edlund and Holmdahl 6
M
7 mo.
Right ventricle
Left ventricle
Left ventricle
»
1956 Conlon 5
F
4 yr.
F
F
,
47 mo.
»
5 mo.
"
1956 Radnai 2 1
1955 McCue and associates"
1955 James and Stanfield10
<
,
Sudden death
Air hunger
Angina-like pain
Congestive failu r e ; syncope
Congestive
failure
Sudden death
None
Sudden death (in
hospital)
M u r m u r (age 3
mo.)
Unknown
M u r m u r ; cardiomegaly
Tachycardia
»
»
»
None
None
Ventricular
aneurysm
Fibroelastosis
Subaortic stenosis
None
Leptomeningitis
Diarrhea
and
emesis
Cardiac t u m o r
None
Subaortic stenosis
Fibroelastosis
"
^
Fibroma
Fibroma
Fibroma
Fibroma
Fibroma
Fibroma
Fibroma
Fibroma
Fibroelastic
hamartoma
Mesenchymoma
Fibroma
Fibroma
'
r
T
fibroma
Subject of present report
E K G : paroxysmal auricular t a c h y c a r d i a , r i g h t
bundle branch block
Died
during
surgery;
E K G : left ventricular
h y p e r t r o p h y ; strain
E K G : old infarct; surgical diagnosis; died postoperative day 3
Gross diagnosis a t surgeo'.
EKG:
bundle
branch block ±
left
ventricular hypertrophy
Incidental finding
Histologically
E K G : left
ventricular
hypertrophy, strain
EKG:
sinoventricular
versus ventricular
tachycardia
T
378
F R E E M A N ET
AL.
Vol. 39
FIG. 2. Gross photograph of a horizontal section of the heart, illustrating the relative size of the interventricular fibroma and the ventricular walls. The lacelike bundles of fibrous connective tissue are evident
grossly. There is a remarkable gross resemblance to uterine leiomyomata.
Of these 27 cases, 21 have occurred in children (78 per cent). No sex predisposition is
noted; 12 of the 27 cases were in males. Two
cases have been recorded as the cause of
death in a newborn (Monckeberg,18 1924;
Boyette and Foushee,3 1960), and 15 cases
in infancy (Table 1). In 9 instances (including the present case), the tumor has
been invoked as the cause of sudden death.
Only 2 cases have been diagnosed premortem, both at operative surgery (Edlund
and Holmdahl, 0 1957; Svejda and Tomasek,25 1960). Only 1 case in the childhood
group was an incidental finding (Auriol and
colleagues,1 1958).
Most intramural fibromas are located in
the interventricular septum or anterior part
of the left ventricular wall, only 1 being
recorded on the posterior left ventricular
wall (Symeonidis and Linzbach,26 1938) and
1 infiltrating the right ventricle (Radnai,21
1956).
Terminology and Pathogenesis
Fibromas and fibrous hamartomas are
grouped together for the purposes of this
report. Only interventricular fibromas are
included, inasmuch as fibromas of the atrium
and heart valves may be related to myxomas, or they have been suspected of being
nonneoplastic growths of inflammatory
origin or Lambl's excrescences (Prichard,20
1951). Other terms have been applied to
these infiltrating fibrous growths, such as
fibroelastic hamartoma and desmoid.
Considerable controversy has arisen regarding the pathogenesis of primary benign
cardiac fibromas. Indeed, some authors will
not accept fibroma as a true diagnosis, but
group the lesions instead with hamartomas
in general (Prichard, 20 1951). Conlon5 (1956)
described a fibrous intramural growth as an
embryonic mesenchymal tumor. Inasmuch
as fibromas develop from mesenchyme, with
an exaggeration of the fibrous element, and
Conlon's report resembles the case herein
presented, his case is included in the table.
The designation of these tumors as embryonic mesenchymal tumors adds nothing to
an understanding of the group to which
they belong, inasmuch as all cardiac tumors
may be said to arise from mesenchyme. The
Fio. 3. Microscopic section, with the well demarcated, but unencapsulated fibrous mass. Unaffected cardiac muscle is at the top of the micrograph. Paraffin embedding. Masson's triclirome. X 240.
Fio. 4. The proliferating, interstitial infiltration of fibrous connective tissue is incarcerating muscle
fibers (M). Capillaries are prominent. Paraffin embedding. Masson's triclirome. X 240.
379
380
FREEMAN ET
Vol. 39
AL.
FIG. 5. Bands of collagen (C) and fibrous connective tissue infiltrating mature cardiac tissue. Maraglas epoxy embedding. (Freeman and Spurlock,81962.) Toluidine blue. X 500.
predominant element of these tumors is
fibrous tissue with abundant collagen and
young fibroblasts. Some, but little, elastic
tissue component is present. Therefore, although it is perhaps semantically correct to
call these tumors fibroelastic hamartomas,
this designation serves no useful purpose.
Hence, the present authors prefer to call
these tumors fibromas.
Symptomatology and Manifestations
As previously mentioned, these growths
may produce sudden death (9 of 27 cases)
without giving rise to previous symptoms.
Although the tumors are histologically benign, they are liable, by virtue of then- location, to interfere with the conduction
system and, therefore, to cause death. They
may, however, induce a variety of clinical
manifestations, the most notable of which
are bizarre electrocardiographic tracings.
Two of the 27 cases manifested the findings
of subaortic stenosis, whereas 2 other cases
were suggestive of fibroelastosis (in children).
Only 2 were diagnosed pre-mortem, both
during cardiac surgery, and a cardiac tumor
was suspected clinically in 1 case, whereas a
ventricular aneurysm was suspected in the
other.
Relation to Fibromatosis
Fibrous growths of other parts of the body
(desmoid fibromatoses, Dupuytren's palmar
fibromata, juvenile aponeurotic fibroma, the
well differentiated fibroma-sarcomas, keloids, and others) are not uncommon (Stout,
22,23
1953 and 1954). The common characteristic of many of these growths is the
unencapsulated, but well circumscribed, infiltrating, proliferating, dense, fibrous connective tissue pattern. These fibromatoses
are regarded as benign, and metastasis is
rare. Inasmuch as behavioral characteristics
differ with the various diagnoses, their subclassification is well justified; however, they
have all been interrelated as the fibromatoses
April 1963
I N T R A M U R A L CARDIAC
381
FIBROMA
SUMMARFO I N 1NTERLINGUA
F I G . 6. An electron micrograph illustrating t h e G'10 A periodic banding characteristic of mature collagen. Maraglas epoxy
embedding. Unstained. X 16,000.
(Stout, 22,2:i ). Inasmuch as the intramural
fibroma of the heart has the same benign,
diffuse, interstitially proliferating, unencapsulated, but well circumscribed, fibrous
growth pattern, it is suggested that this
tumor should be in the same category and
thus related to the desmoid fibromatosis
and the other fibromatoses. Despite the benign character of the fibromatoses, especially
the intramural cardiac fibroma, the anatomic
site of occurrence precludes longevity.
Iste articulo describe un fibroma interventricular que causava le morte subitanee
de un previemente normal feminina de racia
blanc de 17 menses de etate.
Le litteratura pertinente a fibromas
cardiac (hamartoma fibrose, desmoide, hamartoma fibroelastic) es revistate. Ex le 27
exemplos trovate, 21 occurreva in juveniles,
e 2 esseva registrate como causa de morte in
neomatos. II non existe un apparente predisposition sexual. In 9 casos, le tumor
esseva incriminate como le causa de morte.
Histologicamente, le tumores es proliferationes benigne de fibrose tissu conjunctive e non es incapsulate. Illos es simile
in characteristicas histologic e in lor crescentia a fibromatoses in general.
Acknowledgments. We express our appreciation
to M r . Ben 0 . Spurlock, who did the fine sectioning
for electron microscopy, and to Mr. Eugene Wolfe
for t h e photography.
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