Papillary fibroelastoma: clinical and echocardiographic features and initial approach in 54 cases

Cianciulli, Tomás Francisco; Soumoulou, Juan Bautista; Lax, Jorge A.; Saccheri, María Cristina; Cozzarin, Alberto; Beck, Martín; Ferreiro, Daniel Ernesto; Prezioso, Horacio A.

doi:10.1111/echo.13351

Cited by 22 publications

(18 citation statements)

References 34 publications

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“…The valves most frequently affected by PFEs are the mitral and aortic valves. In our experience with a total of 54 papillary fibroelastomas [5], in 29.6% (16/54) of patients, they were located in the endocardium of the left ventricle (LV), as in this case. The most common symptoms are due to a peripheral embolism, and the following symptoms have been described:…”

Section: Discussionmentioning

confidence: 52%

“…Several authors have reported that this type of tumor is more often seen in elderly patients with longstanding heart disease; therefore, this suggests that they occur due to mechanical damage and involve a degenerative process [6]. In accordance with this theory, in a series of 17 patients with a diagnosis of fibroelastoma, echocardiography was shown to correlate well with pathological findings, and these tumors were usually found in areas of cardiac irritation, such as the aortic and mitral valves [5]. This information proved to be very important in our case, since the damage caused by radiotherapy might have affected the valvular and subvalvular mitral apparatus, thus enhancing the potential development of this type of tumor.…”

Section: Discussionmentioning

confidence: 77%

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Actinic Papillary Fibroelastoma of the Left Ventricle

Cianciulli¹,

Saccheri²

2019

Cancer Survivorship

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We present the case of a 69-year-old woman with a history of endometrial carcinoma in 1996, who underwent a total hysterectomy and bilateral adnexectomy. The patient also received chemotherapy (doxorubicin and cisplatinum) and local radiotherapy (50 Gy) because of a single lung metastasis, with total remission during later follow-up. During follow-up, 10 years later following radiotherapy, a transthoracic echocardiogram (TTE) revealed an image consistent with a primary cardiac tumor (papillary fibroelastoma) or metastatic cardiac tumor on the posteromedial papillary muscle. Cardiac magnetic resonance imaging (MRI) revealed a solid mass on the posteromedial papillary muscle with late enhancement, consistent with a primary cardiac tumor. During surgery, the tumor located in the posteromedial papillary muscle was resected. A pathological examination revealed the presence of a tumor mass with a core of dense connective tissue surrounded by a layer of hyperplastic endocardial cells characteristic of a papillary fibroelastoma. After 8 years of follow-up, the patient remains asymptomatic.

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Section: Discussionmentioning

confidence: 52%

Section: Discussionmentioning

confidence: 77%

Actinic Papillary Fibroelastoma of the Left Ventricle

Cianciulli¹,

Saccheri²

2019

Cancer Survivorship

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“…Papillary fibroelastoma (PFE) accounts for 7.9%, the second most common type of benign primary cardiac tumor . PFE is usually found as solitary tumor, and multiple tumors are relatively rare with an incidence of 7%–13% of all PFE cases . The aortic valve (AV) is the most common affected site in multiple PFEs, but it remains to be clarified whether attached side of the AV may affect the incidence of embolism.…”

Section: Introductionmentioning

confidence: 63%

Multiple papillary fibroelastomas attached to left ventricular side and aortic side of the aortic valve: A report of new case and literature review

et al. 2019

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The aortic valve (AV) is the most commonly affected site in multiple papillary fibroelastomas, but the frequency of embolism caused by the attachment side of the AV has not been elucidated. According to the review of the previous literature, 16 cases have been found attached to the AV. Of these, 6 of these have been found to be attached on the aortic side and 4 on the left ventricular side, 1 was bilateral, and 5 cases were unknown. Of the cases found on the aortic side, embolism occurred in 3 of them, and of the left ventricular side cases, embolism occurred in 2 of them. The frequency of embolism is equivalent even if papillary fibroelastoma attached to either side of the AV.

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“…The diagnostic accuracy of echocardiography is good (80%). The diagnosable tumors include myxoma, papillary fibroelastoma, and angiosarcoma [ 18 , 19 ]. The most common tumor type is myxoma, and second is papillary fibroelastoma.…”

Section: Discussionmentioning

confidence: 99%

Primary and metastatic cardiac tumors: echocardiographic diagnosis, treatment and prognosis in a 15-years single center study

Nomoto

Tani

Konda

et al. 2017

J Cardiothorac Surg

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BackgroundThe frequency of primary cardiac tumors is rare at about 0.3% by autopsy. Our objective was to investigate the characteristics and locations of cardiac tumors and to provide a prognostic analysis in our hospital.MethodsWe collected data on 95 patients with echocardiographic diagnosis or detection of cardiac tumors in a prospective analysis from 1999 to 2014. The median follow-up period was 43 months (0.5–183 months).ResultsThe subjects included 56 men and 39 women with a mean age of 65 years. Clinical diagnosis revealed primary tumors in 61 patients (64%) and secondary metastatic tumors in 34 patients (36%). In the 61 patients, 41 patients (67%) underwent surgery and tissue samples were obtained. Of these 41 patients, benign tumors were found in 30 cases (73%). One patient (2%) was diagnosed with thrombus. Among the benign tumors, myxoma (67%) was the most common type followed by papillary fibroelastoma (23%). The most common site was the left atrium (35%) followed by the right atrium (25%). Primary malignant tumors were diagnosed in 10 cases (24%), including 6 angiosarcomas, 3 lymphomas, and 1 leiomyosarcoma. The diagnostic accuracy of echocardiography was 80%. The patients with benign tumors were all alive at the end of the follow-up period. In contrast, 7 patients with malignant tumors died (70%) (p < 0.0001).ConclusionsOur data is in line with previous literature. Our study also suggests the necessity of extending our knowledge of the characteristics of cardiac tumors for diagnosis.

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Papillary fibroelastoma: clinical and echocardiographic features and initial approach in 54 cases

Abstract: PFE is a small tumor, predominantly valvular and benign, but entails a high incidence of cerebral embolism. The initial approach should be individualized according to clinical manifestations, comorbidities, and the experience of the surgical center.

Cited by 22 publications

References 34 publications

Actinic Papillary Fibroelastoma of the Left Ventricle

Actinic Papillary Fibroelastoma of the Left Ventricle

Multiple papillary fibroelastomas attached to left ventricular side and aortic side of the aortic valve: A report of new case and literature review

Primary and metastatic cardiac tumors: echocardiographic diagnosis, treatment and prognosis in a 15-years single center study

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