489
CLINICAL NOTES
MYXOFIBROMA
OF
THE
LEFT
ATRIUM
SIMULATING
RHEUMATIC
HEART
DISEASE
By Pilar A. Centeno, M.D.*
T
HE RELATIVELY high incidence ofcon-genital heart disease, rheumatic fever and bacterial endocarditis in children
re-quires the pediatrician to excludeS these
diagnostic possibilities before considering an unusual condition. In the past 100 years, the incidence of heart tumors in post-mor-tem examinations has varied from 0.05 per
cent
(
11 in 1,888 necropsies) according to Ravid and Sachsl to 1.05 per cent (118 in11,100 necropsies) as observed by Scott
and Garvin.2 The histopathologic and
clini-cal study of Lymburner3 showed a rate of
0.61 per cent (52 in 8,500 necropsies). Scott and Garvin encountered 118 heart tumors
among 1,082 tumor cases, an incidence of
10.9 per cent. Except for 29 cases47 diag-nosed before death, of wilich 20 were due
to metastatic carcinoma, 5 to metastatic sar-coma and 4 to myxoma (1953), heart tumors
have been post-mortem curiosities. The
di-agnostic difficulties are illustrated by tile
following case of myxofibroma of tile left
atrium.
History
REPORT OF A CASE
A 3 3/12-year-old Caucasian boy was ad-mitted to St. Mary’s Hospital, San Francisco,
011 April 28, 1952, with anorexia, weakness, easy fatigabibity and pain ill both legs of 1
week’s duration. One week previously there
had been an acute respiratory infection.
The past history revealed that the child was
born normally at term on January 28, 1949.
The birth weight was 3.7 kg. Neonatal and
subsequent stages of growth and development were within normal limits. The only ibbnesses
* ADDRESS: Hospital for Tumors and Allied
Dis-eases, City of Hope Medical Center, Duarte, Cali-fornia.
were chicken-pox 1t age 3 months and
naso-pharyngitis at 13 years.
He had been admitted to Children’s Hos-pitab 3 months previously because of a sudden onset of lethargy, neck and back stiffness, vomiting and a skin eruption. A clinical diag-nosis of meningococcic meningitis was made
and confirmed bacteriobogically. Penicillin and suifadiazine were administered but gross hematuria developed after 24 hours and
Ter-ramycin was substituted for the sulfadiazine.
He was discharged after 5 days. The cardiac
examination at that time was reported to be
normal.
He was examined 2 months later because of easy fatigability and pallor, and a moderate
anemia was found. The heart was again
re-ported to be normal.
Ten days after a bout of tonsillitis, the child was tired, pale, listless and occasionally slightly
cvailotic. Physical examination revealed a soft
systolic mitral murmur. Cardiac enlargement and a few moist basilar rales were present. Roentgenogram of the chest showed moderate cardiac enlargement, a prominent pulmonary
artery and changes consistent with considera-ble pulmonary vascular congestion, particularly
in the medial third of both lung fields. The tentative diagnosis was congenital heart dis-ease with an arteriovenous shunt, either intra-or extracardiac, and pulmonary congestion.
These findings led to his admission to the hos-pital on April 28, 1952.
Physical and Laboratory Findings
On the day of admission the physical exami-nation revealed injection of the throat together with the cardiac and pulmonary findings pre-viously described. The ervthrocyte count was
3,750,000/mm.3 and the hemoglobin was 10
tricular preponderance and a lowering of the
T-waves as compared with a previous record.
Course
Four days after admission a gallop rhythm was noted. Acquired heart disease, possibly
rheumatic myocarditis, was considered, and
ACTH was administered in dosage of 20 mg. every 6 hours for 11 days, then gradually dim-inished until, on the twenty-third day, the dose was 20 mg. of ACTH gel. This dosage was maintained for 12 days. The gallop rhythm, the pallor and the episodes of slight cyanosis
disappeared and the child was discharged 27
days following admission.
He was again admitted on July 20, 1952,
2 weeks after an acute respiratory disease with
fever (39.5#{176}C.), cough and difficulty in breath-ing. The respiratory symptoms were not re-bieved by antibiotics and for the first time it was noticed that the patient preferred to lie prone with a pillow under the chest, and the head below the level of the thorax. He was pale and appeared “puffy” but not cyanotic. The heart was further enlarged, particularly to the right with a diffuse cardiac impulse and marked precordial and epigastric pulsations. A rather rough widespread hobosystolic cardiac
murmur was present as well as moist rales
at the right base, a palpable liver and slight peripheral edema. The erythrocyte count was 2,860,000/mm.3 and the hemoglobin 8.5 gm. (59 per cent) . The beukocyte count was 34,300/
mm.3 with a marked shift to the left. The
sedimentation rate was normal and a blood cub-ture was negative. Chest roentgenograms July 21 and 25 showed more marked bronchovascu-bar markings than that taken on April 28. The
electrocardiogram showed a greater degree of right ventricular hypertrophy than before and the P-waves suggested auricular hypertrophy. Conduction time was normal. Blood
transfu-sions and ACTH were administered. He
be-came more dyspneic and irritable, the heart
sounds became squeaking in character and
there was increasing pulmonary congestion (Fig. la) and cyanosis. Fever abated only dur-ing the administration of ACTH. Digitalization
and oxygen therapy afforded temporary
im-provement (Fig. ib) and the patient was dis-charged. Four days later he re-entered the hos-pital in extremis with severe precordial pain, dyspnea and vomiting. Marked cardiac
enlarge-out both lungs. He remained afebrile and the
anemia and leukocytosis were unchanged.
Oxygen therapy and other supportive measures
were of no avail, and he expired 5 hours
later.
Necropsy Findings
The heart weighed 180 gm. and was dilated, extending to the left lateral chest wall. The epicardiab surface and coronary arteries showed no abnormalities. There were no thrombi in the chambers and the foramen ovale was closed. The valves were normal. The dilated left atrium contained a large polypoid tumor, light grey-pink in color and of soft gelatinous consistency, measuring 5.5 cm. X 3.5 cm. X 2.5 cm. (Fig. 2). Its external surface was smooth with numer-ous mammiblations or nodular protuberances re-sembbing a grape cluster. It was attached by a narrow pedicle, 1.5 cm. in diameter, to the postero-superior part of the interatrial septum close to the postero-superior margin of the fossa ovalis near the entrance of the right pulmonary vein. The tumor was free otherwise and with-out clots or vegetations. It filled the left atrium and protruded through a distended mitral valve opening into the left ventricle. The detached
tumor weighed 40 gm. Its cut surface was
smooth, soft, gelatinous and appeared grey-pink in color. The endocardial lining of the left atrium was white, opaque and slightly thick-ened.
Histologic examination of the pobypoid mass showed a poorly cellular myxomatous tissue. The cells were sparse and were variously round, elongate or stelbate in shape with an indeter-minate cytoplasm that blended into the inter-cellular material which was somewhat degen-erated. Few capillaries traversed the tumor but small blood vessels were more numerous in the pedicle. There was slight lymphocytic
infiltration in the pedicle and the tumor tissue in the pedicle blended with the thickened endocardium. A layer of endothelial cells was found reflected from the endocardium over the tumor.
in-COMMENT
When a primary tumor is known to exist
elsewhere and the patient develops
unex-pected signs of heart involvement, e.g.,
arrhythmias, conduction defects or cardiac failure, it is logical to suspect a heart
tumor.#{176}8’9 On the other hand, the rarity of
CLINICAL NOTES 491
Fio. 1. a (Upper).
Roentgenogram of the
chest in posteroanterior projection taken July 28, 1952, while the child was receiving ACTU. b (Low-er). Roentgenogram of the chest taken on August 11, 1952, when the clinical (condition of the patient had improved following digitalization.
farction. The major bronchi and vessels were not remarkable. The alveoli contained an abun-dance of brown pigmented phagocytic cells and
precipitated edema fluid, but there was no
in-flammatory exudate. The capillaries of the
alveolar walls were engorged.
The abdominal viscera showed acute passive
Fic. 2. The opened heart showing the left auricle and ventricle. The tumor mass is shown arising from
the septal wall of the left auricle and protruding through tile mitral ring into the left ventricle.
I)ri1ll1ry ileart tumor seldom encourages a
ante-mortem diagnosis.
The keen reasolling of Pavbowsky, to whom Gottel1#{176} gave credit, led to the first clinical recognition of a primary tumor of the heart. The diagnosis was based on the oI)servation tilat the patient preseiited signs and symptoms of mitral stenosis when sitting or standing, but not when lying down. In 1934, 2 reports on primary heart
tumor correctly diagnosed before death
appeared for the first time in the United States. Barnes, Beaver and Snellhl reported
a primary sarcoma of the heart with
elec-trocardiographic and pathological studies. Shebburnel encountered an unexplained
serosanguinous, rapidly accumulating
pen-cardial effusion together with signs of acute cardiac decompensation in a young
individual and made the diagnosis of
pri-mary heart tumor before deatil in the same
year.
The patients preferred prone position,
with his head lower than his trunk, together with the presence of heart failure, were
diagnostic clues in tiliS case. Summarizing tile clinical findings of 30 cases of endo-cardial niyxoma situated in tile left atrium, Von Reis noted that Adams-Stokes attacks
appeared to be an invariable feature. The
attacks seemed more frequent when tile
polyp il11 attained such a size tilat it
telTiporanily obstructed tile mitral orifice.
When tile patients ilead was lower than
the thorax, the polyp slid back from the mitral opening and upon re-estai)lishnlent of circulation the cerebral anemia was al-leviated. The improbability of a heart tumor discouraged more extensive
diag-nostic studies.
In an extensive review of the literature
Pnichardit collected approximately 920
cases of tumors of the heart. He found more than 500 instances of cardiac
metas-tases and approximately 420 primary
car-diac tumors of which about 126 were
myxomas. None of the pure myoxmas
re-viewed by Ilim were diagnosed in life. The first surgical excision of a myxoma
Oc-(‘uses of
Date .iutizor Primary
Heart Tumor
(‘uses (if pulls
Sex Distribution
Jt(la/es Jf(IIC.* tep,ialp’s Renar/es
1)39 ilcnjamiit l2 3
-
“seem equal’’ Primary heart tumors comprised 0.03 of40,000 autopsies.
194! I.isa 64 3 14-66 yr. 4 17 One case of polypoid fibromit excluded;
sex of t patients not indicated; 1
pe-diatric patient; 5cases of h,,trt tumor in
rt. atrium.
1913 Mahaim 118 8 4-83 yr. 3 39 Excluding 55 cases listed as “Tltrombus”
anti ‘Thromb,ts myxomateux’; 4
pt-diatric J)atients; 14 cases of heart
tu-mor in rt. atrium; I involving rt.
ventri-dc, and left ventricle.
1948 Von Itch, - 30 18-76 yr. 3 27 Selected by common site; locations other
titan left atrium were excluded.
1950 McAllen - 95 4-83 yr. includes some of cases reporteti by
Ma-haim.
1951 Prichard
1953 Bahnson
CLINICAL NOTES 493
TABLE I
REVIEW OF’ LITEIIATIIIE OF CARDIAC MYXOMAS 11)39-1953
“Occurs 3 times mare
fre-quently in females titan
males.”
4O+ i’26± 3mo.-68 yr. Equal frequency
450± 150± 3 mo.-68 yr. -
--* l’redouiinamit location is the left atrium.
Most exhaustive review. Four diagnosed before death.
Totals cannot he reliably determined since authors reduplicated cares in other series. The sign ± indicates approximate figure.
tober, 1951, but the patient died in cardiac arrest. The technic was similar to that used in the snaring of a nasal polyp.
Since the study made by Pnichard in
1951, myxomas have been considered true neoplasms. The series of cases of myxomas
presented by Benjamin/3 Lisa,16 Mahaim,T Von Reis,13 McAllen,4 Pnichard/4 and
Bahnson7 present the incidence, age range,
sex distribution, number of cases in pedi-atric age group and predominant location
of the tumor (Table I).
The atypical history, atypical clinical manifestations and inconstant physical findings render diagnosis of heart tumors
very difficult. In the differential diagnosis one has to consider
(
1) valvular disease, (2) myocarditis, (3) pericarditis with effu-sion, (4) bacterial endocarditis with embo-lism, and (5) coronary disease. However, a careful analysis of the clinical data andan exhaustive cardiac study including
angiocardiography may offer the clues to
the diagnosis.
Operation is the treatment of choice for
myxomas or fibromyxomas of the heart.
With the development of improved
methods for cardiac surgery the patient
with myxoma of the heart can now be
SUMMARY
The case of a 3 3/12-year-old Caucasian boy with a myxofibroma of the left atrium
is presented. The diagnosis was made at
necropsy.
Clinically, the case simulated rheumatic carditis, except that the sedimentation rate
remained normal throughout.
A preference for the prone position with ‘the head lower than the trunk was char-tctenistic.
A terminal episode consisting of severe precordial pain, dyspnea and vomiting,
bead-ing to a state of fatal shock, suggested
con-onary occlusion.
ACKNOWLEDGMENT
The author wishes to thank Dr. Gene Teply and Dr. Randolph G. Flood for permitting this patient’s history to be
pub-lished. Sincere gratitude is expressed to Dr. Dorothy Aggeler and to Dr. Ann Purdy for
their encouragement and to Dr. Howard
R. Bierman who gave valuable
heart. Am. Heart
J.,
26:385, 1943. 2. Scott, R. W., and Carvin, C. F. : Tumorsof the heart and pericardium. Am. Heart
J.,
17:431, 1939.3. Lymburner, R. N. : Tumors of the heart; histopathobogical and clinical study. Canad. M. A.
J.,
30:368, 1934.4. McAblen, P. M. : Myxoma of the left auri-cle. Brit. M.
J.,
1:932, 1950.5. Doane,
J.
C., and Pressman, R. :Ante-mortem diagnosis of tumors of the heart. Am.
J.
M. Sc., 203:520, 1942.6. Willius, F. A., and Amberg, S.: Two cases of secondary tumors of the heart in chib-dren, in one of which diagnosis was made during life. M. Clin. North America, 13:1307, 1930.
7. Bahnson, H. T., and Newman, E. V.: Diag-nosis and surgical removal of intra-cavitary myxoma of the right atrium. Bull. Johns Hopkins Hosp., 93:150, 1953.
8. Roesler, 0. A.: Vier Sebtenere Herzbe-funde. Em Beitrage zur Herz Diagnostik. Zentralbl. Herz. u. Cefasskr., 16:261, 1924.
9. Fishberg, A. N.: Auricular fibrillation and flutter in metastatic growths of right auricle. Am.
J.
M. Sc., 180:629, 1930. 10. C#{246}ttei,L.: Em Fall von pnim#{228}remHerz-tumor. Deutsche med. Wchnschr, 45:
937, 1919.
11. Barnes, A. R., Beaver, D. C., and Snell,
pathological studies. Am. Heart
J.,
9:480, 1934.
12. Shebburne, S. A. : Primary tumors of the heart with special reference to certain features which bed to a logical and
cor-rect diagnosis before death. Ann. mt.
Med., 9:340, 1935.
13. Von Reis, C. : Clinical aspects of
endo-cardial myxoma situated in the left
atrium. Acta med. scandinav., 133:214, 1949.
14. Pnichard, R. W. : Tumors of the heart: re-view of the subject and report of one hundred and fifty cases. Arch. Path.,
51:98, 1951.
15. Benjamin, H. C. : Primary fibromyxoma of the heart. Arch. Path., 27:950, 1939.
16. Lisa,
J.
R., Hirschhorn, L., and Hart,C. A.: Tumors of the heart; report of
4 cases and review of literature. Arch.
mt. Med., 67:91, 1941.
17. Mahaim, I.: Les tumeurs et les polypes
du coeur. Etude Anatomo-Clinique, pp. 162-202, 1945.
18. Goldberg, H. P., Glenn, F., Dotter, C. T., and Steinberg, I: Myxoma of the left atrium; diagnosis made during life with operative and postmortem findings. Cir-culation, 6:762, 1952.
