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Background
Fibroelastomas, also known as papillary fibroelastomas, are rare tumors occurring in the heart. Although benign, they may come to clincial attention because of obstructive symptoms on heart valves, or by breaking off and forming emboli which may lead to strokes.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Papillary fibroelastoma
G iant Lambl excrescenceINCIDENCE Third most frequent benign tumor of the heart AGE RANGE-MEDIAN Mean 60 years About equal
DISEASE ASSOCIATIONS CHARACTERIZATION MYOCARDIAL INFARCTION
Recurrent Myocardial Infarction Due to Obstruction of the RCA Ostium by an Aortic Papillary Fibroelastoma.Boulmier D, Verhoye JP.
Departement de Cardiologie et Maladies Vasculaires, Centre Cardiopneumologique (Niveau 0), Centre Hospitalier Universitaire de Rennes, 2, rue Henri Le Guilloux, 35033, RENNES CEDEX 9, France.
J Invasive Cardiol 2002 Nov;14(11):686-8 Abstract quote A 69-year-old healthy woman was hospitalized twice within a few weeks for management of acute inferior myocardial infarction with thrombolysis and stenting of the right coronary artery (RCA) ostium.
After the second myocardial infarction, follow-up angiography showed subocclusion of the proximal RCA, and transesophageal echocardiography and chest computed tomography revealed the presence of a mobile, 2-cm diameter spherical mass partially adherent to the RCA ostium and penetrating the stent.
A benign papillary fibroelastoma was surgically excised, the stent was removed, and vein bypass grafting of the RCA was performed. The literature relevant to this case report is reviewed.
STROKE
Embolic stroke and cardiac papillary fibroelastoma.Shing M, Rubenson DS.
Department of Internal Medicine, Scripps Clinic, La Jolla, California 92037, USA.
Clin Cardiol 2001 Apr;24(4):346-7 Abstract quote Papillary fibroelastomas are rare, benign, primary cardiac tumors. They are, however, the most common primary tumor of the cardiac valves and may cause great morbidity risk from embolization.
This paper reports the case of a healthy 34-year-old man who presented with symptoms of a right occipital embolic stroke. Transesophageal echocardiography revealed a papillary fibroelastoma on the anterior leaflet of the mitral valve. The papillary fibroelastoma was surgically excised and he has had no recurrent symptoms.
This case illustrates the importance of obtaining a transesophageal echocardiogram to investigate a possible embolic source in patients with ischemic stroke.
Cardiac papillary fibroelastoma: aA rare cause of ischemic stroke in the young.Giannesini C, Kubis N, N'Guyen A, Wassef M, Mikol J, Woimant F.
Department of Neurology, Hopital Lariboisiere, Paris, France
Cerebrovasc Dis 1999 Jan-Feb;9(1):45-9 Abstract quote Among etiologies of stroke in young adults, primary cardiac tumors are very rare.
We report the case of a 37-year-old woman who was admitted for an ischemic stroke in the right middle cerebral artery region. Etiologic investigations revealed, after transthoracic and transesophageal echocardiography, an aortic valve tumor. Treatment was first medical with anticoagulation, then surgical.
Histological examination showed a papillary fibroelastoma. After a review of the literature, the possible mechanisms of the ischemic event are discussed and lead to the conclusion that this tumor must be surgically excised, even if asymptomatic, because of recurrent ischemic complications responsible for myocardial infarction, stroke and sudden death.
PATHOGENESIS CHARACTERIZATION VIRAL
Cardiac valve papillary fibroelastomas: clinical, histological and immunohistochemical studies and a physiopathogenic hypothesis.Grandmougin D, Fayad G, Moukassa D, Decoene C, Abolmaali K, Bodart JC, Limousin M, Warembourg H.
Department of Cardiovascular Surgery, H pital Cardiologique, CHU-Lille, France.
J Heart Valve Dis 2000 Nov;9(6):832-41 Abstract quote BACKGROUND AND AIM OF THE STUDY: Cardiac papillary fibroelastoma (CPF) is a rare and histologically benign tumor, but may have a malignant propensity for life-threatening complications; thus, surgical removal is justified. Case histories were reviewed of four patients who underwent surgical management after diagnosis of CPF located on aortic (n = 2) or mitral (n = 2) valves. Our aim was to provide explanations for the clinical diversity of the lesions and, using histological and immunohistochemical methods, to hypothesize the genesis of these tumors.
METHODS: Among four patients with a diagnosis of valvular CPF, two had previous and recent history of neurological embolic symptoms with small echographically located tumors attached to the ventricular side of aortic cusps. Two other patients (one with paroxysmal atrial fibrillation, one with no neurological or rhythmically related stroke) had CPFs located on the posterior or anterior mitral leaflets.
RESULTS: Surgical excision was performed with a conservative valve-sparing approach. Histological and specific immunohistochemical (IHC) studies were conducted on all samples. The postoperative course was uneventful, and histological analysis confirmed the diagnosis of CPF with typical fronds characterized by three successive layers. In the first two patients there was correlation between neurological events and the presence of thrombus aggregated on the injured superficial endothelial layer. In the other patients, no endothelial damage or thrombus was found. IHC studies showed dysfunction of the superficial endothelium, a centrifugal mesenchymal cellular migration arising from the central layer to the superficial layer with differentiation steps, the presence of dendritic cells in the intermediate layer, and remnants of cytomegalovirus (CMV) in the intermediate layer.
CONCLUSION: Despite their benign histological aspect, and independent of their size, CPFs justify surgical excision because of their high potential to systemic embolization. In most cases, valve sparing management is possible with no observed recurrence after complete excision. The presence of dendritic cells and CMV strongly suggests the possibility of a virus-induced tumor, therefore evoking the concept of a chronic form of viral endocarditis.
LABORATORY/RADIOLOGIC/
OTHER TESTSCHARACTERIZATION RADIOLOGIC
Evolution of a papillary fibroelastoma.Malik MF, Sagar K, Wynsen JC, Kenny D.
Zablocki Veterans Affairs, Medical Center, Milwaukee, WI 53226, USA.
J Am Soc Echocardiogr 1998 Jan;11(1):92-4 Abstract quote Papillary fibroelastoma is a rare primary tumor of the heart usually found incidentally at autopsy. Little is known about the natural history of this tumor, but an aggressive surgical approach is recommended because of the high incidence of embolization.
We describe a patient whose tumor was found during transthoracic echocardiography and who had had a normal echo 10 years previously. This finding suggests that papillary fibroelastoma may be an acquired rather than a congenital lesion.
Two-dimensional echocardiographic detection and diagnostic features of tricuspid papillary fibroelastoma.Frumin H, O'Donnell L, Kerin NZ, Levine F, Nathan LE Jr, Klein SP
J Am Coll Cardiol 1983 Nov;2(5):1016-8 Abstract quote Cardiac papillary fibroelastomas are rare and benign primary tumors in the cardiac valves or occasionally the mural endocardium. Before 1977, these tumors were diagnosed exclusively at postmortem examination. Over the last few years, a handful of cases have been diagnosed in vivo by echocardiography.
In this report, we describe the first tricuspid valve papillary fibroelastoma detected by echocardiography in an adult. Clinical and echocardiographic features are discussed.
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL
Iatrogenic cardiac papillary fibroelastoma: A study of 12 cases (1990 to 2000).Kurup AN, Tazelaar HD, Edwards WD, Burke AP, Virmani R, Klarich KW, Orszulak TA.
Mayo Medical School and the Divisions of Anatomic Pathology, Cardiovascular Disease, and Cardiovascular Surgery, Mayo Clinic, Rochester, MN, and the Armed Forces Institute of Pathology, Washington, DC.
Hum Pathol 2002 Dec;33(12):1165-9 Abstract quote Cardiac papillary fibroelastoma (PFE) is a rare, benign, slow-growing tumor of the endocardium. Whether it represents a reactive tumoral lesion or a true neoplasm remains a matter of debate. However, an anecdotal association of PFEs with previous cardiac surgery has been reported.
The current study was undertaken to determine the frequency and nature of iatrogenic events associated with PFEs and to provide a comprehensive review of the topic. The study group comprised 12 cases seen between 1990 and 2000, with specimens from 7 women and 5 men. Six developed postoperatively and 6 developed after thoracic irradiation. The 9 Mayo cases represented 18% of all surgically excised PFEs during the study period. The mean age at operation was 54 years (range, 29 to 79 years). The mean interval between the iatrogenic event and excision of the tumor was 18 years (range, 9 to 31 years).
In 58% of cases, the presence of multiple tumors was either confirmed pathologically (41.7%) or strongly suggested by echocardiography (16.6%).
Among patients who had undergone previous cardiac surgery, PFEs were found in the chamber closest to the procedure. Similarly, in patients who had received radiation therapy, tumors developed in the left atrium, in the right ventricle and atrium, and on the tricuspid valve within the radiation field.
In conclusion, iatrogenic PFEs may be relatively common among all such tumors, are frequently multiple, and often involve nonvalvular endocardial surfaces.
Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients.Sun JP, Asher CR, Yang XS, Cheng GG, Scalia GM, Massed AG, Griffin BP, Ratliff NB, Stewart WJ, Thomas JD.
Cardiovascular Imaging Center, Department of Cardiology, the Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA.
Circulation 2001 Jun 5;103(22):2687-93 Abstract quote BACKGROUND: Cardiac papillary fibroelastoma (CPF) is a primary cardiac neoplasm that is increasingly detected by echocardiography. The clinical manifestations of this entity are not well described.
METHODS AND RESULTS: In a 16-year period, we identified patients with CPF from our pathology and echocardiography databases. A total of 162 patients had pathologically confirmed CPF. Echocardiography was performed in 141 patients with 158 CPFs, and 48 patients had CPFs that were not visible by echocardiography (<0.2 cm), leaving an echocardiographic subgroup of 93 patients with 110 CPFs. An additional 45 patients with a presumed diagnosis of CPF were identified. The mean age of the patients was 60+/-16 years of age, and 46.1% were male. Echocardiographically, the mean size of the CPFs was 9+/-4.6 mm; 82.7% occurred on valves (aortic more than mitral), 43.6% were mobile, and 91.4% were single. During a follow-up period of 11+/-22 months, 23 of 26 patients with a prospective diagnosis of CPF that was confirmed by pathological examination had symptoms that could be attributable to embolization. In the group of 45 patients with a presumed diagnosis of CPF, 3 patients had symptoms that were likely due to embolization (incidence, 6.6%) during a follow-up period of 552+/-706 days.
CONCLUSIONS: CPFs are generally small and single, occur most often on valvular surfaces, and may be mobile, resulting in embolization. Because of the potential for embolic events, symptomatic patients, patients undergoing cardiac surgery for other lesions, and those with highly mobile and large CPFs should be considered for surgical excision.
Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathologic correlation.Klarich KW, Enriquez-Sarano M, Gura GM, Edwards WD, Tajik AJ, Seward JB.
Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
J Am Coll Cardiol 1997 Sep;30(3):784-90 Abstract quote OBJECTIVES: We sought to determine the clinical and echocardiographic characteristics of papillary fibroelastoma (PFE).
BACKGROUND: PFE is a rarely encountered cardiac tumor about which relatively little is known.
METHODS: Institutional records were reviewed for the years 1980 to 1995 for patients with pathologic or echocardiographic diagnosis of PFE. Group 1 included 17 patients with the pathologic diagnosis of PFE who also underwent echocardiography. Echocardiographic features of PFE were established in group 1. Group 2 included 37 patients with only echocardiographic evidence of PFE.
RESULTS: In group 1, 7 (41.2%) of 17 patients had symptoms related to PFE. Neurologic events occurred in 5 (29.4%) of 17 patients. All patients had the tumor surgically removed. During follow-up, no new embolic events occurred. Echocardiographic characteristics of PFE included a small tumor (12.1 +/- 6.5 x 9.0 +/- 4.3 mm), usually pedunculated (14 [94%] of 17 patients) and mobile, with a homogeneous speckled pattern and a characteristic stippling along the edges. PFEs were most common on valvular surfaces (12 [60%] of 20 PFEs) but were not uncommon on other endocardial surfaces (8 [40%] of 20 PFEs). The tumor did not cause valvular dysfunction. In group 2, 16 (43%) of 37 patients were asymptomatic. Five patients (13.5%) had a previous neurologic event. During follow-up (mean 31 months, range 1 to 77), nine neurologic events occurred.
CONCLUSIONS: PFEs are associated with embolism, can be diagnosed with echocardiography, are often an incidental clinical finding and do not cause valvular dysfunction.
Papillary fibroelastoma of the heart. Report of six cases.Almagro UA, Perry LS, Choi H, Pintar K.
Arch Pathol Lab Med 1982 Jul;106(7):318-21 Abstract quote Papillary fibroelastoma is a rare cardiac lesion, mostly encountered as an incidental finding at the time of autopsy but occasionally seen during life. Although this condition generally is asymptomatic, a few cases had been associated with clinical problems, such as embolic phenomena, angina pectoris, outflow tract obstruction, and sudden death.
We studied six examples of this lesion, five occurring on the cardiac valves and one on the left ventricular septum. In one, the lesion was detected by echocardiography, which represents to our knowledge only the third published case in which the lesion was visualized by this diagnostic procedure and only the fourth in which the lesion was seen during life.
We believe that echocardiography is sensitive in the detection of this often small, intracardiac lesion, useful in evaluating whether a case, by virtue of its size or location, carries a significant risk of causing clinical problems and therefore requires treatment.
VARIANTS AORTIC VALVE
Aortic valve papillary fibroelastoma.Alawi A, Kassabian EB, Ashoush R, Jebara VA.
Department of Cardiovascular and Thoracic Surgery, Hotel Dieu de France, Rue Adib Ishac, Beirut, Lebanon.
Cardiovasc Surg 2002 Feb;10(1):65-7 Abstract quote Cardiac papillary fibroelastomas are rare tumors of the heart and affect primarily the cardiac valves. These lesions are responsible for embolic accidents that clinically manifest as neurological and cardiovascular symptoms.
We describe the case of a 40-yr-old woman who presented with a neurological deficit caused by a papillary tumor of the aortic valve. Diagnosis was made by transesophageal echocardiaphy and the tumor was surgically removed.
ATRIUM, LEFT
Unusually located left atrial papillary fibroelastoma with probable embolization to the spinal cord.Friedman GR, Pappas TW, Colangelo R, Tunick PA, Freedberg RS, Scholes JV, Kronzon I.
NYU School of Medicine, 560 First Ave., New York, NY 10016, USA.
Echocardiography 2001 Aug;18(6):523-6 Abstract quote This report presents a patient with flaccid paraplegia, most probably secondary to embolization from a left atrial papillary fibroelastoma.
The unique location of the tumor, attached to the ridge in the left atrium between the left atrial appendage and pulmonary vein, was well documented on transesophageal echocardiography, and the diagnosis was confirmed by histopathology.
Although benign, these tumors may develop in all four cardiac chambers and result in pulmonary or systemic embolization. Paraplegia is a rare embolic complication.
ATRIUM, RIGHT Giant papillary fibroelastoma of the right atrium: an unusual presentation.
Crestanello JA, Orszulak TA.
Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota 55905, USA.
Ann Thorac Surg 2002 Oct;74(4):1252-4 Abstract quote Papillary fibroelastomas are small tumors of the valvular endocardium with a propensity to embolize. Fibroelastomas originating in the nonvalvular endocardium are rare.
We report a giant papillary fibroelastoma of the right atrial septum presenting with hemodynamic compromise that resolved after surgical excision. The current literature and the diagnostic and therapeutic strategies are reviewed.
MITRAL VALVE
Papillary fibroelastoma of a mitral valve chorda.Marnette JM, Hassan H, Sarot J, Jacobs K.
Department of Cardiac Surgery, Namur General Hospital, 185 avenue Albert 1st, 5000 Namur, Belgium.
Eur J Cardiothorac Surg 2001 Dec;20(6):1249-51 Abstract quote A 43-year-old woman presented with an ischemic stroke in the right middle cerebral artery territory. Cardiac echography disclosed a tumor of a primary chordae of the anterior leaflet of the mitral valve. After neurologic recovery, the patient was referred to surgery for excision of the tumor and plastic reconstruction using a chordal transfer technique. Histological examination of the tumor showed a typical papillary fibroelastoma.
Papillary fibroelastoma is the third most frequent cardiac benign tumor. The high embolic potential of this tumor is in favor of an aggressive surgical attitude.
PULMONARY VALVE
Pulmonary valve papillary fibroelastoma. A case report and review of the literature.Saad RS, Galvis CO, Bshara W, Liddicoat J, Dabbs DJ.
Department of Pathology, Allegheny General Hospital, Pittsburgh, PA, USA.
Arch Pathol Lab Med 2001 Jul;125(7):933-4 Abstract quote Papillary fibroelastomas are rare and benign cardiac tumors that typically affect the cardiac valves.
To the best of our knowledge, the English literature contains only 1 case report of pulmonary valve fibroelastoma diagnosed by echocardiogram and confirmed by surgical resection. There is a paucity of pathology literature on this subject.
We describe an additional case of pulmonary valve fibroelastoma diagnosed by transesophageal echocardiography and magnetic resonance imaging confirmed by pathologic examination in a patient who also had a thymoma.
TRICUSPID VALVE
Papillary fibroelastoma of the tricuspid valve chordae with a review of the literature.Fabricius AM, Heidrich L, Gutz U, Mohr FW.
Division of Cardiac Surgery, Heart Centre, University of Leipzig, Leipzig, Germany.
Cardiovasc J S Afr 2002 May-Jun;13(3):122-4 Abstract quote Endothelial papillary fibroelastomas represent a rare entity in cardiac pathology that at times may be associated with embolisation, angina, and sudden death.
We report on a case of a 46-year-old woman with a papillary fibroelastoma originating on the chordae of the tricuspid valve. The tumour was discovered incidentally using transthoracic two-dimensional echocardiography. The patient had an uneventful recovery and remained free of symptoms after six months.
VENTRICLE, LEFT Left ventricular papillary fibroelastoma: two-dimensional echocardiographic detection and surgical resection.
Jobic Y, Etienne Y, Quintin-Roue I, Dewilde J, Cornec P, Gilard M, Le Bras Y, Barra JA, Loire R, Boschat J, et al.
Department of Cardiology, Morvan Hospital, Brest, France
J Am Soc Echocardiogr 1995 Sep-Oct;8(5 Pt 1):756-8 Abstract quote We report a patient with a papillary fibroelastoma arising from the left ventricular posterior wall. The tumor was detected incidentally during echocardiography undertaken to evaluate aortic stenosis.
Possible complication from tumor embolization was avoided by surgical resection during aortic valve replacement.
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSTIC FACTORS INFECTION
Infected papillary fibroelastoma attached to the atrial septum.Koji T, Fujioka M, Imai H, Komada T, Takeuchi M, Ichikawa T, Tameda Y, Sato F, Nakano T.
Department of Internal Medicine, Yamamoto General Hospital, Kuwana, Japan.
Circ J 2002 Mar;66(3):305-7 Abstract quote A 61-year-old woman had intermittent fever of 2 months' duration following a dental extraction. On admission, her body temperature was 39.2 degrees C. A mid-systolic murmur was heard at the apex on ausculation.
A 2-dimensional echocardiogram revealed a mobile, heavy stick-like mass with vegetation (5.0 x 1.5 cm) attached to the left atrial septum. Multiple blood cultures grew Streptococcus constellatus. On diagnosis of an infected left atrial myxoma, antibiotics were administered daily and 4 weeks later, the left atrial tumor was resected. The tumor was 5.3cm long, 1.5cm in diameter at the inter-atrial wall and had vegetation on the free edge.
On microscopic examination, colonies of Gram-positive cocci were found in the thrombus, on the papillary fibroelastoma. After treatment with antibiotics for a further 4 weeks, the patient was discharged. This is the first report of infected papillary fibroelastoma.
RECURRENCE
Occurrence of a second primary papillary fibroelastoma.Hynes MS, Veinot JP, Chan KL.
Department of Cardiac Anesthesia, Ottawa Heart Institute, Ottawa, Ontario.
Can J Cardiol 2002 Jul;18(7):753-6 Abstract quote Papillary fibroelastomas are rare, benign cardiac tumours that most commonly arise from cardiac valves, and often present with signs and symptoms of embolization. A patient presented initially with transient ischemic attacks.
He was found to have a papillary fibroelastoma on his tricuspid valve, which was successfully resected. Nine years later, he presented again with cerebral embolic symptoms and was found to have a new fibroelastoma on his mitral valve.
The presence of a new lesion should be considered if signs or symptoms recur.
TREATMENT Surgical removal SURGERY
Cardiac valve papillary fibroelastoma: surgical excision for revealed or potential embolization.Grinda JM, Couetil JP, Chauvaud S, D'Attellis N, Berrebi A, Fabiani JN, Deloche A, Carpentier A.
Department of Cardiovascular Surgery of Broussais Hospital [1], Paris, France.
J Thorac Cardiovasc Surg 1999 Jan;117(1):106-10 Abstract quote OBJECTIVE: We have reviewed the case histories of 4 patients who underwent operations between September 1994 and November 1997 at Broussais Hospital for cardiac valvular papillary fibroelastoma. METHODS: Diagnosis was strongly suggested by echocardiography. Tumor locations were mitral (1), tricuspid (1), and aortic (2). Indications for operation were previous stroke for the mitral tumor, prophylaxis for the tricuspid tumor, syncopal episodes for the first aortic tumor, and transient ischemic attack and mesenteric ischemia for the second aortic tumor.
RESULTS: Surgical excision with a conservative, valve-sparing approach was performed in all cases. For the first aortic tumor, aortic valve reconstruction was achieved with part of a cryopreserved aortic homograft cusp. Intraoperative transesophageal echocardiography showed no evidence of valvular regurgitation after excision in all cases. All patients had uneventful postoperative recoveries. No evidence of regurgitation or recurrence was seen on echocardiography at follow-up.
CONCLUSIONS: Despite their histologically benign aspect, cardiac papillary fibroelastomas should be excised because of potential embolic complications. A conservative, valve-sparing approach is recommended, however, because of the absence of recurrence after total excision.
Should right-sided fibroelastomas be operated upon?Scalia D, Basso C, Rizzoli G, Lupia M, Budano S, Thiene G, Venturini A.
Department of Cardiovascular Surgery, University of Padova Medical School, Italy.
J Heart Valve Dis 1997 Nov;6(6):647-50 Abstract quote There is general agreement that left-sided elastomas must be removed to avoid systemic complications. Right-sided localization is less risky and surgical treatment is not agreed upon.
We report a case of surgical resected fibroelastoma of the tricuspid valve and discuss the indications to surgery by reviewing the literature on the few reported cases.
We feel that, if surgery is timely, the tumor can be resected, thus preserving the native valve. This provides an opportunity to prevent possible right-sided complications due to increasing tumor size and embolization.
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