Case Report: A Rare Case of Right-Sided Papillary Fibroelastoma in a 1-Year-Old With Congenital Heart Disease.

Published on Jan 27, 2021in Frontiers in Cardiovascular Medicine3.915
· DOI :10.3389/FCVM.2020.624219
Ali Ahmad5
Estimated H-index: 5
(Mayo Clinic),
Edward A. El-Am2
Estimated H-index: 2
(IU: Indiana University)
+ 4 AuthorsKyle W. Klarich27
Estimated H-index: 27
(Mayo Clinic)
Introduction: Cardiac papillary fibroelastomas (PFEs) are the most common primary benign cardiac tumors, although they are somewhat unusual in children and typically seen on the left-sided cardiac valves. Case summary: A 10-week-old patient was found to have a partial atrioventricular canal defect, with associated tricuspid and mitral regurgitation. He was medically managed until 1 year of age, when surgical correction was done. During the procedure, a PFE was found incidentally on the TV. Conclusion: This is one of the youngest patients to be reported with PFE, thus adding to the literature of these unusual cases in children.
Papillary fibroelastoma (PFE) is an increasingly recognized cardiac tumor. Despite its prevalence, controversy exists as to whether it represents a reactive or neoplastic process due to histopathologic similarities with Lambl excrescences (LEs), an accepted reactive phenomenon. Recently, KRAS mutations were reported in a small collection of PFEs, but the incidence of mutations and conditions in which they arise in are unknown. Furthermore, the relationship between PFE and LE has yet to be invest...
1 CitationsSource
#1Joseph J. Maleszewski (Mayo Clinic)H-Index: 41
#2Melanie C. Bois (Mayo Clinic)H-Index: 10
Last. Kyle W. Klarich (Mayo Clinic)H-Index: 27
view all 6 authors...
Abstract The intersection of oncological and cardiovascular diseases is an increasingly recognized phenomenon. This recognition has led to the emergence of cardio-oncology as a true subspecialty. This field is not simply limited to primary cardiac tumors or complications of chemotherapeutic medications. Rather, it also encompasses metastatic cardiovascular complications and secondary cardiovascular effects of the underlying neoplasia. This review will broadly cover primary and metastatic cardiac...
34 CitationsSource
#1Kimiaki Okada (Saitama Medical University)H-Index: 1
#2Ayumu Masuoka (Saitama Medical University)H-Index: 6
Last. Takaaki Suzuki (Saitama Medical University)H-Index: 7
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Although hematological malignancies are a known complication of Down syndrome, few reports have described cases involving solid tumors. We describe the case of a 3-year-old Down syndrome girl with a primary solid cardiac tumor. Outpatient echocardiography after intracardiac repair of a ventricular septal defect at 6 months of age revealed a highly mobile pedunculated mass (8 × 9 mm) on the free wall of the right atrium. Due to potential incarceration of the mass in the tricuspid orifice, it was ...
1 CitationsSource
#1Syahidah Syed Tamin (Mayo Clinic)H-Index: 3
#2Joseph J. Maleszewski (Mayo Clinic)H-Index: 41
Last. Kyle W. Klarich (Mayo Clinic)H-Index: 27
view all 9 authors...
Abstract Background Papillary fibroelastomas (PFE) are benign neoplasms with little available outcome data. Objectives This study sought to describe the frequency and clinical course of patients with surgically removed PFE and echocardiographically suspected, but unoperated, PFE. Methods Mayo Clinic pathology and echocardiography databases (January 1, 1995, to December 31, 2010) were queried, resulting in 511 patients: group 1 (n = 185), including patients with surgically removed, histopathologi...
70 CitationsSource
#1Jing Ping Sun (Cleveland Clinic)H-Index: 21
#2Craig R. Asher (Cleveland Clinic)H-Index: 29
Last. James D. Thomas (Cleveland Clinic)H-Index: 137
view all 10 authors...
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 echocardi...
438 CitationsSource
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