- Email: [email protected]
Sarcoma of the Pulmonary Artery with Metastases to Pancreas and Adrenal Glands
This report records the occurrence of Salmonella 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 difficult 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. 13•14 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.
REFERENCES
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 Doraiswami 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 4:1-15, 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 typhimurium ( Breslau) occurring in Sweden in 1953. Acta 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: Salmonella 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, Zimmerman SL: Coronary arteritis with fatal thrombosis due to Salmonella chlMeasuis variety lcunzendorf. Am Heart J 34:441-446, 1947 12 McNally EM, Kennedy RJ, Grace WR: Salmonella in/antis 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 typhimurium aortic aneurysm. N Engl J Med 281:310-312, 1969 14 Sower ND, Whelan TJ: Suppurative arteritis due to Salmonella. Surgery 52:851-859, 1962
CHEST, 66: 4, OCTOBER, 1974
Sarcoma of the Pulmonary Artery with Metastases to Pancreas and Adrenal Glands* N. Gopala Rao, M.D.;•• S. Krishnaswami, M.D.;t George Cherian, M.D.;JJ and Hemalatha Krishnaswamit
Clinical, hemodynamic:, BDgiographic: and autopsy features of a patient who died of a pleonnorphic: 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 weD as thrombosis, resulted in progressive congestive heart failure. There were metastases in both the adrenal glands and pancreas, which have not been reported earHer. The genesis of cardiac: failure, pulmonary vascular changes and the possibility of antemortem diagnosis are disc:ussed. sarcoma of the great vessels is a rare condiP rimary first described by Mandelstamn in 1923 tion. It was (cited by Sethi et a!t) . 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 Oct. 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 complained 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 em 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 first 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 Cardiology and Pathology, Christian Medical College Hospital, Vellore, S. India. ••senior Registrar, Department of Cardiology. tReader in Cardiologr. !!Professor and Head, Department of Cardiology. tReader in Pathology.
SARCOMA OF THE PULMONARY ARTERY 459
blowing decrescendo early diastolic murmur was heard in tbe pulmonary area. Scattered rales were heard over both sides of the chest. A pleural rub was heard over the right infra-axillary 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 mg percent 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 right 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 V 1 and V2 at the time of admission became upright during an episode of pain, and again became inverted several days 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 filling 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 em 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 artezy 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
460 RAO ET AL
Table !-Cardiae CaJheterisation Data Pressures (mm Hg)
Superior Vena Cava Right atrium
Ot saturation % 46.5
a=20 v=14 x=3 y=lO mean=lO
43.0
Right ventricle
104/4 End dia.stolic= 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/min/W
Arteriovenous oxygen difference
7.8 Vols%
Gradient between right pulmonary artery and main pulmonary artery
50 mm 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, bronchi or hilar nodes. The right ventricle was hypertrophied and its wall thickness measured 0.7 em. There was a secondary tumor deposit 2 X 1 X 1 em in the right lower lobe of the lung peripherally which was surrounded by an area of infarction measuring 3 X 3 X 4 em. 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 stroma. 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
FIGURE 1. Pressure 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
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 65 years. 9 The majority of cases reported in the
3. Gross appearance of tumor in main pulmonary artery and left branch of pulmonary artery. Note involvement and destruction of pulmonary valve by tumor (a"ow). FIGURE
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, occlusion of the left pulmonary artery branches was essentially due to the tumor. The clinical course was punctuated by pleuritic pain and rub and significant (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 al 9 classified the cases in the world literature as those ( 1) 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 al 1 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 metastases. 9 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 others. 1 •9 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, 1 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
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 suddenly died. Haythorn and others 10 have tried irradiation in the treatment of their patient, but were unsuccessful. Friedman and Smith 11 have unsuccessfully attempted resecting a pulmonary artery sarcoma.· The patient of Jacques and Barclay 12 had a localized anaplastic sarcoma of the main pulmonary artery and was cured following left pneumonectomy. Hinsch and others (cited by Sethi et al 1 ) 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. 1 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. REFERENCES
1 Sethi GK, Slaven ]E, Repes ]], et al: Primary sarcoma of
2 3 4 5 6 7
8 9
10 11 12
the pulmonary artery. J Thorac Cardiovasc Surg 63:587593, 1972 Munk J, Griffel T, Kogan J: Primary mesenchymoma of the pulmonary artery-radiological features. Br J Radio} 38:104-111, 1965 Nath V: A case of primary sarcoma of the heart. Indian Med Gaz 66:675-675, 1931 Elphinstone RH, Spector RG: Sarcoma of the pulmonary artery Thorax 14:333-340, 1959 McConnell TH: Bony and cartilagenous tumours of the heart and great vessels. Report of an osteosarcoma of the pulmonary artery. Cancer 25:611-617, 1970 Ali MY, Lee GS: Sarcoma of the pulmonary artery. Cancer 17:1220-1224, 1964 Green JR, Crevasse LE, Shanklin DR: Fibromyxosarcoma of the pulmonary artery-associated with syncope, intractable heart failure, polycythemia and thrombocytopenia. Am J Cardiol13:547-552, 1964 Martin W, Tuohy EL, Will CH: Primary tumour of the heart (entrance of the pulmonary artery). Am Heart J 17:728-734, 1939 Wackers FJTh, Vander 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 Haythorn SR, Ray WB, Wolff RA: Primary fibrimyxosarcoma of the heart and the pulmonary artery. Am J Pathol 17:261-272, 1941 Friedman HM, Smith CK: Leiomyosarcoma of the pulmonary artery JAMA 203:809-809, 1968 Jacques JE, Barclay R: Solid sarcomatous pulmonary artery. Br J Dis Olest 54:217-220, 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 lAB, Ontario, Canada.
Annual1974 Otolaryngologic Assembly The assembly will be held October 26-November I 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 Al
A separate, but correlated course, Conference on 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
REFERENCES
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 Doraiswami 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 4:1-15, 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 typhimurium ( Breslau) occurring in Sweden in 1953. Acta 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: Salmonella 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, Zimmerman SL: Coronary arteritis with fatal thrombosis due to Salmonella chlMeasuis variety lcunzendorf. Am Heart J 34:441-446, 1947 12 McNally EM, Kennedy RJ, Grace WR: Salmonella in/antis 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 typhimurium aortic aneurysm. N Engl J Med 281:310-312, 1969 14 Sower ND, Whelan TJ: Suppurative arteritis due to Salmonella. Surgery 52:851-859, 1962
CHEST, 66: 4, OCTOBER, 1974
Sarcoma of the Pulmonary Artery with Metastases to Pancreas and Adrenal Glands* N. Gopala Rao, M.D.;•• S. Krishnaswami, M.D.;t George Cherian, M.D.;JJ and Hemalatha Krishnaswamit
Clinical, hemodynamic:, BDgiographic: and autopsy features of a patient who died of a pleonnorphic: 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 weD as thrombosis, resulted in progressive congestive heart failure. There were metastases in both the adrenal glands and pancreas, which have not been reported earHer. The genesis of cardiac: failure, pulmonary vascular changes and the possibility of antemortem diagnosis are disc:ussed. sarcoma of the great vessels is a rare condiP rimary first described by Mandelstamn in 1923 tion. It was (cited by Sethi et a!t) . 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 Oct. 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 complained 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 em 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 first 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 Cardiology and Pathology, Christian Medical College Hospital, Vellore, S. India. ••senior Registrar, Department of Cardiology. tReader in Cardiologr. !!Professor and Head, Department of Cardiology. tReader in Pathology.
SARCOMA OF THE PULMONARY ARTERY 459
blowing decrescendo early diastolic murmur was heard in tbe pulmonary area. Scattered rales were heard over both sides of the chest. A pleural rub was heard over the right infra-axillary 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 mg percent 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 right 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 V 1 and V2 at the time of admission became upright during an episode of pain, and again became inverted several days 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 filling 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 em 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 artezy 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
460 RAO ET AL
Table !-Cardiae CaJheterisation Data Pressures (mm Hg)
Superior Vena Cava Right atrium
Ot saturation % 46.5
a=20 v=14 x=3 y=lO mean=lO
43.0
Right ventricle
104/4 End dia.stolic= 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/min/W
Arteriovenous oxygen difference
7.8 Vols%
Gradient between right pulmonary artery and main pulmonary artery
50 mm 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, bronchi or hilar nodes. The right ventricle was hypertrophied and its wall thickness measured 0.7 em. There was a secondary tumor deposit 2 X 1 X 1 em in the right lower lobe of the lung peripherally which was surrounded by an area of infarction measuring 3 X 3 X 4 em. 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 stroma. 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
FIGURE 1. Pressure 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
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 65 years. 9 The majority of cases reported in the
3. Gross appearance of tumor in main pulmonary artery and left branch of pulmonary artery. Note involvement and destruction of pulmonary valve by tumor (a"ow). FIGURE
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, occlusion of the left pulmonary artery branches was essentially due to the tumor. The clinical course was punctuated by pleuritic pain and rub and significant (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 al 9 classified the cases in the world literature as those ( 1) 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 al 1 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 metastases. 9 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 others. 1 •9 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, 1 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
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 suddenly died. Haythorn and others 10 have tried irradiation in the treatment of their patient, but were unsuccessful. Friedman and Smith 11 have unsuccessfully attempted resecting a pulmonary artery sarcoma.· The patient of Jacques and Barclay 12 had a localized anaplastic sarcoma of the main pulmonary artery and was cured following left pneumonectomy. Hinsch and others (cited by Sethi et al 1 ) 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. 1 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. REFERENCES
1 Sethi GK, Slaven ]E, Repes ]], et al: Primary sarcoma of
2 3 4 5 6 7
8 9
10 11 12
the pulmonary artery. J Thorac Cardiovasc Surg 63:587593, 1972 Munk J, Griffel T, Kogan J: Primary mesenchymoma of the pulmonary artery-radiological features. Br J Radio} 38:104-111, 1965 Nath V: A case of primary sarcoma of the heart. Indian Med Gaz 66:675-675, 1931 Elphinstone RH, Spector RG: Sarcoma of the pulmonary artery Thorax 14:333-340, 1959 McConnell TH: Bony and cartilagenous tumours of the heart and great vessels. Report of an osteosarcoma of the pulmonary artery. Cancer 25:611-617, 1970 Ali MY, Lee GS: Sarcoma of the pulmonary artery. Cancer 17:1220-1224, 1964 Green JR, Crevasse LE, Shanklin DR: Fibromyxosarcoma of the pulmonary artery-associated with syncope, intractable heart failure, polycythemia and thrombocytopenia. Am J Cardiol13:547-552, 1964 Martin W, Tuohy EL, Will CH: Primary tumour of the heart (entrance of the pulmonary artery). Am Heart J 17:728-734, 1939 Wackers FJTh, Vander 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 Haythorn SR, Ray WB, Wolff RA: Primary fibrimyxosarcoma of the heart and the pulmonary artery. Am J Pathol 17:261-272, 1941 Friedman HM, Smith CK: Leiomyosarcoma of the pulmonary artery JAMA 203:809-809, 1968 Jacques JE, Barclay R: Solid sarcomatous pulmonary artery. Br J Dis Olest 54:217-220, 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 lAB, Ontario, Canada.
Annual1974 Otolaryngologic Assembly The assembly will be held October 26-November I 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.
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A separate, but correlated course, Conference on 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