Case Study
Papillary fibroelastoma of tricuspid valve presenting as pulmonary embolism
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(7) 858–860 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314526601 aan.sagepub.com
Ruchit Ashok Shah1, Latchumanadhas Kalidoss1, Anbarasu Mohanraj2, Anitha Lakshmanan1, Muniraj Kanchanamala Thirumurthi3 and Mullasari S Ajit1
Abstract A 65-year-old gentleman was admitted with recurrent dyspnea. Two-dimensional and transesophageal echocardiography revealed a highly mobile echogenic mass attached to the tricuspid valve. A 99mTc lung perfusion scan was suggestive of pulmonary embolism. The patient underwent surgical resection of the mass. Histopathological examination revealed a papillary fibroelastoma.
Keywords Fibroma, Heart neoplasms, Pulmonary embolism, Tricuspid valve
Introduction Papillary fibroelastoma is a rare primary cardiac neoplasm. It is usually asymptomatic. Rarely, a primary cardiac tumor may be a cause of pulmonary embolism. Herein we report a case of recurrent dyspnea presenting as pulmonary embolism due to papillary fibroelastoma of the tricuspid valve. We discuss the morphology, histopathology, clinical features, and surgical treatment of papillary fibroelastoma.
Case report A 65-year-old gentleman was admitted with repeated episodes of breathlessness. His pulse was 100 beats per minute and blood pressure was 150/90 mm Hg. Auscultation findings were unremarkable. An electrocardiogram showed sinus tachycardia. An echocardiogram and transesophageal echocardiography showed a highly mobile echogenic mass attached by a small pedicle to the tricuspid valve. There was mild tricuspid regurgitation and severe pulmonary artery hypertension with good biventricular function (Figure 1). A 99m Tc lung perfusion scan showed a wedge-shaped cold area in the left lower lobe of the lung, suggestive of pulmonary embolism. Serial blood cultures were sterile. A D-dimer and lower limb venous Doppler study was normal. A coronary angiogram revealed
normal epicardial coronary arteries. On the basis of the echocardiography findings and the 99mTc lung perfusion scan, we suspected fragments of the tricuspid valve mass as the probable cause of pulmonary embolism. The patient underwent excision of the tricuspid valve mass via a minimally invasive limited-exposure procedure through a right anterior thoracotomy. A grayish polypoidal glistening tissue measuring 10 10 5 mm was resected from the anterior tricuspid valve leaflet, along with 5 mm of normal leaflet. The resultant defect was repaired with an autologous pericardial patch. The other leaflets looked normal. The patient had mild tricuspid regurgitation at the end of the procedure; hence annuloplasty was not performed. Histopathological examination showed polypoidal tissue covered by endothelial cells containing multiple 1 Department of Cardiology, Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India 2 Department of Cardiac Surgery, Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India 3 Department of Pathology Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India
Corresponding author: Ruchit Ashok Shah, Institute of Cardiovascular Diseases, Madras Medical Mission, 4A JJ Nagar, Mogappair, Chennai 600 037, India. Email: [email protected]
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filiform processes, with a central core of acellular collagen surrounded by myxomatous stromal tissue. The peripheral stroma showed coarse fragmented elastic fibrils. No mitotic activity was seen (Figure 2). Thus a diagnosis of papillary fibroelastoma of the tricuspid valve was made. The postoperative course was uneventful and a 12-month follow-up with echocardiographic examination showed no recurrence of the mass, mild
Figure 1. Transesophageal echocardiography showing a mass attached to the anterior leaflet of the tricuspid valve.
tricuspid regurgitation, and mild pulmonary artery hypertension.
Discussion The incidence of papillary fibroelastoma was 0.33% in an autopsy series,1 and it is the third most common benign primary cardiac tumor. The exact etiology is unknown. Multiple theories suggest that it may be due to mechanical trauma in chronic rheumatic heart disease, a neoplasm, hamartoma, or inflammatory nodules.2 Grossly, the tumor has a frond-like appearance and resembles a sea anemone. It is soft, white, and friable, with or without thrombus.1,3 It is usually single (91.4%) and mobile, with a stalk of 1–3 mm (43.6%) and a mean size of 9 4.6 mm. Most (82.7%) are located on valves (aortic valve 44.5%, mitral valve 36.4%, pulmonary valve 1.8%), followed by the left ventricle, right ventricle, and left atrium.3 Rare cases of tricuspid valve involvement have been reported,4 as in our patient. Histopathological examination of the tumor reveals a central stalk with multiple radiating avascular papillary villous fronds. The frond surface is covered with a hyperplastic layer of endothelial cells.
Figure 2. (A) Hematoxylin and eosin stain (original magnification 40), (B) Verhoeff’s elastic stain (original magnification 100), (C) Masson’s trichrome stain (original magnification 200), and (D) Alcian blue stain (original magnification 100) showing polypoidal tissue covered by endothelial cells, containing multiple filiform processes with a central core of acellular collagen surrounded by myxomatous stromal tissue. The peripheral stroma shows coarse fragmented elastic fibrils. No mitotic activity is seen.
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Asian Cardiovascular & Thoracic Annals 23(7)
The frond matrix contains mucopolysaccharides, collagen, elastic fibers, and smooth muscle cells.2–6 The mean age at presentation is 60 16 years.3 Most patients are clinically asymptomatic.6 Patients with left-sided valve tumors may present with transient ischemic attack, stroke, or myocardial infarction. Patients with right-sided tumors may present with pulmonary embolism and dyspnea.1,3,4,6,7 Fibrin or thrombus from the papillary frond can embolise,8 as in our case. Transthoracic echocardiography has a sensitivity and specificity of 88.9% and 87.8% for a size 50.2 cm.3 Transesophageal echocardiography is important to detect the extent and site of the tumor attachment. It is seen as round, oval, or irregular, with a speckled appearance and stippling around the border. Computed tomography and cardiac magnetic resonance imaging with contrast (delayed enhancement) can also be used to diagnose this lesion.5 Asymptomatic patients with small left-sided nonmobile lesions can be followed up. Larger (>1 cm) and mobile papillary fibroelastomas require surgical excision.3 Surgical resection of benign cardiac tumors is safe and curative using a minimally invasive right thoracotomy approach, and it has an excellent prognosis with no reported recurrence.3,9 No data are available regarding the use of systemic anticoagulation for prevention of embolic complications. Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Howard RA, Aldea GS, Shapira OM, Kasznica JM and Davidoff R. Papillary fibroelastoma: increasing recognition of a surgical disease. Ann Thorac Surg 1999; 68: 1881–1885. 2. McAllistar HA Jr and Fenoglio JJ Jr. Tumors of the cardiovascular system. Atlas of pathology, Fascicle 15, second series. Washington, DC: Armed Forces Institute of Pathology, 1978, pp. 1–25. 3. Sun JP, Asher CR, Yang XS, et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: A retrospective and prospective study in 162 patients. Circulation 2001; 103: 2687. 4. Neerukonda SK, Jantz RD, Vijay NK, Narrod JA and Schoonmaker FW. Pulmonary embolization of papillary fibroelastoma arising from the tricuspid valve. Tex Heart Inst J 1991; 18: 132–135. 5. Sydow K, Willems S, Reichenspurner H, et al. Papillary fibroelastomas of the heart. Thorac Cardiovasc Surg 2008; 56: 9–13. 6. Edwards FH, Hale D, Cohen A, Thompson L, Pezzella AT and Virmani R. Primary cardiac valve tumors. Ann Thorac Surg 1991; 52: 1127–1231. 7. Boone S, Higginson LA and Walley VM. Endothelial papillary fibroelastomas arising in and around the aortic sinus, filling the ostium of the right coronary artery. Arch Pathol Lab Med 1992; 116: 135–137. 8. Roberts WC. Papillary fibroelastomas of the heart. Am J Cardiol 1997; 80: 973–975. 9. Bossert T, Gummert JF, Battellini R, et al. Surgical experience with 77 primary cardiac tumors. Interact Cardiovasc Thorac Surg 2005; 4: 311–315.
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Papillary fibroelastoma of tricuspid valve presenting as pulmonary embolism
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(7) 858–860 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314526601 aan.sagepub.com
Ruchit Ashok Shah1, Latchumanadhas Kalidoss1, Anbarasu Mohanraj2, Anitha Lakshmanan1, Muniraj Kanchanamala Thirumurthi3 and Mullasari S Ajit1
Abstract A 65-year-old gentleman was admitted with recurrent dyspnea. Two-dimensional and transesophageal echocardiography revealed a highly mobile echogenic mass attached to the tricuspid valve. A 99mTc lung perfusion scan was suggestive of pulmonary embolism. The patient underwent surgical resection of the mass. Histopathological examination revealed a papillary fibroelastoma.
Keywords Fibroma, Heart neoplasms, Pulmonary embolism, Tricuspid valve
Introduction Papillary fibroelastoma is a rare primary cardiac neoplasm. It is usually asymptomatic. Rarely, a primary cardiac tumor may be a cause of pulmonary embolism. Herein we report a case of recurrent dyspnea presenting as pulmonary embolism due to papillary fibroelastoma of the tricuspid valve. We discuss the morphology, histopathology, clinical features, and surgical treatment of papillary fibroelastoma.
Case report A 65-year-old gentleman was admitted with repeated episodes of breathlessness. His pulse was 100 beats per minute and blood pressure was 150/90 mm Hg. Auscultation findings were unremarkable. An electrocardiogram showed sinus tachycardia. An echocardiogram and transesophageal echocardiography showed a highly mobile echogenic mass attached by a small pedicle to the tricuspid valve. There was mild tricuspid regurgitation and severe pulmonary artery hypertension with good biventricular function (Figure 1). A 99m Tc lung perfusion scan showed a wedge-shaped cold area in the left lower lobe of the lung, suggestive of pulmonary embolism. Serial blood cultures were sterile. A D-dimer and lower limb venous Doppler study was normal. A coronary angiogram revealed
normal epicardial coronary arteries. On the basis of the echocardiography findings and the 99mTc lung perfusion scan, we suspected fragments of the tricuspid valve mass as the probable cause of pulmonary embolism. The patient underwent excision of the tricuspid valve mass via a minimally invasive limited-exposure procedure through a right anterior thoracotomy. A grayish polypoidal glistening tissue measuring 10 10 5 mm was resected from the anterior tricuspid valve leaflet, along with 5 mm of normal leaflet. The resultant defect was repaired with an autologous pericardial patch. The other leaflets looked normal. The patient had mild tricuspid regurgitation at the end of the procedure; hence annuloplasty was not performed. Histopathological examination showed polypoidal tissue covered by endothelial cells containing multiple 1 Department of Cardiology, Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India 2 Department of Cardiac Surgery, Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India 3 Department of Pathology Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India
Corresponding author: Ruchit Ashok Shah, Institute of Cardiovascular Diseases, Madras Medical Mission, 4A JJ Nagar, Mogappair, Chennai 600 037, India. Email: [email protected]
Downloaded from aan.sagepub.com by guest on November 16, 2015
Shah et al.
859
filiform processes, with a central core of acellular collagen surrounded by myxomatous stromal tissue. The peripheral stroma showed coarse fragmented elastic fibrils. No mitotic activity was seen (Figure 2). Thus a diagnosis of papillary fibroelastoma of the tricuspid valve was made. The postoperative course was uneventful and a 12-month follow-up with echocardiographic examination showed no recurrence of the mass, mild
Figure 1. Transesophageal echocardiography showing a mass attached to the anterior leaflet of the tricuspid valve.
tricuspid regurgitation, and mild pulmonary artery hypertension.
Discussion The incidence of papillary fibroelastoma was 0.33% in an autopsy series,1 and it is the third most common benign primary cardiac tumor. The exact etiology is unknown. Multiple theories suggest that it may be due to mechanical trauma in chronic rheumatic heart disease, a neoplasm, hamartoma, or inflammatory nodules.2 Grossly, the tumor has a frond-like appearance and resembles a sea anemone. It is soft, white, and friable, with or without thrombus.1,3 It is usually single (91.4%) and mobile, with a stalk of 1–3 mm (43.6%) and a mean size of 9 4.6 mm. Most (82.7%) are located on valves (aortic valve 44.5%, mitral valve 36.4%, pulmonary valve 1.8%), followed by the left ventricle, right ventricle, and left atrium.3 Rare cases of tricuspid valve involvement have been reported,4 as in our patient. Histopathological examination of the tumor reveals a central stalk with multiple radiating avascular papillary villous fronds. The frond surface is covered with a hyperplastic layer of endothelial cells.
Figure 2. (A) Hematoxylin and eosin stain (original magnification 40), (B) Verhoeff’s elastic stain (original magnification 100), (C) Masson’s trichrome stain (original magnification 200), and (D) Alcian blue stain (original magnification 100) showing polypoidal tissue covered by endothelial cells, containing multiple filiform processes with a central core of acellular collagen surrounded by myxomatous stromal tissue. The peripheral stroma shows coarse fragmented elastic fibrils. No mitotic activity is seen.
Downloaded from aan.sagepub.com by guest on November 16, 2015
860
Asian Cardiovascular & Thoracic Annals 23(7)
The frond matrix contains mucopolysaccharides, collagen, elastic fibers, and smooth muscle cells.2–6 The mean age at presentation is 60 16 years.3 Most patients are clinically asymptomatic.6 Patients with left-sided valve tumors may present with transient ischemic attack, stroke, or myocardial infarction. Patients with right-sided tumors may present with pulmonary embolism and dyspnea.1,3,4,6,7 Fibrin or thrombus from the papillary frond can embolise,8 as in our case. Transthoracic echocardiography has a sensitivity and specificity of 88.9% and 87.8% for a size 50.2 cm.3 Transesophageal echocardiography is important to detect the extent and site of the tumor attachment. It is seen as round, oval, or irregular, with a speckled appearance and stippling around the border. Computed tomography and cardiac magnetic resonance imaging with contrast (delayed enhancement) can also be used to diagnose this lesion.5 Asymptomatic patients with small left-sided nonmobile lesions can be followed up. Larger (>1 cm) and mobile papillary fibroelastomas require surgical excision.3 Surgical resection of benign cardiac tumors is safe and curative using a minimally invasive right thoracotomy approach, and it has an excellent prognosis with no reported recurrence.3,9 No data are available regarding the use of systemic anticoagulation for prevention of embolic complications. Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Howard RA, Aldea GS, Shapira OM, Kasznica JM and Davidoff R. Papillary fibroelastoma: increasing recognition of a surgical disease. Ann Thorac Surg 1999; 68: 1881–1885. 2. McAllistar HA Jr and Fenoglio JJ Jr. Tumors of the cardiovascular system. Atlas of pathology, Fascicle 15, second series. Washington, DC: Armed Forces Institute of Pathology, 1978, pp. 1–25. 3. Sun JP, Asher CR, Yang XS, et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: A retrospective and prospective study in 162 patients. Circulation 2001; 103: 2687. 4. Neerukonda SK, Jantz RD, Vijay NK, Narrod JA and Schoonmaker FW. Pulmonary embolization of papillary fibroelastoma arising from the tricuspid valve. Tex Heart Inst J 1991; 18: 132–135. 5. Sydow K, Willems S, Reichenspurner H, et al. Papillary fibroelastomas of the heart. Thorac Cardiovasc Surg 2008; 56: 9–13. 6. Edwards FH, Hale D, Cohen A, Thompson L, Pezzella AT and Virmani R. Primary cardiac valve tumors. Ann Thorac Surg 1991; 52: 1127–1231. 7. Boone S, Higginson LA and Walley VM. Endothelial papillary fibroelastomas arising in and around the aortic sinus, filling the ostium of the right coronary artery. Arch Pathol Lab Med 1992; 116: 135–137. 8. Roberts WC. Papillary fibroelastomas of the heart. Am J Cardiol 1997; 80: 973–975. 9. Bossert T, Gummert JF, Battellini R, et al. Surgical experience with 77 primary cardiac tumors. Interact Cardiovasc Thorac Surg 2005; 4: 311–315.
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