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Multiple aortic valve papillary fibroelastoma: an unusual presentation of a rare tumor
CASE REPORTS
Multiple Aortic Valve Papillary Fibroelastoma: An Unusual Presentation of a Rare Tumor Yoram Neuman, MD, Daniel J. Luthringer, MD, Sergio Kobal, MD, Takashi Miyamoto, MD, Alfredo Trento, MD, and Robert J. Siegel, MD, FACC, Los Angeles, California
Cardiac papillary fibroelastoma is a rare cardiac tumor and occurs mainly on cardiac valves. The incidence of multiple lesions is exceedingly rare and is about 7% of all reported cases. Careful echocardiographic evaluation of the patient before operation is of high importance as the there is no recurrence after surgical excision. We present the first
Cardiac papillary fibroelastoma (CPF) is a rare
cardiac tumor that originates mainly from the valvular endocardium. It can lead to serious embolic complications including death1 or may be found incidentally at autopsy. Most of these lesions are single but in 6.8% to 7.5% of the cases, multiple CPF are detected.2-5 Because it is completely curable by surgical resection with no reported cases of recurrence, it is important to identify all lesions before cardiac operation. Only 3 cases of multiple aortic CPF have been reported in the English-language literature; in all, the diagnosis of multiple lesions was made only during operation.6 This is the first reported case of the presurgical diagnosis of multiple CPF of the aortic valve.
CASE REPORT A 75-year-old man with a medical history of smoking, emphysema, adult-onset diabetes mellitus, hyperlipidemia, and systemic hypertension was evaluated for an episode of diaphoresis. Physical examination was unremarkable. Specifically, blood pressure was 130/75 mm Hg and he had no cardiac murmurs, abnormal heart sounds, or signs of peripheral embolization. Laboratory results showed normal blood count and sedimentation rate. Kidney and liver tests were within normal range. A stress echocardiogram was negative for ischemia, however, it revealed an indistinct mass involving the aortic valve. A From the Division of Cardiology, Anatomic Pathology, and Division of Cardiothoracic Surgery (A.T.), Cedars-Sinai Medical Center. Reprint requests: Robert J. Siegel, MD, FACC, Cardiac Noninvasive Laboratory, Room No. 5335, Cedars-Sinai Medical Center, Los Angeles, CA 90048 (E-mail: [email protected]). Copyright 2003 by the American Society of Echocardiography. 0894-7317/2003/$30.00 ⫹ 0 doi:10.1016/S0894-7317(03)00117-2
494
case of a patient with multiple aortic valve papillary fibroelastomas diagnosed before operation. The patient underwent surgical removal of the 3 masses that were confirmed as cardiac papillary fibroelastomas by pathologic examination. There was no evidence of aortic insufficiency after operation. (J Am Soc Echocardiogr 2003;16:494-6.)
transesophageal echocardiogram (TEE) was performed and 3 echo densities were found on the aortic valve cusps. The masses measured 14 ⫻ 15 mm on the noncoronary cusp, 8 ⫻ 9 mm on the right coronary cusp lesion, and 2 ⫻ 2 mm on the left coronary cusp. As shown in Figure 1, all 3 lesions were on the ventricular aspect of the aortic valve. Additional findings included moderate mitral regurgitation, an atrial septal aneurysm, and moderate atheromatous plaquing of the descending thoracic aorta. Subsequently 3 sets of blood cultures were obtained to exclude infective endocarditis and were negative. A diagnosis of CPF of the aortic valve was made and an operation was recommended to resect the lesions as a result of their potential for embolization. At operation, by direct inspection of the aortic valve through an aortotomy as demonstrated in Figure 2, the largest lesion was a mobile, gelatinous mass attached to a short stalk on the free edge of the noncoronary cusp; a second mass was on the right coronary cusp; and the smallest lesion was on the left coronary cusp. All the lesions were excised and the aortic valve was preserved. The patient tolerated the procedure well with no residual valvular regurgitation found on intraoperative TEE. Light microscopic assessment (Figure 3) using hematoxylin and eosin sections demonstrated lesions characterized by papillary architecture, having elongated, narrow stalks with central cores of dense fibrous connective tissue devoid of blood vessels. Extracellular matrix was composed of collagen, myxoid mucopolysaccharides, and elastic fibers (elastin-van Gieson’s stain). The surface was partially lined by attenuated endothelium.
DISCUSSION CPF is an uncommon benign cardiac tumor. It accounts for up to 16% of cardiac tumors in pathologic studies,2 but only 1% in surgical series.3 It is the most common tumor of cardiac valves. CPF is
Journal of the American Society of Echocardiography Volume 16 Number 5
Neuman et al 495
Figure 1 Transesopageal echocardiographic short-axis view of aortic valve. A, Systolic view demonstrating large noncoronary cusp (NCC) papillary fibroelastoma. B, Diastolic view demonstrating both NCC and right coronary cusp (RCC) masses, and partial visualization of small left coronary cusp (LCC) lesion.
most commonly located on the aortic valve cusps (44.5%) and on the mitral valve leaflets (36.4%).4 In more then 90% of reported cases it was found to be a solitary lesion, however, in 7% to 13% of cases, lesions were found in more than 1 site (ie, on more than 1 valve or on a valve and on the ventricular endocardium).4,5 However, multiple lesions on the same valve are extremely rare with only 3 cases reported in the English-language literature for multiple lesions on the aortic valve.6 None of those CPF were diagnosed as multiple lesions before operation as the concomitant tumors were small (2 mm). In our case, the 3 lesions were identified by TEE. These findings were confirmed by direct visualization during operation. Because CPF can be small, they can easily be missed by TEE. This emphasizes the importance of careful direct visual examination of the valve during operation and optimal visualization of the other valves by TEE to reduce the likelihood of missing lesions on the same valve, other valves, or other cardiac structures. Sun et al4 have suggested that the presence of large (⬎1.0 cm) mobile CPF on the aortic or mitral valve may be an indication for prophylactic operation in the patient who is asymptomatic. A second issue of major clinical importance is the evaluation of concomitant ischemic heart disease before operation. Because the mean age of these patients is about 60 years old4 and many, as in our case, have risk factors for ischemic heart disease, information regarding possible coronary artery disease and, hence, a need for bypass operation as well, is important. However, because of the friability of these aortic valve lesions and the potential for embolization,7 it is probably better to avoid catheter manipulation near the aortic valve and the coronary artery ostia. Therefore, noninvasive modalities for evaluation of ischemia should be considered. In our case we preformed a stress thallium scan that did not show any evidence of inducible ischemia. The exact histogenesis of the papillary fibroelastoma is uncertain. Despite classification by many as
Figure 2 Intraoperative findings. Surgical view through aortotomy demonstrates 2 larger masses on noncoronary cusp (NCC) and right coronary cusp (RCC), and smaller lesion on left coronary cusp (LCC). Asterisk marks right coronary artery ostium.
Figure 3 A, Gross specimen of 3 masses demonstrating characteristic gelatinous appearance of papillary fibroelastoma. B, Hematoxylin and eosin sections of masses revealing typical papillary architecture of cardiac papillary fibroelastoma, composed of central cores of avascular fibrous connective tissue, in combination with myxoid extracellular matrix and partial lining of endothelial cells on surface (original magnification ⫻100). Inset demonstrates higher magnification (original magnification ⫻20). LCC, Left coronary cusp; NCC, noncoronary cusp; RCC, right coronary cusp.
a neoplasm, it may represent exuberant organization of thrombi, in many ways similar to the much smaller Lambl’s excrescences, which are frequently found on the aortic valve of older individuals.8
496 Neuman et al
Colocalization of Lambl’s excrescences and papillary fibroelastomas on the same valve suggests a common histogenesis.6 A virus-induced tumor hypothesis has also been suggested on the basis of finding dendritic cells and cytomegalovirus virus remnants in the intermediate layer of the tumor.9 Such a mechanism could also explain multicentricity. In summary, our report is the first documented case of the presurgical diagnosis of multiple aortic valve papillary fibroelastomas.
REFERENCES 1. Takada A, Saito K, Ro A, Tokudome S, Murai T. Papillary fibroelastoma of the aortic valve: a sudden death case of coronary embolism with myocardial infarction. Forensic Sci Int 2000;113:209-14. 2. Goldhaber S, Braunwald E. Primary cardiac tumors. In: Atlas of heart diseases. Philadelphia: Current Medicine; 1995. p. 15.115.22.
Journal of the American Society of Echocardiography May 2003
3. Molina JE, Edwards JE, Ward HB. Primary cardiac tumors: experience at the University of Minnesota. Thorac Cardiovasc Surg 1990;38:183-91. 4. Sun JP, Asher CR, Yang XS, Cheng GG, Scalia GM, Massed AG, et al. Clinical and echocardiographic characteristics of papillary fibroelastoma: a retrospective and prospective study in 162 patients. Circulation 2001;103:2687-93. 5. Grinda JM, Couetil JP, Chauvaud S, D’Attelis N, Berrebi A, Fabiani JN, et al. Cardiac valve papillary fibroelastoma: surgical excision for revealed or potential embolization. J Thorac Cardiovasc Surg 1999;17:106-10. 6. Touati GD, Carmi D, Sevestre H, Poulain H. Multiple aortic valve papillary fibroelastoma: do not miss the other one [letter]. Eur J Cardiothorac Surg 2002;21:596-7. 7. Valente M, Basso C, Thiene G, Bressan M, Stritoni P, Cocco P, et al. Fibroelastic papilloma: a not-so-benign cardiac tumor. Cardiovasc Pathol 1992;1:161-6. 8. Schen FJ. The heart. In: Cotran RS, Kumar V, Collins T, editors. Robbins pathologic basis of disease. 6th ed. Philadelphia: W.B. Saunders; 1999. p. 590. 9. Grandmougin D, Fayad G, Moukassa D, Decoence C, Abolmaali K, Bodart JC, et al. Cardiac valve papillary fibroelastomas: clinical, histological and immunohistochemical studies and a physiopathogenic hypothesis. J Heart Valve Dis 2000;9:832-41.
Multiple Aortic Valve Papillary Fibroelastoma: An Unusual Presentation of a Rare Tumor Yoram Neuman, MD, Daniel J. Luthringer, MD, Sergio Kobal, MD, Takashi Miyamoto, MD, Alfredo Trento, MD, and Robert J. Siegel, MD, FACC, Los Angeles, California
Cardiac papillary fibroelastoma is a rare cardiac tumor and occurs mainly on cardiac valves. The incidence of multiple lesions is exceedingly rare and is about 7% of all reported cases. Careful echocardiographic evaluation of the patient before operation is of high importance as the there is no recurrence after surgical excision. We present the first
Cardiac papillary fibroelastoma (CPF) is a rare
cardiac tumor that originates mainly from the valvular endocardium. It can lead to serious embolic complications including death1 or may be found incidentally at autopsy. Most of these lesions are single but in 6.8% to 7.5% of the cases, multiple CPF are detected.2-5 Because it is completely curable by surgical resection with no reported cases of recurrence, it is important to identify all lesions before cardiac operation. Only 3 cases of multiple aortic CPF have been reported in the English-language literature; in all, the diagnosis of multiple lesions was made only during operation.6 This is the first reported case of the presurgical diagnosis of multiple CPF of the aortic valve.
CASE REPORT A 75-year-old man with a medical history of smoking, emphysema, adult-onset diabetes mellitus, hyperlipidemia, and systemic hypertension was evaluated for an episode of diaphoresis. Physical examination was unremarkable. Specifically, blood pressure was 130/75 mm Hg and he had no cardiac murmurs, abnormal heart sounds, or signs of peripheral embolization. Laboratory results showed normal blood count and sedimentation rate. Kidney and liver tests were within normal range. A stress echocardiogram was negative for ischemia, however, it revealed an indistinct mass involving the aortic valve. A From the Division of Cardiology, Anatomic Pathology, and Division of Cardiothoracic Surgery (A.T.), Cedars-Sinai Medical Center. Reprint requests: Robert J. Siegel, MD, FACC, Cardiac Noninvasive Laboratory, Room No. 5335, Cedars-Sinai Medical Center, Los Angeles, CA 90048 (E-mail: [email protected]). Copyright 2003 by the American Society of Echocardiography. 0894-7317/2003/$30.00 ⫹ 0 doi:10.1016/S0894-7317(03)00117-2
494
case of a patient with multiple aortic valve papillary fibroelastomas diagnosed before operation. The patient underwent surgical removal of the 3 masses that were confirmed as cardiac papillary fibroelastomas by pathologic examination. There was no evidence of aortic insufficiency after operation. (J Am Soc Echocardiogr 2003;16:494-6.)
transesophageal echocardiogram (TEE) was performed and 3 echo densities were found on the aortic valve cusps. The masses measured 14 ⫻ 15 mm on the noncoronary cusp, 8 ⫻ 9 mm on the right coronary cusp lesion, and 2 ⫻ 2 mm on the left coronary cusp. As shown in Figure 1, all 3 lesions were on the ventricular aspect of the aortic valve. Additional findings included moderate mitral regurgitation, an atrial septal aneurysm, and moderate atheromatous plaquing of the descending thoracic aorta. Subsequently 3 sets of blood cultures were obtained to exclude infective endocarditis and were negative. A diagnosis of CPF of the aortic valve was made and an operation was recommended to resect the lesions as a result of their potential for embolization. At operation, by direct inspection of the aortic valve through an aortotomy as demonstrated in Figure 2, the largest lesion was a mobile, gelatinous mass attached to a short stalk on the free edge of the noncoronary cusp; a second mass was on the right coronary cusp; and the smallest lesion was on the left coronary cusp. All the lesions were excised and the aortic valve was preserved. The patient tolerated the procedure well with no residual valvular regurgitation found on intraoperative TEE. Light microscopic assessment (Figure 3) using hematoxylin and eosin sections demonstrated lesions characterized by papillary architecture, having elongated, narrow stalks with central cores of dense fibrous connective tissue devoid of blood vessels. Extracellular matrix was composed of collagen, myxoid mucopolysaccharides, and elastic fibers (elastin-van Gieson’s stain). The surface was partially lined by attenuated endothelium.
DISCUSSION CPF is an uncommon benign cardiac tumor. It accounts for up to 16% of cardiac tumors in pathologic studies,2 but only 1% in surgical series.3 It is the most common tumor of cardiac valves. CPF is
Journal of the American Society of Echocardiography Volume 16 Number 5
Neuman et al 495
Figure 1 Transesopageal echocardiographic short-axis view of aortic valve. A, Systolic view demonstrating large noncoronary cusp (NCC) papillary fibroelastoma. B, Diastolic view demonstrating both NCC and right coronary cusp (RCC) masses, and partial visualization of small left coronary cusp (LCC) lesion.
most commonly located on the aortic valve cusps (44.5%) and on the mitral valve leaflets (36.4%).4 In more then 90% of reported cases it was found to be a solitary lesion, however, in 7% to 13% of cases, lesions were found in more than 1 site (ie, on more than 1 valve or on a valve and on the ventricular endocardium).4,5 However, multiple lesions on the same valve are extremely rare with only 3 cases reported in the English-language literature for multiple lesions on the aortic valve.6 None of those CPF were diagnosed as multiple lesions before operation as the concomitant tumors were small (2 mm). In our case, the 3 lesions were identified by TEE. These findings were confirmed by direct visualization during operation. Because CPF can be small, they can easily be missed by TEE. This emphasizes the importance of careful direct visual examination of the valve during operation and optimal visualization of the other valves by TEE to reduce the likelihood of missing lesions on the same valve, other valves, or other cardiac structures. Sun et al4 have suggested that the presence of large (⬎1.0 cm) mobile CPF on the aortic or mitral valve may be an indication for prophylactic operation in the patient who is asymptomatic. A second issue of major clinical importance is the evaluation of concomitant ischemic heart disease before operation. Because the mean age of these patients is about 60 years old4 and many, as in our case, have risk factors for ischemic heart disease, information regarding possible coronary artery disease and, hence, a need for bypass operation as well, is important. However, because of the friability of these aortic valve lesions and the potential for embolization,7 it is probably better to avoid catheter manipulation near the aortic valve and the coronary artery ostia. Therefore, noninvasive modalities for evaluation of ischemia should be considered. In our case we preformed a stress thallium scan that did not show any evidence of inducible ischemia. The exact histogenesis of the papillary fibroelastoma is uncertain. Despite classification by many as
Figure 2 Intraoperative findings. Surgical view through aortotomy demonstrates 2 larger masses on noncoronary cusp (NCC) and right coronary cusp (RCC), and smaller lesion on left coronary cusp (LCC). Asterisk marks right coronary artery ostium.
Figure 3 A, Gross specimen of 3 masses demonstrating characteristic gelatinous appearance of papillary fibroelastoma. B, Hematoxylin and eosin sections of masses revealing typical papillary architecture of cardiac papillary fibroelastoma, composed of central cores of avascular fibrous connective tissue, in combination with myxoid extracellular matrix and partial lining of endothelial cells on surface (original magnification ⫻100). Inset demonstrates higher magnification (original magnification ⫻20). LCC, Left coronary cusp; NCC, noncoronary cusp; RCC, right coronary cusp.
a neoplasm, it may represent exuberant organization of thrombi, in many ways similar to the much smaller Lambl’s excrescences, which are frequently found on the aortic valve of older individuals.8
496 Neuman et al
Colocalization of Lambl’s excrescences and papillary fibroelastomas on the same valve suggests a common histogenesis.6 A virus-induced tumor hypothesis has also been suggested on the basis of finding dendritic cells and cytomegalovirus virus remnants in the intermediate layer of the tumor.9 Such a mechanism could also explain multicentricity. In summary, our report is the first documented case of the presurgical diagnosis of multiple aortic valve papillary fibroelastomas.
REFERENCES 1. Takada A, Saito K, Ro A, Tokudome S, Murai T. Papillary fibroelastoma of the aortic valve: a sudden death case of coronary embolism with myocardial infarction. Forensic Sci Int 2000;113:209-14. 2. Goldhaber S, Braunwald E. Primary cardiac tumors. In: Atlas of heart diseases. Philadelphia: Current Medicine; 1995. p. 15.115.22.
Journal of the American Society of Echocardiography May 2003
3. Molina JE, Edwards JE, Ward HB. Primary cardiac tumors: experience at the University of Minnesota. Thorac Cardiovasc Surg 1990;38:183-91. 4. Sun JP, Asher CR, Yang XS, Cheng GG, Scalia GM, Massed AG, et al. Clinical and echocardiographic characteristics of papillary fibroelastoma: a retrospective and prospective study in 162 patients. Circulation 2001;103:2687-93. 5. Grinda JM, Couetil JP, Chauvaud S, D’Attelis N, Berrebi A, Fabiani JN, et al. Cardiac valve papillary fibroelastoma: surgical excision for revealed or potential embolization. J Thorac Cardiovasc Surg 1999;17:106-10. 6. Touati GD, Carmi D, Sevestre H, Poulain H. Multiple aortic valve papillary fibroelastoma: do not miss the other one [letter]. Eur J Cardiothorac Surg 2002;21:596-7. 7. Valente M, Basso C, Thiene G, Bressan M, Stritoni P, Cocco P, et al. Fibroelastic papilloma: a not-so-benign cardiac tumor. Cardiovasc Pathol 1992;1:161-6. 8. Schen FJ. The heart. In: Cotran RS, Kumar V, Collins T, editors. Robbins pathologic basis of disease. 6th ed. Philadelphia: W.B. Saunders; 1999. p. 590. 9. Grandmougin D, Fayad G, Moukassa D, Decoence C, Abolmaali K, Bodart JC, et al. Cardiac valve papillary fibroelastomas: clinical, histological and immunohistochemical studies and a physiopathogenic hypothesis. J Heart Valve Dis 2000;9:832-41.