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Location, localization and surgical treatment of cardiac pheochromocytoma
CASE REPORT
Location, Localization and Surgical Treatment of Cardiac Pheochromocytoma Dan J. Aravot, MD, Nicholas R. Banner, BM , MRCP, Abraham M.Cantor, MD, Stergios Theodoropoulos, MD, and Magdi H. Yacoub, FRCS heochromocytoma is a rare tumor. It can occur at anv site in P the sympathoadrenal system. Most lesions occur within the adrenal gland; <2% occur within the chest and most of these are located in the posterior mediastinum.’ Twenty intrapericardial lesions were reported (17 outside or on the surface of the heart, and only 3 intracardiac [intracavitary] ). l -6 Although intrapericardial pheochromocytomas are exceedingly rare they are being reported with increasing frequency, probably because of improved diagnostic techniques. Diagnosis and localization are important because surgical resection is often feasible and potentially curative, because most pheochromocytomas are benign. This article reports an intracardiac pheochromocytoma that was successfully resected, discusses diagnostic techniques and reviews previous publications regarding location, localization and surgical treatment of such lesions. A I j-year-old girl was referred in 1981 with history of sweating attacks. Her blood pressure was 150/l 10 mm Hg, and her urinary excretion of noradrenaline was 10,700 nmo1/24 hours (normal 120 to 500) and of adrenaline 1,210 nmo1124 hours (normal 30 to 120). A computerized tomography scan showed the adrenal glands to be normal, but revealed a mass in the left atrium. After preoperative treatment with phenoxybenzamine and labetalol, a right thoracotomy was performed, but excision of the tumor was not considered feasible at that time. Subsequently an iodine-131 metaiodobenzylguanidine scan was perFrom the Cardiothoracic Unit, Harefield HCSpital, Haretield, Middlesex, National Heart and Lung Institute, London, and Rotherham District General Hospital, Rotherham, England. Dr. Aravot’s present address is: The Transplant Unit, Papworth Hospital, Cambridge. CB3 8RE E&and. Manuscript receiv&‘May 23, 1991; devised manuscript received September 6, 199 1, and accepted Sep tember 9.
formed that outlined the tumor and confirmed it to be a pheochromocytoma. No evidence of me&stases was found. The patient remained symptom-free on phenoxybenzamine and propranololfor 8 years during which her blood pressure was satisfactorily controlled, and she was able to fully participate in the activities of her teenage friends. Her growth and development were normal. She married in 1988, and an unplanned pregnancy occurred in 1989. Termination of pregnancy was advised because of the potential risk and was performed
at 10 weeks gestation. The patient was referred to Harefield hospital. A repeat computerized tomography scan and iodine-131 meta-iodobenzylguanidine scintigraphy (Figure 1) showed no evidence of metastases. During surgery, the left atrium was markedly enlarged. The tumor located within the left atria1 cavity was firmly attached to the wall of the atrium, had a lobulated surface and measured 15 X 9 cm. The tumor was supplied by numerous arteries arising from the posterior mediastinum (probably from the bronchial circulation) passing through the wall of the left atrium to reach the point of attachment. The tumor was resected while the patient underwent cardiopulmonary bypass, and the wall of the left atrium was reconstructed using a large patch of autologous pericardium. Histopathologic examina-
FIGURE 1. Left, iodne-131 metam ecmefchestamlduppar&demen(~view) ~uptAehRveramdwithhtwnersitwtedinM aWan.TheresrenoothesareasofuptakesugwdveofmetaaeKI.Ri~,repe8l scalafterreaectheftumer.
FlGURE2.-m-ofm.CaRshave ebmdmt~crtoplumandreUwirlllHormmidei.Lew-pewere~ (netshewn)deawhwdthetthesecalrwere~edhmste~byreleUvely webvesabbd stmme. (blemete~n-eoain
X250, rerhed
by so%.)
CASE REPORT
283
r
TABLE
I Data on 20 Previously
No.
Year
Location
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21
19601 19613 19631 19745 19783 19794 19812 19822 19822 19822 19822 19822 19822 1982l 1982* 19821 19821 1983l 1985’j 198g4 1990
APwindow LA surface Aortic root LA cavity IA groove RA cavity AP window LA surface LA surface LA surface IA groove LA surface AV groove LA surface LA surface LA surface IA groove LA surface LA surface RA cavity LA cavity
Reported
Cases
of Cardiac
Size
Pheochromocytoma Presentation -
8x5 8x9
Palpitation, Palpitation, Angina SH
3x4x6
Palpitation,
murmur murmur
-
4x5x6 5x5x3 -
Palpitation, SH
5x3x3 5x6 6x 12 -
SH SH SH
9x
murmur -
-
Together Localization
angina, SH
Angiography Angiography Angiography I-MIBG, CT I-MIBG, CT I-MIBG, CT I-MIBG, CT I-MIBG, CT I-MIBG, CT Angiography I-MIBG, CT CT I-MIBG, CT I-MIBG, CT
-
SH SH
15
I-MIBG I-MIBG
with
the Present CPB -
Case Surgical Approach
Outcome
-
Died
Yes No No Yes No No No Yes Yes Yes Yes
Sternotomy Sternotomy Right thoracotomy Thoracotomy Sternotomy Thoracotomy Thoracotomy Thoracotomy Thoracotomy Thoracotomy Sternotomy
Alive Alive
Yes Yes Yes Yes Yes
Right thoracotomy Right thoracotomy Sternotomy Sternotomy Sternotomy
-
Alive Alive Alive Alive Died Alive Alive Alive Alive Alive Alive Died Died Alive Alive
AP = aortiwpulmonary; AV = atrioventricular; CPB = cardiopulmonary bypass; CT = computerized tomography; IA = interatrial; I-MIBG = iodine-131 meta-icdobenzylguanidine; LA = left atrial; RA = right atrial; SH = systemic hypertension.
tion revealed the tumor to be a benign pheochromocytoma (Figure 2). Thepatient had an uneventful recovery after surgery, and imaging 2 weeks postoperatively showed no evidence of residual tumor (Figure I). Uptake of iodine-131 meta-iodobenzylguanidine by the myocardium was absent before surgery, but
showed a normal pattern after the tumor was excised. The patient’s blood pressure returned to normal levels without medication, and urinary catecholamine levels returned to the normal range.
Because pheochromocytomas are usually benign, surgical resection may be curative. Although medical
FlGURE3.DhWmtbnof21urdrrc~A,2right~~a-
hbabialse$&ml.R921eRabid~ cdtaw--from (lmishgframpootak.waB,nd.cua~-from100fd*R~ C,3tumorstocatdatrootof~-4LRmormleR~surface
rrtary.E,9tlanorsanouter~of
imoMnpkft---
kdtabialwd.F,3tImnorsin
bteraWdgroove.G,ltmorhatdomMaW
-. 284
THE AMERICAN
JOURNAL
OF CARDIOLOGY
VOLUME
69
JANUARY
15. 1992
therapy can control hypertension, it may not protect the patient from a hypertensive crisis during stress. The details of the 20 previously reported casesof cardiac pheochromocytomas that were treated surgically are listed in Table I. The location of each tumor is shown in Figure 3. Successful resection of these lesions depends on diagnosis and localization before surgery. Improvements in cardiac imaging techniques have contributed to the management of such cases. The iodine- 13 1 meta-iodobenzylguanidine scan is particularly helpful in localizing and confirming the nature of a pheochromocytoma, but it does not provide precise information about the local extent and anatomical relations of the lesion. It can also provide preoperative information on the presence and extent of unanticipated metastases. *-* Two-dimensional echocardiography is noncontributory,in most cases,because the extent of the tumor cannot be clearly defined.* Theoretically, transesophageal echocardiography may be of value, although its use has not been reported in cardiac pheochromocytoma. Coronary angiography may reveal the tumor circulation, which should be taken into consideration during surgery.* If necessary, the precise spatial relations of the tumor to adjacent cardiac structures can be dem-
onstrated by magnetic resonance imaging, because of its high contrast resolution and multiplane imaging capacity.4 In the present case, dynamic computerized tomography scanning localized the tumor to the left atrium, and scintigraphic scanning confirmed its nature and excluded metastases. The location of the tumor indicated that the major coronary arteries were not involved. In this case, cardiopulmonary bypass was performed before handling the left atrium to reduce the risk of intraoperative hypertension. Cardiac pheochromocytomas do not “shell out” from adjacent tissues, as in the case of tumors in the abdomen or posterior mediastinum.’ In this case, the tumor was located within the cavity of the left atrium with extensive infiltration of the wall. Complete removal of the tumor could only be
achieved by a full thickness excision of the atria1 wall and by replacement with a pericardial patch. In another case where the lesion was intimately related to the coronary arteries, the tumor could only be excised by reimplanting the left main coronary artery and performing a bypass graft to the circumflex coronary artery. l The highly vascular nature of these tumors was demonstrated in 3 patients who died of intraoperative hemorrhage.4 Large nutrient tumor vessels must be identified and tied before dissection. If the lesion is not resectable using conventional techniques, explantation of the heart, and “bench” surgery followed by autotransplantation has been usA Provided that the tumor is confined to the heart, allotransplantation may be a therapeutic option, as it is in other types of cardiac tumors.’
1. Orringer MB, Sisson JC, Glazer G, Shapiro B, Francis I, Behrendt DM, Thompson NW, Lloyd RV. Surgical treatment of cardiac pheochromocytomas. J Thorac Cardiovasc
Surg 1985:89:153-757.
2. Shapiro B, Sisson J, Kalff V, Glowniak J, Satterlee W, Glazer G, Francis I, Bowers R, Thompson N, Orineer M. Gross M. Beierwaltes W. The location of middle mediastinal pheochromocytomas. J Thorac Cardiwasc
Surg 1984;87:814-820.
3. Gopalakrishnan R, Ticzon AR, Cruz PA, Kennedy FB, Daffy FC, Barmada B, Giacobine JW. Cardiac paraganglioma (chemodectoma). A case report and review of the literature. J Thorac Cardiovosc Surg 1978;76:183-189. 4. Kawasuji M, Matsunaga Y, Iwa T. Cardiac phaeochmmocytoma of the interatrial septum. Eur J Cardiothorac Surg 1989;3:175-177. 5. Besterman E, Bmmley LL, Peart WS. An intrapericardial phaeochromocytoma. Br Heart J 1974; 36:318-320. 6. Cooley DA, Deardon MI, Frazier OH, Angelini P. Human cardiac explantation and autotransplantation in a patient with a large cardiac phaeochromocy toma. Tex Heart Imt J 1985;12:171-173. 7. Aravot DJ, Banner NR, Madden B, Aranki S, Khaahani A. Fitzgerald M. Radlev-Smith R. Yaco& MH. Primary cardiac turnours-is there a place for cardiac transplantation? Eur J Cardiorhorac Surg 1989;3:521-524.
CASE REPORT
285
Location, Localization and Surgical Treatment of Cardiac Pheochromocytoma Dan J. Aravot, MD, Nicholas R. Banner, BM , MRCP, Abraham M.Cantor, MD, Stergios Theodoropoulos, MD, and Magdi H. Yacoub, FRCS heochromocytoma is a rare tumor. It can occur at anv site in P the sympathoadrenal system. Most lesions occur within the adrenal gland; <2% occur within the chest and most of these are located in the posterior mediastinum.’ Twenty intrapericardial lesions were reported (17 outside or on the surface of the heart, and only 3 intracardiac [intracavitary] ). l -6 Although intrapericardial pheochromocytomas are exceedingly rare they are being reported with increasing frequency, probably because of improved diagnostic techniques. Diagnosis and localization are important because surgical resection is often feasible and potentially curative, because most pheochromocytomas are benign. This article reports an intracardiac pheochromocytoma that was successfully resected, discusses diagnostic techniques and reviews previous publications regarding location, localization and surgical treatment of such lesions. A I j-year-old girl was referred in 1981 with history of sweating attacks. Her blood pressure was 150/l 10 mm Hg, and her urinary excretion of noradrenaline was 10,700 nmo1/24 hours (normal 120 to 500) and of adrenaline 1,210 nmo1124 hours (normal 30 to 120). A computerized tomography scan showed the adrenal glands to be normal, but revealed a mass in the left atrium. After preoperative treatment with phenoxybenzamine and labetalol, a right thoracotomy was performed, but excision of the tumor was not considered feasible at that time. Subsequently an iodine-131 metaiodobenzylguanidine scan was perFrom the Cardiothoracic Unit, Harefield HCSpital, Haretield, Middlesex, National Heart and Lung Institute, London, and Rotherham District General Hospital, Rotherham, England. Dr. Aravot’s present address is: The Transplant Unit, Papworth Hospital, Cambridge. CB3 8RE E&and. Manuscript receiv&‘May 23, 1991; devised manuscript received September 6, 199 1, and accepted Sep tember 9.
formed that outlined the tumor and confirmed it to be a pheochromocytoma. No evidence of me&stases was found. The patient remained symptom-free on phenoxybenzamine and propranololfor 8 years during which her blood pressure was satisfactorily controlled, and she was able to fully participate in the activities of her teenage friends. Her growth and development were normal. She married in 1988, and an unplanned pregnancy occurred in 1989. Termination of pregnancy was advised because of the potential risk and was performed
at 10 weeks gestation. The patient was referred to Harefield hospital. A repeat computerized tomography scan and iodine-131 meta-iodobenzylguanidine scintigraphy (Figure 1) showed no evidence of metastases. During surgery, the left atrium was markedly enlarged. The tumor located within the left atria1 cavity was firmly attached to the wall of the atrium, had a lobulated surface and measured 15 X 9 cm. The tumor was supplied by numerous arteries arising from the posterior mediastinum (probably from the bronchial circulation) passing through the wall of the left atrium to reach the point of attachment. The tumor was resected while the patient underwent cardiopulmonary bypass, and the wall of the left atrium was reconstructed using a large patch of autologous pericardium. Histopathologic examina-
FIGURE 1. Left, iodne-131 metam ecmefchestamlduppar&demen(~view) ~uptAehRveramdwithhtwnersitwtedinM aWan.TheresrenoothesareasofuptakesugwdveofmetaaeKI.Ri~,repe8l scalafterreaectheftumer.
FlGURE2.-m-ofm.CaRshave ebmdmt~crtoplumandreUwirlllHormmidei.Lew-pewere~ (netshewn)deawhwdthetthesecalrwere~edhmste~byreleUvely webvesabbd stmme. (blemete~n-eoain
X250, rerhed
by so%.)
CASE REPORT
283
r
TABLE
I Data on 20 Previously
No.
Year
Location
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21
19601 19613 19631 19745 19783 19794 19812 19822 19822 19822 19822 19822 19822 1982l 1982* 19821 19821 1983l 1985’j 198g4 1990
APwindow LA surface Aortic root LA cavity IA groove RA cavity AP window LA surface LA surface LA surface IA groove LA surface AV groove LA surface LA surface LA surface IA groove LA surface LA surface RA cavity LA cavity
Reported
Cases
of Cardiac
Size
Pheochromocytoma Presentation -
8x5 8x9
Palpitation, Palpitation, Angina SH
3x4x6
Palpitation,
murmur murmur
-
4x5x6 5x5x3 -
Palpitation, SH
5x3x3 5x6 6x 12 -
SH SH SH
9x
murmur -
-
Together Localization
angina, SH
Angiography Angiography Angiography I-MIBG, CT I-MIBG, CT I-MIBG, CT I-MIBG, CT I-MIBG, CT I-MIBG, CT Angiography I-MIBG, CT CT I-MIBG, CT I-MIBG, CT
-
SH SH
15
I-MIBG I-MIBG
with
the Present CPB -
Case Surgical Approach
Outcome
-
Died
Yes No No Yes No No No Yes Yes Yes Yes
Sternotomy Sternotomy Right thoracotomy Thoracotomy Sternotomy Thoracotomy Thoracotomy Thoracotomy Thoracotomy Thoracotomy Sternotomy
Alive Alive
Yes Yes Yes Yes Yes
Right thoracotomy Right thoracotomy Sternotomy Sternotomy Sternotomy
-
Alive Alive Alive Alive Died Alive Alive Alive Alive Alive Alive Died Died Alive Alive
AP = aortiwpulmonary; AV = atrioventricular; CPB = cardiopulmonary bypass; CT = computerized tomography; IA = interatrial; I-MIBG = iodine-131 meta-icdobenzylguanidine; LA = left atrial; RA = right atrial; SH = systemic hypertension.
tion revealed the tumor to be a benign pheochromocytoma (Figure 2). Thepatient had an uneventful recovery after surgery, and imaging 2 weeks postoperatively showed no evidence of residual tumor (Figure I). Uptake of iodine-131 meta-iodobenzylguanidine by the myocardium was absent before surgery, but
showed a normal pattern after the tumor was excised. The patient’s blood pressure returned to normal levels without medication, and urinary catecholamine levels returned to the normal range.
Because pheochromocytomas are usually benign, surgical resection may be curative. Although medical
FlGURE3.DhWmtbnof21urdrrc~A,2right~~a-
hbabialse$&ml.R921eRabid~ cdtaw--from (lmishgframpootak.waB,nd.cua~-from100fd*R~ C,3tumorstocatdatrootof~-4LRmormleR~surface
rrtary.E,9tlanorsanouter~of
imoMnpkft---
kdtabialwd.F,3tImnorsin
bteraWdgroove.G,ltmorhatdomMaW
-. 284
THE AMERICAN
JOURNAL
OF CARDIOLOGY
VOLUME
69
JANUARY
15. 1992
therapy can control hypertension, it may not protect the patient from a hypertensive crisis during stress. The details of the 20 previously reported casesof cardiac pheochromocytomas that were treated surgically are listed in Table I. The location of each tumor is shown in Figure 3. Successful resection of these lesions depends on diagnosis and localization before surgery. Improvements in cardiac imaging techniques have contributed to the management of such cases. The iodine- 13 1 meta-iodobenzylguanidine scan is particularly helpful in localizing and confirming the nature of a pheochromocytoma, but it does not provide precise information about the local extent and anatomical relations of the lesion. It can also provide preoperative information on the presence and extent of unanticipated metastases. *-* Two-dimensional echocardiography is noncontributory,in most cases,because the extent of the tumor cannot be clearly defined.* Theoretically, transesophageal echocardiography may be of value, although its use has not been reported in cardiac pheochromocytoma. Coronary angiography may reveal the tumor circulation, which should be taken into consideration during surgery.* If necessary, the precise spatial relations of the tumor to adjacent cardiac structures can be dem-
onstrated by magnetic resonance imaging, because of its high contrast resolution and multiplane imaging capacity.4 In the present case, dynamic computerized tomography scanning localized the tumor to the left atrium, and scintigraphic scanning confirmed its nature and excluded metastases. The location of the tumor indicated that the major coronary arteries were not involved. In this case, cardiopulmonary bypass was performed before handling the left atrium to reduce the risk of intraoperative hypertension. Cardiac pheochromocytomas do not “shell out” from adjacent tissues, as in the case of tumors in the abdomen or posterior mediastinum.’ In this case, the tumor was located within the cavity of the left atrium with extensive infiltration of the wall. Complete removal of the tumor could only be
achieved by a full thickness excision of the atria1 wall and by replacement with a pericardial patch. In another case where the lesion was intimately related to the coronary arteries, the tumor could only be excised by reimplanting the left main coronary artery and performing a bypass graft to the circumflex coronary artery. l The highly vascular nature of these tumors was demonstrated in 3 patients who died of intraoperative hemorrhage.4 Large nutrient tumor vessels must be identified and tied before dissection. If the lesion is not resectable using conventional techniques, explantation of the heart, and “bench” surgery followed by autotransplantation has been usA Provided that the tumor is confined to the heart, allotransplantation may be a therapeutic option, as it is in other types of cardiac tumors.’
1. Orringer MB, Sisson JC, Glazer G, Shapiro B, Francis I, Behrendt DM, Thompson NW, Lloyd RV. Surgical treatment of cardiac pheochromocytomas. J Thorac Cardiovasc
Surg 1985:89:153-757.
2. Shapiro B, Sisson J, Kalff V, Glowniak J, Satterlee W, Glazer G, Francis I, Bowers R, Thompson N, Orineer M. Gross M. Beierwaltes W. The location of middle mediastinal pheochromocytomas. J Thorac Cardiwasc
Surg 1984;87:814-820.
3. Gopalakrishnan R, Ticzon AR, Cruz PA, Kennedy FB, Daffy FC, Barmada B, Giacobine JW. Cardiac paraganglioma (chemodectoma). A case report and review of the literature. J Thorac Cardiovosc Surg 1978;76:183-189. 4. Kawasuji M, Matsunaga Y, Iwa T. Cardiac phaeochmmocytoma of the interatrial septum. Eur J Cardiothorac Surg 1989;3:175-177. 5. Besterman E, Bmmley LL, Peart WS. An intrapericardial phaeochromocytoma. Br Heart J 1974; 36:318-320. 6. Cooley DA, Deardon MI, Frazier OH, Angelini P. Human cardiac explantation and autotransplantation in a patient with a large cardiac phaeochromocy toma. Tex Heart Imt J 1985;12:171-173. 7. Aravot DJ, Banner NR, Madden B, Aranki S, Khaahani A. Fitzgerald M. Radlev-Smith R. Yaco& MH. Primary cardiac turnours-is there a place for cardiac transplantation? Eur J Cardiorhorac Surg 1989;3:521-524.
CASE REPORT
285