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Papillary Fibroelastoma

MedGen UID:
408153
•Concept ID:
C1947920
•
Neoplastic Process
Synonyms: Cardiac Papillary Fibroelastoma; Cardiac Papillary Fibroelastomas; Fibroelastoma, Cardiac Papillary; Fibroelastoma, Papillary; Papillary Fibroelastoma, Cardiac; Papillary Fibroelastomas
SNOMED CT: Papillary fibroelastoma of heart (725909004); Cardiac papillary fibroelastoma (725909004)

Definition

A rare benign neoplasm of the endocardium. The vast majority of cases arise in the heart valves. Histopathologic examination reveals a papilloma lined by endothelial cells with a central avascular core which contains fibroblasts and elastic fibers. Patients may be asymptomatic or present with ischemic or embolic events. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPapillary Fibroelastoma

Professional guidelines

PubMed

Primary and metastatic cardiac tumors: echocardiographic diagnosis, treatment and prognosis in a 15-years single center study.
Nomoto N, Tani T, Konda T, Kim K, Kitai T, Ota M, Kaji S, Imai Y, Okada Y, Furukawa Y
J Cardiothorac Surg 2017 Nov 28;12(1):103. doi: 10.1186/s13019-017-0672-7. PMID: 29183343Free PMC Article
Twenty years experience in oncologic surgery for primary cardiac tumors.
Dell'amore A, Albertini A, Lamarra M
G Chir 2013 Apr;34(4):106-11. PMID: 23660160Free PMC Article

Recent clinical studies

Etiology

Operative management of cardiac papillary fibroelastomas.
Mazur P, Kurmann R, Klarich KW, Dearani JA, Arghami A, Daly RC, Greason K, Schaff HV, Ahmad A, El-Am E, Sorour A, Bois MC, Viehman J, King KS, Maleszewski JJ, Crestanello JA
J Thorac Cardiovasc Surg 2024 Mar;167(3):1088-1097.e2. Epub 2022 Jul 16 doi: 10.1016/j.jtcvs.2022.06.022. PMID: 35989118
Surgery for Valvular and Nonvalvular Papillary Fibroelastomas.
Alozie A, Zimpfer A, Erbersdobler A, Neßelmann C, Öner A, Dohmen PM
Semin Thorac Cardiovasc Surg 2022 Summer;34(2):560-568. Epub 2021 May 20 doi: 10.1053/j.semtcvs.2021.03.037. PMID: 34022368
Recurrence of Pathologically Proven Papillary Fibroelastoma.
Sorour AA, Kurmann RD, El-Am EA, Bois MC, Scott CG, Lee AT, Dearani JA, Maleszewski JJ, Klarich KW
Ann Thorac Surg 2022 Apr;113(4):1208-1214. Epub 2021 May 19 doi: 10.1016/j.athoracsur.2021.03.114. PMID: 34022212
Cardiac Fibroelastoma: 10-Year Experience.
Pinheiro Santos J, Banazol N, Cruz Tomás A, Varela-Afonso D, Fragata J
Rev Port Cir Cardiotorac Vasc 2017 Jul-Dec;24(3-4):161. PMID: 29701392
Papillary fibroelastoma: clinical and echocardiographic features and initial approach in 54 cases.
Cianciulli TF, Soumoulou JB, Lax JA, Saccheri MC, Cozzarin A, Beck MA, Ferreiro DE, Prezioso HA
Echocardiography 2016 Dec;33(12):1811-1817. Epub 2016 Aug 26 doi: 10.1111/echo.13351. PMID: 27566126

Diagnosis

Massive Mysteries.
Aston A, Harowicz MR, Grizzard JD, Bottinor W
Am J Cardiol 2023 Oct 1;204:178-182. Epub 2023 Aug 4 doi: 10.1016/j.amjcard.2023.07.030. PMID: 37544141Free PMC Article
Left Ventricular Papillary Fibroelastoma.
Balboa C, Souaf S, Suárez JM, El-Diasty MM, Fernandez AL
Port J Card Thorac Vasc Surg 2023 Apr 4;30(1):49-52. doi: 10.48729/pjctvs.332. PMID: 37029939
Recurrence of Pathologically Proven Papillary Fibroelastoma.
Sorour AA, Kurmann RD, El-Am EA, Bois MC, Scott CG, Lee AT, Dearani JA, Maleszewski JJ, Klarich KW
Ann Thorac Surg 2022 Apr;113(4):1208-1214. Epub 2021 May 19 doi: 10.1016/j.athoracsur.2021.03.114. PMID: 34022212
Unusual place… unusual number.
Vieira MJ, Craveiro N, Alves M, Pitta L
Echocardiography 2020 Mar;37(3):467-468. Epub 2020 Feb 20 doi: 10.1111/echo.14616. PMID: 32077509
Cardiac Fibroelastoma: 10-Year Experience.
Pinheiro Santos J, Banazol N, Cruz Tomás A, Varela-Afonso D, Fragata J
Rev Port Cir Cardiotorac Vasc 2017 Jul-Dec;24(3-4):161. PMID: 29701392

Therapy

Clinicopathologic features and outcomes of primary cardiac tumors: a 16-year-experience with 212 patients at a Chinese medical center.
Wang JG, Wang B, Hu Y, Liu JH, Liu B, Liu H, Zhao P, Zhang L, Li YJ
Cardiovasc Pathol 2018 Mar-Apr;33:45-54. Epub 2018 Jan 6 doi: 10.1016/j.carpath.2018.01.003. PMID: 29414432
Robotic Excision of a Papillary Fibroelastoma of the Mitral Chordae.
Arsalan M, Smith RL, Squiers JJ, Wang A, DiMaio JM, Mack MJ
Ann Thorac Surg 2016 Jun;101(6):e187-8. doi: 10.1016/j.athoracsur.2015.10.015. PMID: 27211977
Stroke and cardiac papillary fibroelastoma: mechanical thrombectomy after thrombolytic therapy.
Santos AF, Pinho J, Ramos V, Pardal J, Rocha J, Ferreira C
J Stroke Cerebrovasc Dis 2014 May-Jun;23(5):1262-4. Epub 2013 Oct 19 doi: 10.1016/j.jstrokecerebrovasdis.2013.09.018. PMID: 24144594
Thrombotic thrombocytopenic purpura and cardiac papillary fibroelastoma: a 'unique coexistence'.
Ramakrishnan Geethakumari P, Rubin A, Varadi G
Blood Coagul Fibrinolysis 2013 Dec;24(8):881-3. doi: 10.1097/MBC.0b013e32836466ec. PMID: 24077148
Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases.
Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ
Am Heart J 2003 Sep;146(3):404-10. doi: 10.1016/S0002-8703(03)00249-7. PMID: 12947356

Prognosis

Rare Cardiac Papillary Fibroelastoma: Right Atrial, Non-Valvular, Large, Symptomatic With Pulmonary Embolism.
Prasad RM, Osman AF, Garces CC, Gumbita R, Elshafie A, Pandrangi P, Kehdi M
Perm J 2021 Nov 12;25 doi: 10.7812/TPP/21.069. PMID: 35348102Free PMC Article
Right atrial papillary fibroelastoma arising from the Chiari network detected by echocardiography: a case report and literature review.
Hu N, Shen W, Li H, Zhang Q, Cai G, Zhang Y, Du G
Cardiovasc Pathol 2021 Nov-Dec;55:107372. Epub 2021 Jul 25 doi: 10.1016/j.carpath.2021.107372. PMID: 34320368
Cardiac papillary fibroelastoma originating from the coumadin ridge and review of literature.
Lak HM, Kerndt CC, Unai S, Maroo A
BMJ Case Rep 2020 Aug 26;13(8) doi: 10.1136/bcr-2020-235361. PMID: 32847879Free PMC Article
Cardiac papillary fibroelastoma: Report of a surgical series.
Rodrigues JD, Ferreira J, Almeida J, Campelo M, Maciel MJ, Pinho P
Rev Port Cardiol (Engl Ed) 2018 Dec;37(12):981-986. Epub 2018 Dec 7 doi: 10.1016/j.repc.2018.02.011. PMID: 30528564
Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases.
Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ
Am Heart J 2003 Sep;146(3):404-10. doi: 10.1016/S0002-8703(03)00249-7. PMID: 12947356

Clinical prediction guides

Surgery for Valvular and Nonvalvular Papillary Fibroelastomas.
Alozie A, Zimpfer A, Erbersdobler A, Neßelmann C, Öner A, Dohmen PM
Semin Thorac Cardiovasc Surg 2022 Summer;34(2):560-568. Epub 2021 May 20 doi: 10.1053/j.semtcvs.2021.03.037. PMID: 34022368
Unusual place… unusual number.
Vieira MJ, Craveiro N, Alves M, Pitta L
Echocardiography 2020 Mar;37(3):467-468. Epub 2020 Feb 20 doi: 10.1111/echo.14616. PMID: 32077509
Papillary fibroelastoma entangled with tendinous chords: gap between echocardiographic and operative findings.
Nakanaga H, Matsumoto S, Tabata M
Int J Cardiovasc Imaging 2020 Mar;36(3):457-458. Epub 2019 Dec 20 doi: 10.1007/s10554-019-01740-4. PMID: 31863238
Cardiac papillary fibroelastoma: retrospective clinicopathologic study of 17 tumors with resection at a single institution and literature review.
Val-Bernal JF, Mayorga M, Garijo MF, Val D, Nistal JF
Pathol Res Pract 2013 Apr;209(4):208-14. Epub 2013 Feb 28 doi: 10.1016/j.prp.2013.02.001. PMID: 23455367
Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases.
Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ
Am Heart J 2003 Sep;146(3):404-10. doi: 10.1016/S0002-8703(03)00249-7. PMID: 12947356

Recent systematic reviews

Papillary fibroelastoma of the pulmonary valve--a systematic review.
Hakim FA, Aryal MR, Pandit A, Pandit AA, Alegria JR, Kendall CB, Click RL
Echocardiography 2014 Feb;31(2):234-40. Epub 2013 Oct 15 doi: 10.1111/echo.12388. PMID: 24128270

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