CLINICAL CASE REPORTS and
as
Letters to the Editor will be published if suitable, space permits. They should not exceed 1,000 words (double in length, and may be subject to editing or abridgment.
spaced)
Lymphoma Associated with Superior Vena Caval Syndrome and Cardiac Tamponade: Case History
Cardiac
H. Hwang, M.D. Alan Brown, M.D. Zhen En Piao, M.D.
Ming
and Patrick J. Scanlon, M.D. HINES and MAYWOOD, ILLINOIS
Abstract A sixty-three-year-old patient with malignant histiocytic lymphoma of the heart presented with both superior vena cava syndrome and cardiac tamponade. A two-dimensional echocardiogram showed a large tumor mass in the right atrium and pericardial effusion with right ventricular compression. Superior and inferior vena cavagrams disclosed a lobulated tumor located in the right atrium that extended into and obstructed the superior vena cava. After the pericardial effusion was drained and the diagnosis was established, the patient was irradiated and given chemotherapy with resolution of the tamponade and superior vena cava obstruction.
Introduction
Malignant lymphoma is one of the common neoplasms to involve the heart.’-5 However, it rarely presents simultaneously with pericardial tamponade and superior vena cava syndrome, two major medical emergencies. 1.3-5 We report a case of malignant histocytic lymphoma of the heart involving the superior vena cava, pericardium, and right atrium and producing these two life-threatening medical emergencies concomitantly. Case History A sixty-three-year-old black man had had irradiation therapy to the larynx and cervical lymph nodes for squamous cell cancer of the vocal chords four years before admission. Two months prior to admission, at which time he complained of an irritable dry cough and mild From the Department of Cardiology, Hines Veterans Administration School of Medicine, Maywood, Illinois
Hospital, Hines,
328
Downloaded from ang.sagepub.com at UCSF LIBRARY & CKM on March 27, 2015
and
Loyola University,
Stritch
329
FIG. 1. Two-dimensional echocardiographic examinations. Apical 4-chamber view showing pericardial effusion and tumor (outlined by arrow), present in the pretherapy examination but absent in the posttherapy examination. Pre: preirradiation and chemotherapy examination; Post: postirradiation and chemotherapy examination; LA: left atrium; LV: left ventricle; RA: right atrium; RV: right ventricle; PE: pericardial effusion. mass
dyspnea, complete physical, chest roentogenographic, and laryngoscopic examinations did not reveal any abnormalities. He was admitted to a local hospital with right upper limb and facial swelling, dyspnea, coughing, and chest pain. A pericardial rub was heard on cardiac examination. An echocardiogram demonstrated a moderate pericardial effusion and a large right atrial mass. Although chest x-ray demonstrated mild cardiomegaly and small bilateral pleural effusions, extensive tumor work-ups, including liver spleen scan, total body gallium scan, bone scan, intravenous pyelogram, upper and lower gastrointestinal series, abdominal ultrasound and sputum cytology, failed to detect any other pathology except of the heart. A computed tomographic (CT) scan of the chest confirmed the pericardial effusion and right atrial mass but did not show any enlarged mediastinal or pulmonary nodules. A lymph node biopsy from the right axillary area was performed and showed reactive hyperplasia. At the time of transfer two weeks later to our institution, he was in moderate distress. Blood pressure was 120/90 mmHg with a 15 mmHg paradoxical pulse and heart rate was 110 beats/minute. Marked swelling of the right upper limb and face was noted. The jugular viens were distended up to the angle of the jaw in a sitting position. Cardiac examination revealed normal S, and S2 and a loud three-component rub. There was a draining surgical wound with induration in the right axilla. Examinations of the abdomen and lower extremities produced nothing remarkable. Laboratory test results, including those from CBC, SMA-18, and electrolytes, were in the normal range. Low voltage and right bundle branch block were seen on ECG. A repeated echocardiogram showed a large right atrial mass and massive pericardial effusion with right ventricular compression (Figure 1). Superior and inferior vena cavograms were performed and showed a lobulated tumor mass in the right atrium extending into the superior vena cava (Figure 2). Therefore, a right-heart catheterization of the right atrium, right ventricle, pulmonary artery, and pulmonary capillary wedge could not be performed. The pressure recordings of superior and inferior venae cavae
Downloaded from ang.sagepub.com at UCSF LIBRARY & CKM on March 27, 2015
330
Fm. 2. (Left) Superior vena cavogram: a lobulated tumor mass in the right atrium extending into and obstructing the superior vena cava. PA: pulmonary artery; RV: right ventricle; SVC: superior vena cava. (Right) Inferior vena cavogram: the tumor mass in the right atrium partially obstructed the inflow of the inferior vena cava. IVC: inferior vena cava; PA: pulmonary artery; RV: right ventricle.
mmHg and 24 mmHg respectively. Shortly thereafter, he developed episodes of tachycardia, hypotension, and orthopnea that were consistent with cardiac tamponade. An emergency pericardial drainage and window, with the intention of removing the intracardiac tumor, was performed. After the removal of 700-800 cc of hemorrhagic pericardial effusion, his systolic blood pressure returned to 100 mmHg. A biopsy of the tumor proved it was a malignant histiocytic lymphoma. However, the tumor masses encased the right hilum and invaded the superior vena cava, the entire right atrium, and parts of the left atrium and pulmonary veins, making surgical excision impossible. A course of 2,800 total rad mediastinal irradiation was given beginning immediately postoperatively with hopes of decreasing the superior vena caval and right atrial obstruction. After two sessions of 400 rad irradiations, the right upper limb and facial swelling was markedly diminished in four days. One week later, in a sternal debridement and sternal rewiring procedure for sternal dehiscence, the surgeon noticed a marked decrease in the size of the tumor mass of the right atrium and superior vena cava. Within three weeks, superior vena cava syndrome was completely resolved and an echocardiogram confirmed the disappearance of the right atrial mass (Figure 1). Although he responded to irradiation well, the therapy was limited by sternal necrosis. With the adjunct chemotherapy, he went into partial remission and was discharged in fair condition. However, four months later, he succumbed to the complication of sternal necrosis and infection. Post mortem showed the lymphoma was in remission. were
34
Discussion
Approximately one fourth of malignant lymphomas involve the heart.’-5 The pericardium is common site of invasion. 1,2,5 Hence, chest pain and pericardial effusion are the common presenting symptoms.’-5 Lymphomas can also involve the great vessels. 1,1,6 Superior vena cava obstruction by an extracavitary and/or intraluminal lymphoma produces superior vena cava syndrome of facial, neck, and upper limb swelling and headache and is a medical emergency.6 Irradiation and chemotherapy are the standard treatments . ~9 Serial echocardiograms show increasing pericardial effusion, and the development of right ventricular compression is characteristic of cardiac tamponade.’° This is another medical emergency. Our patient prethe most
Downloaded from ang.sagepub.com at UCSF LIBRARY & CKM on March 27, 2015
331
sented
simultaneously with cardiac tamponade and superior vena cava syndrome. From the split approach, we not only performed pericardial drainage but also intended to vena caval obstruction by resecting the tumor. Unfortunately, the tumor extended far beyond the ability of surgical removal. Postoperatively, his tumor responded to irradiation and adjuvant chemotherapy with complete resolution of superior vena cava syndrome and pericardial effusion. Echocardiography (M-mode and two-dimensional) is a noninvasive method that easily detects the pericardial effusion and intracavitary tumor masses . 4,11 It may not, however, be able to midsternal relieve the
examine the tumor extension into the vena cava, pulmonary arteries, and veins. CT scan of the chest has been shown to be useful in detecting cardiac tumors . 4,12 In our case, it did not add any more information than that obtained with the echocardiographic examinations. Angiography is another method that can delineate intracardiac lesions.’3 In our patient, superior and inferior vena cavograms clearly showed the extent of the tumor involvement better than echocardiography and CT scanning of the chest did. Cardiac tamponade has characteristic findings from the right-heart catheterization with nearly equal filling pressure of the four cardiac chambers. 14.15 Right-heart catheterization could not be performed on this patient, because of the tumor obstruction in the right atrium. However, both the superior and inferior venae cavae are highly elevated, especially the superior vena cava. After pericardial drainage, the systolic blood pressure increased from 70 mmHg to 110 mmHg immediately. This is strong evidence for cardiac tamponade. &dquo;
Conclusions When a patient with an intracardiac mass and pericardial effusion presents with superior vena caval obstruction and cardiac tamponade without a tissue diagnosis, surgical drainage with the intention of surgical resection of the tumor is a reasonable approach. After relieving the tamponade and establishing the diagnosis, we could not ascertain the primary site of the tumor, and surgical resection could not be performed, because of the extensive involvement of the tumor mass. Luckily, the lymphoma responded well to the combination of irradiation and
chemotherapy.
Acknowledgment The authors express their special thanks to Ms. Susan Ross for her secretarial assistance in the preparation of this manuscript.
Ming H. Hwang, M. D. Cardiology Department (111G) Hines V.A. Hospital Hines, IL 60141
Downloaded from ang.sagepub.com at UCSF LIBRARY & CKM on March 27, 2015
332 ’
References 1. Bisel HF, Wroblewski F, LaDue JS: Incidence and clinical manifestations of cardiac metastases. JAMA 153:712-715, 1953. 2. McAllister HA, Fenoglio JJ: Tumors of the cardiovascular system. In: Atlas of Tumor Pathology, ed. by Hartman WH, Cowan WR. Washington:Armed Forces Institute of Pathology, 1978, Fasc 15, 2nd series. 3. Roberts WC, Glancy DL, DeVita VT: Heart in malignant
4.
5. 6. 7.
8.
lymphoma (Hodgkin’s disease, lymphosarcoma,
reticulum cell sarcoma and mycosis fungoides). A study of 196 autopsy cases. Am J Cardiol 22:85-107, 1968. Wong DWH, Guthaner DF, Gordon EP, et al: Lymphoma of the heart: Catheterization and Cardiovascular Diagnosis 10:377-384, 1984. Griffiths GC: Primary tumors of the heart. Clin Cardiol 13 :183-194, 1962. Lochrige SK, Knibbe WP, Doty DB: Obstruction of the superior vena cava. Surgery 85:14-24, 1979. Perdue DG Jr, Smith RB III: Disease of the Peripheral Veins and the Venae Cavae in the Heart, 5th ed., ed. by Hurst JW. New York: McGraw-Hill Book Co, 1982, p 1515. Perez CA, Presant CA, Amburg AL: Management of superior vena cava syndrome. Semin Oncol 5:128,
.
1978. 9. Hinshaw HC, Murray JF: Disorders of the mediastinum. In: Diseases of the Chest, 4th ed. Philadelphia: WB Saunders Co, 1980, pp 933-934. 10. Schiller NB, Botvinick EH: Right ventricular compression as a sign of cardiac tamponade: An analysis of echocardiographic ventricular dimensions and theii clinical implications. Circulation 56:774, 1977. 11. Feigenbaum H: Pericardial disease and cardiac mass. In: Echocardiography, 4th ed., ed. by Feigenbaum H. Philadelphia: Lea and Febiger, 1986, pp 548-578 and 579-605. 12. Hidalgo H, Korobkin M, Breiman RS, et al: CT of intra-cardiac tumor. AJR 137:608-609, 1981. 13. Webb WR, Gamsu G, Rohlfing BM: Catheter venography in the superior vena cava syndrome. AJR 129:146, 1977. 14. Reddy PS, Curtiss EI, O’Toole JD, et al: Cardiac tamponade : Hemodynamic observations in man. Circulation 58:265, 1978. 15. Lorrel BH, Grossman W: Profiles in constrictive pericarditis, restrictive cardiomyopathy and cardiac tamponade. In: Cardiac Catheterization and Angiography, 3rd ed., ed. by Grossman W. Philadelphia:Lea & Febiger, 1986, pp 433-441.
Downloaded from ang.sagepub.com at UCSF LIBRARY & CKM on March 27, 2015
as
Letters to the Editor will be published if suitable, space permits. They should not exceed 1,000 words (double in length, and may be subject to editing or abridgment.
spaced)
Lymphoma Associated with Superior Vena Caval Syndrome and Cardiac Tamponade: Case History
Cardiac
H. Hwang, M.D. Alan Brown, M.D. Zhen En Piao, M.D.
Ming
and Patrick J. Scanlon, M.D. HINES and MAYWOOD, ILLINOIS
Abstract A sixty-three-year-old patient with malignant histiocytic lymphoma of the heart presented with both superior vena cava syndrome and cardiac tamponade. A two-dimensional echocardiogram showed a large tumor mass in the right atrium and pericardial effusion with right ventricular compression. Superior and inferior vena cavagrams disclosed a lobulated tumor located in the right atrium that extended into and obstructed the superior vena cava. After the pericardial effusion was drained and the diagnosis was established, the patient was irradiated and given chemotherapy with resolution of the tamponade and superior vena cava obstruction.
Introduction
Malignant lymphoma is one of the common neoplasms to involve the heart.’-5 However, it rarely presents simultaneously with pericardial tamponade and superior vena cava syndrome, two major medical emergencies. 1.3-5 We report a case of malignant histocytic lymphoma of the heart involving the superior vena cava, pericardium, and right atrium and producing these two life-threatening medical emergencies concomitantly. Case History A sixty-three-year-old black man had had irradiation therapy to the larynx and cervical lymph nodes for squamous cell cancer of the vocal chords four years before admission. Two months prior to admission, at which time he complained of an irritable dry cough and mild From the Department of Cardiology, Hines Veterans Administration School of Medicine, Maywood, Illinois
Hospital, Hines,
328
Downloaded from ang.sagepub.com at UCSF LIBRARY & CKM on March 27, 2015
and
Loyola University,
Stritch
329
FIG. 1. Two-dimensional echocardiographic examinations. Apical 4-chamber view showing pericardial effusion and tumor (outlined by arrow), present in the pretherapy examination but absent in the posttherapy examination. Pre: preirradiation and chemotherapy examination; Post: postirradiation and chemotherapy examination; LA: left atrium; LV: left ventricle; RA: right atrium; RV: right ventricle; PE: pericardial effusion. mass
dyspnea, complete physical, chest roentogenographic, and laryngoscopic examinations did not reveal any abnormalities. He was admitted to a local hospital with right upper limb and facial swelling, dyspnea, coughing, and chest pain. A pericardial rub was heard on cardiac examination. An echocardiogram demonstrated a moderate pericardial effusion and a large right atrial mass. Although chest x-ray demonstrated mild cardiomegaly and small bilateral pleural effusions, extensive tumor work-ups, including liver spleen scan, total body gallium scan, bone scan, intravenous pyelogram, upper and lower gastrointestinal series, abdominal ultrasound and sputum cytology, failed to detect any other pathology except of the heart. A computed tomographic (CT) scan of the chest confirmed the pericardial effusion and right atrial mass but did not show any enlarged mediastinal or pulmonary nodules. A lymph node biopsy from the right axillary area was performed and showed reactive hyperplasia. At the time of transfer two weeks later to our institution, he was in moderate distress. Blood pressure was 120/90 mmHg with a 15 mmHg paradoxical pulse and heart rate was 110 beats/minute. Marked swelling of the right upper limb and face was noted. The jugular viens were distended up to the angle of the jaw in a sitting position. Cardiac examination revealed normal S, and S2 and a loud three-component rub. There was a draining surgical wound with induration in the right axilla. Examinations of the abdomen and lower extremities produced nothing remarkable. Laboratory test results, including those from CBC, SMA-18, and electrolytes, were in the normal range. Low voltage and right bundle branch block were seen on ECG. A repeated echocardiogram showed a large right atrial mass and massive pericardial effusion with right ventricular compression (Figure 1). Superior and inferior vena cavograms were performed and showed a lobulated tumor mass in the right atrium extending into the superior vena cava (Figure 2). Therefore, a right-heart catheterization of the right atrium, right ventricle, pulmonary artery, and pulmonary capillary wedge could not be performed. The pressure recordings of superior and inferior venae cavae
Downloaded from ang.sagepub.com at UCSF LIBRARY & CKM on March 27, 2015
330
Fm. 2. (Left) Superior vena cavogram: a lobulated tumor mass in the right atrium extending into and obstructing the superior vena cava. PA: pulmonary artery; RV: right ventricle; SVC: superior vena cava. (Right) Inferior vena cavogram: the tumor mass in the right atrium partially obstructed the inflow of the inferior vena cava. IVC: inferior vena cava; PA: pulmonary artery; RV: right ventricle.
mmHg and 24 mmHg respectively. Shortly thereafter, he developed episodes of tachycardia, hypotension, and orthopnea that were consistent with cardiac tamponade. An emergency pericardial drainage and window, with the intention of removing the intracardiac tumor, was performed. After the removal of 700-800 cc of hemorrhagic pericardial effusion, his systolic blood pressure returned to 100 mmHg. A biopsy of the tumor proved it was a malignant histiocytic lymphoma. However, the tumor masses encased the right hilum and invaded the superior vena cava, the entire right atrium, and parts of the left atrium and pulmonary veins, making surgical excision impossible. A course of 2,800 total rad mediastinal irradiation was given beginning immediately postoperatively with hopes of decreasing the superior vena caval and right atrial obstruction. After two sessions of 400 rad irradiations, the right upper limb and facial swelling was markedly diminished in four days. One week later, in a sternal debridement and sternal rewiring procedure for sternal dehiscence, the surgeon noticed a marked decrease in the size of the tumor mass of the right atrium and superior vena cava. Within three weeks, superior vena cava syndrome was completely resolved and an echocardiogram confirmed the disappearance of the right atrial mass (Figure 1). Although he responded to irradiation well, the therapy was limited by sternal necrosis. With the adjunct chemotherapy, he went into partial remission and was discharged in fair condition. However, four months later, he succumbed to the complication of sternal necrosis and infection. Post mortem showed the lymphoma was in remission. were
34
Discussion
Approximately one fourth of malignant lymphomas involve the heart.’-5 The pericardium is common site of invasion. 1,2,5 Hence, chest pain and pericardial effusion are the common presenting symptoms.’-5 Lymphomas can also involve the great vessels. 1,1,6 Superior vena cava obstruction by an extracavitary and/or intraluminal lymphoma produces superior vena cava syndrome of facial, neck, and upper limb swelling and headache and is a medical emergency.6 Irradiation and chemotherapy are the standard treatments . ~9 Serial echocardiograms show increasing pericardial effusion, and the development of right ventricular compression is characteristic of cardiac tamponade.’° This is another medical emergency. Our patient prethe most
Downloaded from ang.sagepub.com at UCSF LIBRARY & CKM on March 27, 2015
331
sented
simultaneously with cardiac tamponade and superior vena cava syndrome. From the split approach, we not only performed pericardial drainage but also intended to vena caval obstruction by resecting the tumor. Unfortunately, the tumor extended far beyond the ability of surgical removal. Postoperatively, his tumor responded to irradiation and adjuvant chemotherapy with complete resolution of superior vena cava syndrome and pericardial effusion. Echocardiography (M-mode and two-dimensional) is a noninvasive method that easily detects the pericardial effusion and intracavitary tumor masses . 4,11 It may not, however, be able to midsternal relieve the
examine the tumor extension into the vena cava, pulmonary arteries, and veins. CT scan of the chest has been shown to be useful in detecting cardiac tumors . 4,12 In our case, it did not add any more information than that obtained with the echocardiographic examinations. Angiography is another method that can delineate intracardiac lesions.’3 In our patient, superior and inferior vena cavograms clearly showed the extent of the tumor involvement better than echocardiography and CT scanning of the chest did. Cardiac tamponade has characteristic findings from the right-heart catheterization with nearly equal filling pressure of the four cardiac chambers. 14.15 Right-heart catheterization could not be performed on this patient, because of the tumor obstruction in the right atrium. However, both the superior and inferior venae cavae are highly elevated, especially the superior vena cava. After pericardial drainage, the systolic blood pressure increased from 70 mmHg to 110 mmHg immediately. This is strong evidence for cardiac tamponade. &dquo;
Conclusions When a patient with an intracardiac mass and pericardial effusion presents with superior vena caval obstruction and cardiac tamponade without a tissue diagnosis, surgical drainage with the intention of surgical resection of the tumor is a reasonable approach. After relieving the tamponade and establishing the diagnosis, we could not ascertain the primary site of the tumor, and surgical resection could not be performed, because of the extensive involvement of the tumor mass. Luckily, the lymphoma responded well to the combination of irradiation and
chemotherapy.
Acknowledgment The authors express their special thanks to Ms. Susan Ross for her secretarial assistance in the preparation of this manuscript.
Ming H. Hwang, M. D. Cardiology Department (111G) Hines V.A. Hospital Hines, IL 60141
Downloaded from ang.sagepub.com at UCSF LIBRARY & CKM on March 27, 2015
332 ’
References 1. Bisel HF, Wroblewski F, LaDue JS: Incidence and clinical manifestations of cardiac metastases. JAMA 153:712-715, 1953. 2. McAllister HA, Fenoglio JJ: Tumors of the cardiovascular system. In: Atlas of Tumor Pathology, ed. by Hartman WH, Cowan WR. Washington:Armed Forces Institute of Pathology, 1978, Fasc 15, 2nd series. 3. Roberts WC, Glancy DL, DeVita VT: Heart in malignant
4.
5. 6. 7.
8.
lymphoma (Hodgkin’s disease, lymphosarcoma,
reticulum cell sarcoma and mycosis fungoides). A study of 196 autopsy cases. Am J Cardiol 22:85-107, 1968. Wong DWH, Guthaner DF, Gordon EP, et al: Lymphoma of the heart: Catheterization and Cardiovascular Diagnosis 10:377-384, 1984. Griffiths GC: Primary tumors of the heart. Clin Cardiol 13 :183-194, 1962. Lochrige SK, Knibbe WP, Doty DB: Obstruction of the superior vena cava. Surgery 85:14-24, 1979. Perdue DG Jr, Smith RB III: Disease of the Peripheral Veins and the Venae Cavae in the Heart, 5th ed., ed. by Hurst JW. New York: McGraw-Hill Book Co, 1982, p 1515. Perez CA, Presant CA, Amburg AL: Management of superior vena cava syndrome. Semin Oncol 5:128,
.
1978. 9. Hinshaw HC, Murray JF: Disorders of the mediastinum. In: Diseases of the Chest, 4th ed. Philadelphia: WB Saunders Co, 1980, pp 933-934. 10. Schiller NB, Botvinick EH: Right ventricular compression as a sign of cardiac tamponade: An analysis of echocardiographic ventricular dimensions and theii clinical implications. Circulation 56:774, 1977. 11. Feigenbaum H: Pericardial disease and cardiac mass. In: Echocardiography, 4th ed., ed. by Feigenbaum H. Philadelphia: Lea and Febiger, 1986, pp 548-578 and 579-605. 12. Hidalgo H, Korobkin M, Breiman RS, et al: CT of intra-cardiac tumor. AJR 137:608-609, 1981. 13. Webb WR, Gamsu G, Rohlfing BM: Catheter venography in the superior vena cava syndrome. AJR 129:146, 1977. 14. Reddy PS, Curtiss EI, O’Toole JD, et al: Cardiac tamponade : Hemodynamic observations in man. Circulation 58:265, 1978. 15. Lorrel BH, Grossman W: Profiles in constrictive pericarditis, restrictive cardiomyopathy and cardiac tamponade. In: Cardiac Catheterization and Angiography, 3rd ed., ed. by Grossman W. Philadelphia:Lea & Febiger, 1986, pp 433-441.
Downloaded from ang.sagepub.com at UCSF LIBRARY & CKM on March 27, 2015
