Download Sarcoma of the Pulmonary Artery with Metastases to Pancreas and

This report records the occurrence of SalnwneUo
typhimurium abscess formation in a ventricular aneurysm. The calcified aneurysm in our patient represented
a nidus for implantation of organisms during septicemia.
Viable organisms were cultured from the left ventricular aneurysm in our patient despite the administration of
parenteral ampicillin and chloramphenicol. This parallels the experience of others who have found that once a
Salmonella species has infected the heart or major blood
vessels it has been extremely W c u l t to eradicate with
antibiotics alone.12-14 In Salmonella arteritis with or
without aneurysm formation in large vessels, particularly
the aorta, cures have been reported with surgical excision of the diseased tissue in addition to the administration of intravenous antibiotics.lssl4 Higher doses of
ampicillin early in the hospital course of our patient
might have eradicated the organism.
ACKNOWLEDGMENTS: We are grateful to Dr. Robert
Berger who performed surgery and to Drs. John Richardson
and James Graham who assisted in preparation of pathologic material.
1 Saphra I, Winter JW: Clinical manifestations of salmonellosis in man. N Engl J Med 256:1128-1134, 1957
2 Schneider PJ, Nernoff J, Gold JA: Acute salmonella
endocarditis: report of a case and review. Arch Intern
Med 120:478-482, 1967
3 Doraiswarni S, Friedman SA, Kagan A, et al: Salmonella
endocarditis complicated by a myocardial abscess. Am J
Cardiol26: 102-105, 1970
4 Decker JP, Clancy CF: Salmonella endocarditis: report of
three cases including one of mural endocarditis. Bull Ayer
Clin Lab 4:l-15, 1959
5 Sanders V, Misanik LF: Salmonella myocarditis: report of
a case with ventricular rupture. Am Heart J 68:682-685,
1964
6 Bengtsson E, Hedland R, Nisell A, et al: An epidemic due
to Salmonella typhfmurium (Breslau) occurring in
Sweden in 1953. A d a Med Scand 153:1-20, 1955
7 Hennigar GR,Thabet R, Bundy WE, et al: Salmonellosis
complicated by pancarditis. J Pediat 43:524-531, 1953
8 Shillcin KB: Sdnumella typhimurium pancarditis. Postgrad Med J 45:40-43, 1969
9 Levin HS, Hosier DM: Salmonella pericarditis: report of
a case and review of the literature. Ann Intern Med
55:817-823, 1961
10 Schatz JW, Weiner L, Gallagher HS, et al: Salmonella
pericarditis: an unusual complication of myocardial infarction. Chest 64:264-269, 1973
11 Barnett RN, Zirnmerman SL: Coronary arteritis with fatal
thrombosis due to Salmonella chloreasuis variety kunzendorf. Am Heart J 34441-446, 1947
12 McNally EM, Kennedy RJ, Grace WR: Sdmoneh tnfantis infection of a pre-existent ventricular aneurysm.
Am Heart J 68:541-548, 1964
13 Meade RH, Moran JM: Salmonella arteritis: preoperative
diagnosis and cure of salmonella typhirnurium aortic
aneurysm. N Engl J Med 281:310-312,1969
14 Sower ND, Whelan TJ: Suppurative arteritis due to
Sahonella. Surgery 52:851-859, 1962
CHEST, 66: 4, OCTOBER, 1974
Sarcoma of the Pulmonary Artery
with Metastases to Pancreas and
Adrenal Glands*
N. Gopala Roo, M.D.;OO S. Krishmami, M.D.;?
George Cherkm, M.D.;II and Hemakztha Krishmamt$
Clinical, hemodynamic, angiographic and autopsy features of a patient who died of a pleormorphic sarcoma of
the pulmonary artery are presented. The tumor destroyed
the pulmonary valves and produced pulmonary Incompetence. This, along with the obstruction of the pulmonary
arterial tree due to tumor invasion as well as thrombosis,
resulted in progressive congestive heart failure. There
were metastases In both the adrenal glands and pancreas,
which have not been reported earlier. The gem& of cardiac failure, pulmonary vascular changes and the possibility of antemortem diagnosis are discussed.
P rimary
sarcoma of the great vessels is a rare condition. It was first described by Mandelstamn in 1923
(cited by Sethi et all). Since then, 28 cases have been
reported in the world literature.2-8 The diagnosis is
usually made at autopsy or during surgery. Hemodynamic data are not available in the literature in this
condition. The clinical, hemodynamic, angiographic and
autopsy features of a case of sarcoma of the pulmonary
artery with metastases to the pancreas and adrenal
glands forms the basis of this report. Metastatic involvement of the adrenal glands and pancreas by a pulmonary
artery tumor are being reported for the first time.
A 59-year-old man was admitted to the hospital on Od. 26,
1971, with a history of sharp, severe pain on both sides of the
chest of three months' duration. The pain radiated to the
inferior angle of the left scapula and was aggravated on
walking. There was grade 4 effort intolerance and paroxysmal
cough associated with scanty mucoid expectoration. He cwnplained of vertigo on walking even a few feet and had to lie
down to get relief. The symptoms were rapidly progressive.
At a hospital elsewhere, he was discovered to be hypertensive with congestive heart failure and for this, therapy was
initiated. There was no history of hemoptysis, edema or
cerebrovascular accidents. The patient was a moderate
smoker.
On examination, he was found to be tachypneic with mild
peripheral cyanosis, and peripheral arteriosclerosis. His pulse
rate was 100/min and blood pressure 110/70 mm Hg. Jugular venous pressure was raised to 7 crn from the sternal angle
and displayed prominent 'a' and 'v' waves. The chest was
emphysematous and the apex beat was not palpable. There
were no thrills. Auscultation revealed a normal &-st sound
and slightly accentuated second sound. The splitting of the
second sound was not clear. A grade 2/6 systolic murmur was
heard along the left sternal border and the pulmonary area. A
'From the Departments of Cardiolo and Pathology, Christian Medical College Hospital, ~ e K r e S.
, India.
"Senior Registrar, Department of Cardiology.
Reader in Cardiolo
tiProfesror and ~ e a r b e ~ a r t m eofn tCardiology.
$Reader in Pathology.
SARCOMA OF THE PULMONARY ARTERY 459
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blowing decrescendo early diastolic murmur was heard in the
pulmonary area.
Scattered rales were heard over both sides of the chest. A
pleural rub was heard over the right i n f r a - d a r y region.
The liver was enlarged and tender.
Laboratory studies disclosed the following values: hemoglobin level, 15.2 gm percent; total leukocyte count, 10,700/
cu mm; polymorphonuclears, 66 percent; lymphocytes, 28
percent; eosinophils, 3 percent; basophils, 1 percent and
monocytes, 2 percent. The ESR was 65 mm/lst hour.
Urinalysis and blood sugar levels were normal. The blood
urea nitrogen was 25 mg percent, serum cholesterol was 210
mn
- -Dercent and the VDRL was nonreactive.
Electrocardiogram at the time of admission revealed sinus
rhythm with a QRS axis of +120° in the frontal plane with
evidence of right ventricular hypertrophy with T wave inversion over the rinht
- chest leads.
A posteroanterior roentgenogram revealed cardiomegaly
with a cardiothoracic ratio of 60 percent. The aorta was
unfolded, and there was enlargement of the right atrium. The
main pulmonary artery was not prominent. There was a
peripheral oligemia of the lung fields.
An electrocardiogram repeated later revealed right axis
deviation, right ventricular hypertrophy and clockwise rotation. In addition, the inverted T wave over V1 and Vz at the
time of admission became upright during an episode of pain,
and again
- became invested several davs later.
A diagnosis of thromboembolic pulmonary hypertension
and pulmonary incompetence was made and anticoagulants
were administered. The source of possible embolism was not
apparent. The pleural rub disappeared on the 12th day, but
the cardiac failure remained refractory. The weight increased
by 2 kg.
Right heart catheterization and angiocardiography were
done on the 20th day. The hemodynamic data are presented
in Table 1. The catheter entered one of the lower lobe
pulmonary arteries and a withdrawal tracing showed a gradient of 50 mm Hg ( Fig 1). A wide pulse pressure was seen
in the main pulmonary artery; the diastolic pressure equaled
the right ventricular end diastolic pressure, which was
markedly elevated. The cardiac index and arterial oxygen
saturation were low and the arteriovenous oxygen difference
was increased.
The selective pulmonary arteriogram revealed obstruction
of right middle and lower lobe arteries (Fig 2 ) and the
pulmonary artery branches to the left lung. The main pulmonary artery was of normal size and showed a 6lling defect
near its bifurcation. There was free regurgitation into the
right ventricle. The patient tolerated the procedure well, but
deteriorated during the next 24 hours. He became more
dyspneic with severe bronchospasm and failed to show any
improvement with intermittent positive pressure respiration
and bronchodilators, and subsequently expired.
At autopsy, significant findings were present in the heart,
pulmonary artery, lungs, adrenal glands, pancreas and liver.
The pulmonary arterial wall was thickened and contained,
at its bifurcation, a firm, round, pale grey tumor measuring
2.5 x 2 x 2 cm which bulged into and almost occluded the
lumen. The tumor involved the entire wall of the pulmonary
artery and extended proximally to the pulmonary valve, the
leaflets of which had been completely destroyed by the
tumor (Fig 3 ) . , Distally, the tumor extended along the left
pulmonary artery down to its tertiary branches producing
uniform thickening of the wall and narrowing of the lumen,
the endothelial surface being thrown into longitudinal folds.
The main left lower lobar artery was completely occluded by
the tumor. The right pulmonary artery was not involved by
tumor, but was found to have been occluded by antemortem
thrombus. The lumen of the pulmonary trunk, the right
46U RAO ET A 1
Table 1 4 a r d i a e Catheterisation Data
Pressures
(mm Hg)
Superior Vena Cava
Right atrium
Ot saturation O/,
40.5
a=20
v=14
x=3
y=10
mean = 10
43.0
Right ventricle
104/4
End diastolic = 15
45.5
Main pulmonary artery
95/5
Mean =45
43.0
Right pulmonary artery
45/22
Mean = 35
43.0
Right brachial artery
100/65
Mean =70
96.5
Cardiac index
1.2 L/rnin/M1
Arteriovenous oxygen
dzerence
7.8 Vols%
Gradient between right
pulmonary artery and
main pulmonary artery
50 m m Hg
pulmonary artery and part of the left pulmonary artery
contained antemortem thrombus. The tumor was confined to
the vessel wall and had not involved the aorta, trachea,
bronch'i or hilar nodes.
The right ventricle was hypertrophied and its wall thickness measured 0.7 cm.There was a secondary tumor deposit 2
x 1 x 1 cm in the right lower lobe of the lung peripherally
which was surrounded by an area of infarction measuring 3 x
3 x 4 cm. Large secondary deposits were present in the
adrenal glands, and a secondary nodule was also seen in the
tail of the pancreas.
Histologically, the tumor was composed of hyperchromatic,
spindle-shaped cells with abundant pink staining cytoplasm
showing considerable pleomorphism. The tumor cells were
separated by large amounts of hyalinized strorna. Numerous
mitotic figures and occasional tumor giant cells were seen. NO
cross striations were demonstrable. The secondary deposits
showed a similar appearance.
Many of the peripheral branches of the pulmonary artery
FIGURE1. P r e m pattern in right lower lobe branch of pulmonary artery, right ventricular and withdrawal pressure
tracing from pulmonary artery branch to right lower lobe to
main pulmonary artery. Note gradient of 50 mm Hg wide
pulse pressure in main pulmonary artery, elevated right ventricular end diastolic pressure.
CHEST, 66: 4, OCTOBER, 1974
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FIGURE
2. Selective pulmonary arteriogram showing occlusion
of branches to right middle and lower lobes and branches to
left lower lobe.
were markedly thickened due to intimal fibrosis and organizing thrombus with evidence of recanalization. There was an
area of infarction surrounding a secondary deposit in the
lower lobe of right lung. Both lungs showed many microscopic tumor emboli.
DISCUSSION
The patients' ages at recognition of these tumors have
been between 23 and 81 years, most being between 50
and 85 years.s The majority of cases reported in the
FIGURE3. Gkvrr mppalllllb=
~-ior in main pulmonary
artery and left branch of pulmonary artery. Note involvement
and destruction of pulmonary valve by tumor (arrow).
CHEST, 66: 4, OCTOBER, 1974
literature had progressive congestive right heart failure.
This is explained by the obstruction of the pulmonary
artery and obliteration of a significant part of the pulmonary arterial tree either by tumor emboli or secondary
thrombus formation. In our patient, while the gradient
across the right pulmonary artery branches was due to
thrombosis, ocdusion of the left pulmonary artery
branches was essentially due to the tumor. The clinical
course was punctuated by pleuritic pain and rub and
sigui6cant (evanescent) T wave changes, but there was
no hemoptysis. The occurrence of florid pulmonary valve
incompetence in a clinical setting of pulmonary embolism of short duration and without antecedent disease,
probably suggests destruction of the pulmonary valves
and offers a clue to the diagnosis.
The progressive cardiac failure is reflected by the
diminished cardiac index and marked increase in the
arteriovenous oxygen difference, the latter being a more
sensitive index since the forward flow is adversely affected, as in any other valvular obstruction. The pulmonary valve incompetence enhances the refractoriness
of the cardiac failure. The ventricularization of the pulmonary artery pressure pulse indicates free pulmonary
valve incompetence. Thus,the right ventricle which was
subjected to progressively increasing afterload, is now
confronted with sudden diastolic overload due to free
pulmonary incompetence.
Wackers et als classified the cases in the world literature as those ( l ) involving pulmonary trunk alone, (2)
involving pulmonary trunk and semilunar valves and
(3) involving pulmonary trunk, semilunar valves and
right ventricular outflow tract.
Ours is the fourth case reported with semilunar valve
involvement. Sethi et all have suggested that failure to
hear the pulmonary component of the second sound may
indicate destruction of the valve cusps.
While half the patients had metastases to the lungs,
there were only three instances of distant metastase~.~
In
our patient, in addition to tumor emboli in pulmonary
arteries, there were bilateral adrenal deposits and metastasis to the pancreas which have not been reported
earlier. Pulmonary venous invasion is the plausible
mechanism. The intimal changes in pulmonary arterioles
observed in this patient have been observed by
~thers.l*~
By angiographic examination it was possible to say
that a part of the lung vasculature was occluded, but the
degree of involvement was far more than what was
revealed by the angiographic examination. The differing
modes of involvement of the left pulmonary artery by
tumor and the right pulmonary artery by thrombus
formation was apparent only at autopsy. It must be
mentioned that diagnosis of this condition has been
made only at autopsy in most cases. In the case reported
by Sethi et al,' the patient was diagnosed at thoracotomy having a left hilar mass with involvement of the
left pulmonary artery and also extension into the main
pulmonary artery. A radical left pneumonectomy was
done, and the patient was discharged in good condition.
After six months of relative well-being, the patient was
readmitted with exacerbation of symptoms. During this
visit, pulmonary angiographic examination was per-
SARCOMA OF THE PULMONARY ARTERY 481
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formed and revealed almost complete occlusion of the
pulmonary artery. An emergency pulmonary arteriotomy
using cardiopulmonary bypass was performed; a soft
whitish tumor was removed from the main pulmonary
artery, the right pulmonary artery and its distal branches.
The patient did well for three months after which she
sudden1y died.
Haythorn and others1° have tried irradiation in the
treatment of their patient, but were unsuccessful. Friedman and Smith" have unsuccessfully attempted resecting a pulmonary artery sarcoma. The patient of Jacques
and B a r ~ l a y 'had
~ a localized anaplastic sarcoma of the
main pulmonary artery and was cured following left
pneumonectomy. Hinsch and others (cited by Sethi et
all) reported a case which was diagnosed antemortem
and survived for eight years following pneumonectomy.
Microscopically, the tumor in our patient was composed of spindle cell sarcoma with considerable pleomorphism. Other histologically identified tumors of the
pulmonary artery have included fibrosarcoma, leiomyosarcoma with giant cells, rhabdomyosarcoma, intimal
sarcoma of Hedenger and anaplastic sarcoma.l It is
likely that the advanced stage at which these cases
present and the difficulties encountered in establishing
the diagnosis contribute to the poor prognosis in these
patients.
1 Sethi GK, Slaven JE,Repes JJ, et al: Primary sarcoma of
the pulmonary artery. J Thorac Cardiwasc Surg 63:587593, 1972
2 Munk J, Griffel T, Kogan J: Primary mesenchymoma of
the pulmonary artery-radiological features. Br J Radio1
38:104-111, 1965
3 Nath V: A case of primary sarcoma of the heart. Indian
Med Gaz 66:675-675, 1931
4 Elphinstone RH, Spedor RG: Sarcoma of the pulmonary
artery Thorax 14:333-340, 1959
5 McConnell TH: Bony and cartilagenous turnours of the
heart and great vessels. Report of an osteosarcoma of the
pulmonary artery. Cancer 25:611617,1970
6 Ali MY, Lee GS: Sarcoma of the pulmonary artery.
Cancer 17:1220-1224, 1964
7 Green JR, Crevasse LE,Shanklin DR: Fibromyxosarcoma
of the pulmonary artery-associated with syncope, intractable heart failure, polycythemia and thrombocytopenia.
Am J Cardiol 13:547-552, 1964
8 Martin W, Tuohy EL, Will CH: Primary tumour of the
heart (entrance of the pulmonary artery). Am Heart J
17:728-734, 1939
9 Wackers FJTh, Van der Schoot JD, Hampe JF: Sarcoma
of the pulmonary trunk associated with hemorrhagic tendency. A case report and review of the literature. Cancer
23:339-351, 1969
10 Haythorn SR, Ray WB, WOE RA: Primary fibrimyxosarcoma of the heart and the pulmonary artery. Am J
Path01 17:261-272, 1941
11 Friedman HM, Smith CK: Leiomyosarcoma of the pulmonary artery JAMA 203:809-809, 1968
12 Jacques JE, Barclay R: Solid sarcomatous pulmonary
artery. Br J Dis Chest 543217-220,1960
ANNOUNCEMENTS
Refresher Course on Radiology of Chest Disease
The University of Toronto Faculty of Medicine,
Division of Postgraduate Medical Education and Department of Radiology offer a postgraduate refresher
course on radiology of chest disease, October 25 and
26 at the Mount Sinai Hospital, Toronto. Guest facul-
ty includes Drs. Robert G. Fraser and Richard
Greenspan. For information, please write: The Director, Division of Postgraduate Medical Education,
Medical Sciences Building, University of Toronto,
Toronto M5S 1A8, Ontario, Canada.
Annual 1974 Otolaryngologic Assembly
The assembly will be held October 26-November 1
A separate, but correlated course, Conference on
at the Eye and Ear Infirmary, University of Illinois
Hospital, Chicago. The Department of Otolaryngology, Abraham Lincoln School of Medicine, University
of Illinois at the Medical Center, offers a condensed
basic and clinical program under the direction of Drs.
Emanuel M. Skolnik and Burton J. Soboroff.
482 RAO ET A 1
Radiology in Otolaryngology and Ophthalmology,
will be held November 29 and 30 under the direction
of Dr. Galdino E. Valvassori.
For information, write the appropriate department,
Abraham Lincoln School of Medicine, PO Box 6998,
Chicago, Illinois 60680.
CHEST, 66: 4, OCTOBER, 1974
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