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Cardiac fibroma

MedGen UID:
203335
Concept ID:
C1096654
Neoplastic Process
Synonyms: Cardiac Fibroelastic Hamartoma; Cardiac Fibroma; Cardiac Fibrous Hamartoma; Fibroelastic Hamartoma of Heart; Fibroelastic Hamartoma of the Heart; Heart Fibroelastic Hamartoma; Heart Fibroma; Heart Fibrous Hamartoma
 
HPO: HP:0010617

Definition

A fibroma of the heart. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCardiac fibroma

Conditions with this feature

Gorlin syndrome
MedGen UID:
2554
Concept ID:
C0004779
Neoplastic Process
Nevoid basal cell carcinoma syndrome (NBCCS) is characterized by the development of multiple jaw keratocysts, frequently beginning in the second decade of life, and/or basal cell carcinomas (BCCs) usually from the third decade onward. Approximately 60% of individuals have a recognizable appearance with macrocephaly, frontal bossing, coarse facial features, and facial milia. Most individuals have skeletal anomalies (e.g., bifid ribs, wedge-shaped vertebrae). Ectopic calcification, particularly in the falx, is present in more than 90% of affected individuals by age 20 years. Cardiac and ovarian fibromas occur in approximately 2% and 20% of individuals respectively. Approximately 5% of all children with NBCCS develop medulloblastoma (primitive neuroectodermal tumor), generally the desmoplastic subtype. The risk of developing medulloblastoma is substantially higher in individuals with an SUFU pathogenic variant (33%) than in those with a PTCH1 pathogenic variant (<2%). Peak incidence is at age one to two years. Life expectancy in NBCCS is not significantly different from average.

Professional guidelines

PubMed

Juaneda I, Bueno N, Ochoa J, Díaz J, Peirone A
World J Pediatr Congenit Heart Surg 2022 Jan;13(1):101-104. Epub 2021 May 26 doi: 10.1177/2150135121992692. PMID: 34039104

Recent clinical studies

Etiology

Wilke PI, Biermann D, Grafmann M, Kozlik-Feldmann R, Papingi D, Sachweh JS, Stute F, Olfe J
Orphanet J Rare Dis 2023 Jul 5;18(1):178. doi: 10.1186/s13023-023-02792-5. PMID: 37408081Free PMC Article
Covington MK, Young PM, Bois MC, Maleszewski JJ, Anand V, Dearani JA, Klarich KW
Am J Cardiol 2022 Nov 1;182:95-103. Epub 2022 Aug 30 doi: 10.1016/j.amjcard.2022.06.062. PMID: 36055811
Teng F, Yang S, Chen D, Fang W, Shang J, Dong S, Cui Y, Fu W, Zheng M, Li Y, Lian G
Cardiovasc Pathol 2022 Jan-Feb;56:107381. Epub 2021 Aug 22 doi: 10.1016/j.carpath.2021.107381. PMID: 34433104
Carreon CK, Sanders SP, Perez-Atayde AR, Del Nido PJ, Walsh EP, Geva T, Alexander ME
JACC Clin Electrophysiol 2019 May;5(5):563-575. Epub 2019 Mar 27 doi: 10.1016/j.jacep.2019.01.022. PMID: 31122378
Sargar KM, Sheybani EF, Shenoy A, Aranake-Chrisinger J, Khanna G
Radiographics 2016 Jul-Aug;36(4):1195-214. doi: 10.1148/rg.2016150191. PMID: 27399243

Diagnosis

Covington MK, Young PM, Bois MC, Maleszewski JJ, Anand V, Dearani JA, Klarich KW
Am J Cardiol 2022 Nov 1;182:95-103. Epub 2022 Aug 30 doi: 10.1016/j.amjcard.2022.06.062. PMID: 36055811
Juaneda I, Bueno N, Ochoa J, Díaz J, Peirone A
World J Pediatr Congenit Heart Surg 2022 Jan;13(1):101-104. Epub 2021 May 26 doi: 10.1177/2150135121992692. PMID: 34039104
Ascaso M, Astrosa E, Moscoso B, Fernandez A, Barriuso C, Castella M
Multimed Man Cardiothorac Surg 2019 Mar 12;2019 doi: 10.1510/mmcts.2019.010. PMID: 30990581
Sargar KM, Sheybani EF, Shenoy A, Aranake-Chrisinger J, Khanna G
Radiographics 2016 Jul-Aug;36(4):1195-214. doi: 10.1148/rg.2016150191. PMID: 27399243
Chu ZG, Zhu ZY, Liu MQ, Lv FJ
J Card Surg 2014 Mar;29(2):159-62. Epub 2013 Nov 25 doi: 10.1111/jocs.12251. PMID: 24635211

Therapy

Ünsal H, Ekici E
Turk Kardiyol Dern Ars 2015 Jul;43(5):481-3. doi: 10.5543/tkda.2015.25295. PMID: 26148084
Massin M, Ould AF, Jacquemart C, Damry N
Acta Cardiol 2013 Oct;68(5):513-5. doi: 10.1080/ac.68.5.2994476. PMID: 24283114
Horovitz A, van Geldorp IE, Roubertie F, Thambo JB
Pediatr Cardiol 2012 Dec;33(8):1458-60. Epub 2012 May 29 doi: 10.1007/s00246-012-0390-9. PMID: 22644419Free PMC Article
Leja MJ, Perryman L, Reardon MJ
Tex Heart Inst J 2011;38(3):279-81. PMID: 21720472Free PMC Article
Leatherbury L, Chandra RS, Shapiro SR, Perry LW
J Am Coll Cardiol 1988 Dec;12(6):1547-54. doi: 10.1016/s0735-1097(88)80024-x. PMID: 3192852

Prognosis

Gao H, Yuan S, Hu Z, Zheng Z, Wang Y, Wu S
Cardiovasc J Afr 2024 Jan-Apr 23;35(1):64-66. Epub 2023 Mar 3 doi: 10.5830/CVJA-2023-007. PMID: 36947158
Beroukhim RS, Geva T, Del Nido P, Sleeper LA, Lu M, Muter A, Harrild DM, Walsh EP, Nathan M
Circ Cardiovasc Imaging 2021 Feb;14(2):e011748. Epub 2021 Feb 1 doi: 10.1161/CIRCIMAGING.120.011748. PMID: 33517672
Ma H, Sun H, Yang Y, Lv F, Ran J
Cardiol Young 2020 Sep;30(9):1328-1331. Epub 2020 Jul 17 doi: 10.1017/S1047951120002048. PMID: 32677597
Sargar KM, Sheybani EF, Shenoy A, Aranake-Chrisinger J, Khanna G
Radiographics 2016 Jul-Aug;36(4):1195-214. doi: 10.1148/rg.2016150191. PMID: 27399243
Torimitsu S, Nemoto T, Wakayama M, Okubo Y, Yokose T, Kitahara K, Ozawa T, Nakayama H, Shinozaki M, Sasai D, Ishiwatari T, Takuma K, Shibuya K
Eur J Med Res 2012 Mar 27;17(1):5. doi: 10.1186/2047-783X-17-5. PMID: 22472419Free PMC Article

Clinical prediction guides

Covington MK, Young PM, Bois MC, Maleszewski JJ, Anand V, Dearani JA, Klarich KW
Am J Cardiol 2022 Nov 1;182:95-103. Epub 2022 Aug 30 doi: 10.1016/j.amjcard.2022.06.062. PMID: 36055811
Teng F, Yang S, Chen D, Fang W, Shang J, Dong S, Cui Y, Fu W, Zheng M, Li Y, Lian G
Cardiovasc Pathol 2022 Jan-Feb;56:107381. Epub 2021 Aug 22 doi: 10.1016/j.carpath.2021.107381. PMID: 34433104
Yuan X, Li B, Yang Y, Wang H, Sun H, Song Y, Wang W
J Card Surg 2020 Aug;35(8):1912-1919. Epub 2020 Jul 11 doi: 10.1111/jocs.14790. PMID: 32652694
Torimitsu S, Nemoto T, Wakayama M, Okubo Y, Yokose T, Kitahara K, Ozawa T, Nakayama H, Shinozaki M, Sasai D, Ishiwatari T, Takuma K, Shibuya K
Eur J Med Res 2012 Mar 27;17(1):5. doi: 10.1186/2047-783X-17-5. PMID: 22472419Free PMC Article
Gorlin RJ
Medicine (Baltimore) 1987 Mar;66(2):98-113. doi: 10.1097/00005792-198703000-00002. PMID: 3547011

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