Pathology Outlines
Papillary giant fibroelastoma of aortic valve (Giant Lambl’s Excrescences of the Aortic Valve), H&E
Images and discussion contributed by Pallavi Khattar, M.D. (New York Medical College at Westchester Medical Center), Puneet Bedi, M.D. (Brookdale University Hospital and Medical Center, New York) and John T Fallon, MD, PhD (New York Medical College at Westchester Medical Center).
A 64 year old man presented with a past medical history of hypertension, congestive heart failure (CHF), atrial fibrillation and NSTEMI. He was admitted for CHF exacerbation and acute kidney failure. He continued to have worsening cardiac status and experienced sudden cardiac arrest. Findings at autopsy revealed evidence of myocardial ischemic damage and bulky rubbery lesion on the aortic valve, completely occluding right coronary ostium.
Discussion:
Cardiac papillary fibroelastomas (CPFEs) are rare benign tumors of the endocardium and represent the most common primary valvular tumors of the heart (Am Heart J 2003;146:404). These tumors are also referred to as giant Lambl’s excrescences, fibroelastic hamartomas or papilliferous tumors.
CPFE is sporadically reported with an incidence between 0.002% and 0.33% at autopsy. CPFE can occur in any age group, with the majority occurring in adults, and the highest prevalence in the eighth decade. They occur most frequently on the valvular surfaces (73%), particularly on the aortic (44%) and mitral (35%) valves (Can J Cardiol 2007;23:301).
Grossly, the tumor is characterized by broad-based filiform processes, with or without a central stalk. These lesions are more common near the lines of valvular closure.
Microscopically, CPFEs consists of papillary, pedunculated and avascular tumors, covered by a single layer of endothelium, containing variable amounts of elastic fibrils arranged in whorls in a hyaline stroma. The connective tissue contains a mucopolysaccharide acid matrix, smooth muscle cells, collagen and elastin fibers.
Differential diagnoses to be considered are other cardiac tumors (e.g. myxomas, thrombi and bacterial vegetation’s).
Although many papillary fibroelastomas do not cause symptoms, early diagnosis of CPFE is of prior importance to prevent patients from fatal complications. Nevertheless, this tumor can present with a variety of clinical manifestations, making diagnosis challenging. Transesophageal echocardiography is known to have high sensitivity to detect excrescences and should always be included in the diagnostic assessment. Asymptomatic patients who are found to have evidence of CPFE should be monitored closely (Circulation 1999;99:1919).
CPFEs are benign and can be removed by surgical resection.
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Papillary giant fibroelastoma of aortic valve (Giant Lambl’s Excrescences of the Aortic Valve), H&E
Images and discussion contributed by Pallavi Khattar, M.D. (New York Medical College at Westchester Medical Center), Puneet Bedi, M.D. (Brookdale University Hospital and Medical Center, New York) and John T Fallon, MD, PhD (New York Medical College at Westchester Medical Center).
A 64 year old man presented with a past medical history of hypertension, congestive heart failure (CHF), atrial fibrillation and NSTEMI. He was admitted for CHF exacerbation and acute kidney failure. He continued to have worsening cardiac status and experienced sudden cardiac arrest. Findings at autopsy revealed evidence of myocardial ischemic damage and bulky rubbery lesion on the aortic valve, completely occluding right coronary ostium.
Discussion:
Cardiac papillary fibroelastomas (CPFEs) are rare benign tumors of the endocardium and represent the most common primary valvular tumors of the heart (Am Heart J 2003;146:404). These tumors are also referred to as giant Lambl’s excrescences, fibroelastic hamartomas or papilliferous tumors.
CPFE is sporadically reported with an incidence between 0.002% and 0.33% at autopsy. CPFE can occur in any age group, with the majority occurring in adults, and the highest prevalence in the eighth decade. They occur most frequently on the valvular surfaces (73%), particularly on the aortic (44%) and mitral (35%) valves (Can J Cardiol 2007;23:301).
Grossly, the tumor is characterized by broad-based filiform processes, with or without a central stalk. These lesions are more common near the lines of valvular closure.
Microscopically, CPFEs consists of papillary, pedunculated and avascular tumors, covered by a single layer of endothelium, containing variable amounts of elastic fibrils arranged in whorls in a hyaline stroma. The connective tissue contains a mucopolysaccharide acid matrix, smooth muscle cells, collagen and elastin fibers.
Differential diagnoses to be considered are other cardiac tumors (e.g. myxomas, thrombi and bacterial vegetation’s).
Although many papillary fibroelastomas do not cause symptoms, early diagnosis of CPFE is of prior importance to prevent patients from fatal complications. Nevertheless, this tumor can present with a variety of clinical manifestations, making diagnosis challenging. Transesophageal echocardiography is known to have high sensitivity to detect excrescences and should always be included in the diagnostic assessment. Asymptomatic patients who are found to have evidence of CPFE should be monitored closely (Circulation 1999;99:1919).
CPFEs are benign and can be removed by surgical resection.
Click here to see topic.