Cystic tumour of the atrioventricular nodal region

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Cystic tumour of the atrioventricular nodal region, abbreviated CTAVNR, is a rare cardiac tumour that can lead to sudden cardiac death.

Cystic tumour of the atrioventricular nodal region
Diagnosis in short

Cystic tumour of the atrioventricular nodal region. H&E stain.

Synonyms cystic tumour of the atrioventricular node

LM cystic spaces lined by single layer of bland epithelial cells, +/-focal mononuclear inflammation, +/-psammoma bodies
LM DDx cystic metastatic carcinoma
IHC CEA +ve, EMA +ve, calretinin -ve, WT1 -ve
Gross very small cysts in region of AV node
Grossing notes see conducting system of heart
Site heart - see cardiac tumours

Associated Dx sudden cardiac death
Clinical history usu. women - young
Signs conduction abnormalities, +/-bradycardia (due to complete heart block)
Prevalence very rare
Prognosis good if caught in time
Clin. DDx other cardiac tumours
Treatment excision for diagnosis + pacemaker

It is also known as cystic tumour of the atrioventricular node.

Benign mesothelioma of the atrioventricular node is also used; however, these tumours are not mesotheliomas.[1][2]

General

Clinical:[2]

  • Conduction abnormalities.
    • Complete heart block.
  • Usually women - mean age 38 years.

Treatment:

Gross

  • "Bump" in the triangle of Koch.
  • Usually 1-2 mm.
  • Cystic spaces - often not apparent at gross.

Microscopic

Features:[4]

  • Cystic spaces lined with single layer of epithelial cells.
  • +/-Focal mononuclear inflammation.
  • +/-Psammoma bodies.

DDx:

Images

IHC

Features:[1][7]

  • CEA +ve.
  • EMA +ve.
  • CK7 +ve.
  • Calretinin -ve.
  • WT1 -ve.

See also

References

  1. 1.0 1.1 Evans, CA.; Suvarna, SK. (Nov 2005). "Cystic atrioventricular node tumour: not a mesothelioma.". J Clin Pathol 58 (11): 1232. PMID 16254122.
  2. 2.0 2.1 2.2 Sharma, G.; Linden, MD.; Schultz, DS.; Inamdar, KV.. "Cystic tumor of the atrioventricular node: an unexpected finding in an explanted heart.". Cardiovasc Pathol 19 (3): e75-8. doi:10.1016/j.carpath.2008.10.011. PMID 19144541.
  3. Kaminishi, Y.; Watanabe, Y.; Nakata, H.; Shimokama, T.; Jikuya, T. (Jan 2002). "Cystic tumor of the atrioventricular nodal region.". Jpn J Thorac Cardiovasc Surg 50 (1): 37-9. PMID 11855098.
  4. 4.0 4.1 Paniagua, JR.; Sadaba, JR.; Davidson, LA.; Munsch, CM. (Apr 2000). "Cystic tumour of the atrioventricular nodal region: report of a case successfully treated with surgery.". Heart 83 (4): E6. PMID 10722558.
  5. Guo, J.; Zuo, S.; Lin, C.; Ji, Y. (May 2009). "Surgical treatment of a giant cystic tumor of the atrioventricular nodal region.". Interact Cardiovasc Thorac Surg 8 (5): 592-3. doi:10.1510/icvts.2008.191866. PMID 19237400.
  6. Saito, S.; Kobayashi, J.; Tagusari, O.; Bando, K.; Niwaya, K.; Nakajima, H.; Yamagishi, M.; Yagihara, T. et al. (Oct 2005). "Successful excision of a cystic tumor of the atrioventricular nodal region.". Circ J 69 (10): 1293-4. PMID 16195634.
  7. Pan, Y.; Chen, JL.; Li, ZJ.; Zhao, LX.; Wang, M.; Li, YK.; Tian, LB.; Guo, H. (Dec 2012). "Cystic tumour of the atrioventricular node: a case report and review of the literature.". Chin Med J (Engl) 125 (24): 4514-6. PMID 23253729.