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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

Cystic tumor of the atrioventricular node: computed tomography and magnetic resonance imaging findings.
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
Cardiac tumors.
Petris AO, Alexandrescu DM, Costache II
Rev Med Chir Soc Med Nat Iasi 2014 Apr-Jun;118(2):289-92. PMID: 25076689

Diagnosis

Variations in subtle cystic tumors of the atrioventricular node: Five autopsy cases.
Muramatsu H, Asakura K, Hosoya T, Kurosu A
Cardiovasc Pathol 2024 Mar-Apr;69:107607. Epub 2024 Jan 20 doi: 10.1016/j.carpath.2024.107607. PMID: 38253301
Fetal cystic and solid tumor of the atrioventricular node: Autopsy diagnosis at 12 weeks of gestational age.
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
Partial resection of cystic tumor of atrioventricular node.
Fukui S, Mitsuno M, Yamamura M, Ryomoto M, Hao H, Miyamoto Y
Ann Thorac Surg 2014 Dec;98(6):2223-6. Epub 2014 Dec 1 doi: 10.1016/j.athoracsur.2014.02.061. PMID: 25468099
Cardiac tumors.
Petris AO, Alexandrescu DM, Costache II
Rev Med Chir Soc Med Nat Iasi 2014 Apr-Jun;118(2):289-92. PMID: 25076689
Cystic tumor of the atrioventricular node: rare antemortem diagnosis.
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

Prognosis

Sex-dependent expression of prostatic markers and hormone receptors in cystic tumor of the atrioventricular node: A histopathological study of three cases.
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
Cystic tumor of the atrioventricular node: computed tomography and magnetic resonance imaging findings.
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
Cardiac tumors.
Petris AO, Alexandrescu DM, Costache II
Rev Med Chir Soc Med Nat Iasi 2014 Apr-Jun;118(2):289-92. PMID: 25076689
Congenital cystic tumors of the atrio-ventricular node: successful demonstration by an abbreviated dissection of the conduction system.
Ford SE
Cardiovasc Pathol 1999 Jul-Aug;8(4):233-7. doi: 10.1016/s1054-8807(99)00013-7. PMID: 10724528

Clinical prediction guides

Cystic tumor of the atrioventricular node: rare antemortem diagnosis.
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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