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Cystic Tumor of the Atrioventricular Node

MedGen UID:
220412
Concept ID:
C1266075
Neoplastic Process
Synonyms: Benign Mesothelioma of Mahaim; Cystic tumor of atrio-ventricular node; Cystic Tumor of Atrioventricular Node; Endodermal Rest; Intracardiac Endodermal Heterotopia; Mesothelioma of Atrioventricular Node; Tawarian Node
SNOMED CT: Cystic tumor of atrio-ventricular node (128693001)

Definition

A multicystic tumor arising in the inferior interatrial septum in the region of the atrioventricular node. The vast majority of patients present with complete heart block and a minority with partial heart block. Sudden death is reported in approximately 10% of the cases. It is a morphologically benign tumor composed of cuboidal, transitional, or squamoid cells. The cells may also show sebaceous differentiation and originate from the endoderm. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCystic Tumor of the Atrioventricular Node

Recent clinical studies

Etiology

Suzuki K, Matsushita S, Suzuki H, Kuroda K, Inaba H, Arakawa A, Hayashi T, Amano A, Kuwatsuru R
J Thorac Imaging 2014 Nov;29(6):W97-9. doi: 10.1097/RTI.0000000000000111. PMID: 25303965
Petris AO, Alexandrescu DM, Costache II
Rev Med Chir Soc Med Nat Iasi 2014 Apr-Jun;118(2):289-92. PMID: 25076689

Diagnosis

Nogueira R, Marinho-Cardoso B, Gomes M, Amorim G
Cardiovasc Pathol 2024 Jan-Feb;68:107586. Epub 2023 Nov 4 doi: 10.1016/j.carpath.2023.107586. PMID: 37926350
Ichimata S, Hata Y, Yajima N, Katayama Y, Nomoto K, Nishida N
Pathol Int 2021 Feb;71(2):141-146. Epub 2020 Dec 14 doi: 10.1111/pin.13052. PMID: 33316142
Petris AO, Alexandrescu DM, Costache II
Rev Med Chir Soc Med Nat Iasi 2014 Apr-Jun;118(2):289-92. PMID: 25076689
Law KB, Feng T, Nair V, Cusimano RJ, Butany J
Cardiovasc Pathol 2012 Mar-Apr;21(2):120-7. Epub 2011 Aug 4 doi: 10.1016/j.carpath.2011.05.004. PMID: 21820324
Tran TT, Starnes V, Wang X, Getzen J, Ross BD
J Cardiovasc Magn Reson 2009 Apr 30;11(1):13. doi: 10.1186/1532-429X-11-13. PMID: 19405937Free PMC Article

Prognosis

Ichimata S, Hata Y, Yajima N, Katayama Y, Nomoto K, Nishida N
Pathol Int 2021 Feb;71(2):141-146. Epub 2020 Dec 14 doi: 10.1111/pin.13052. PMID: 33316142
Suzuki K, Matsushita S, Suzuki H, Kuroda K, Inaba H, Arakawa A, Hayashi T, Amano A, Kuwatsuru R
J Thorac Imaging 2014 Nov;29(6):W97-9. doi: 10.1097/RTI.0000000000000111. PMID: 25303965
Petris AO, Alexandrescu DM, Costache II
Rev Med Chir Soc Med Nat Iasi 2014 Apr-Jun;118(2):289-92. PMID: 25076689
Ford SE
Cardiovasc Pathol 1999 Jul-Aug;8(4):233-7. doi: 10.1016/s1054-8807(99)00013-7. PMID: 10724528

Clinical prediction guides

Law KB, Feng T, Nair V, Cusimano RJ, Butany J
Cardiovasc Pathol 2012 Mar-Apr;21(2):120-7. Epub 2011 Aug 4 doi: 10.1016/j.carpath.2011.05.004. PMID: 21820324

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