Difference between revisions of "Cystic tumour of the atrioventricular nodal region"

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| Assdx      = [[sudden cardiac death]]
| Assdx      = [[sudden cardiac death]]
| Syndromes  =
| Syndromes  =
| Clinicalhx =  
| Clinicalhx = usu. women - young
| Signs      = conduction abnormalities, +/-bradycardia (due to complete heart block)
| Signs      = conduction abnormalities, +/-bradycardia (due to complete heart block)
| Symptoms  =
| Symptoms  =
Line 28: Line 28:
| Prognosis  = good if caught in time
| Prognosis  = good if caught in time
| Other      =
| Other      =
| ClinDDx    =
| ClinDDx    = other [[cardiac tumours]]
| Tx        = excision
| Tx        = excision for diagnosis + pacemaker
}}
}}
'''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''', abbreviated '''CTAVNR''', is a rare cardiac tumour that can lead to [[sudden cardiac death]].
Line 40: Line 40:
*Very rare.<ref name=pmid11855098>{{Cite journal  | last1 = Kaminishi | first1 = Y. | last2 = Watanabe | first2 = Y. | last3 = Nakata | first3 = H. | last4 = Shimokama | first4 = T. | last5 = Jikuya | first5 = T. | title = Cystic tumor of the atrioventricular nodal region. | journal = Jpn J Thorac Cardiovasc Surg | volume = 50 | issue = 1 | pages = 37-9 | month = Jan | year = 2002 | doi =  | PMID = 11855098 }}</ref>
*Very rare.<ref name=pmid11855098>{{Cite journal  | last1 = Kaminishi | first1 = Y. | last2 = Watanabe | first2 = Y. | last3 = Nakata | first3 = H. | last4 = Shimokama | first4 = T. | last5 = Jikuya | first5 = T. | title = Cystic tumor of the atrioventricular nodal region. | journal = Jpn J Thorac Cardiovasc Surg | volume = 50 | issue = 1 | pages = 37-9 | month = Jan | year = 2002 | doi =  | PMID = 11855098 }}</ref>
*May cause [[Sudden_natural_death|sudden cardiac death]].<ref name=pmid10722558/>
*May cause [[Sudden_natural_death|sudden cardiac death]].<ref name=pmid10722558/>
*Antemortem diagnosis in case reports; only four reported in literature as of 2010.<ref name=pmid19144541>{{Cite journal  | last1 = Sharma | first1 = G. | last2 = Linden | first2 = MD. | last3 = Schultz | first3 = DS. | last4 = Inamdar | first4 = KV. | title = Cystic tumor of the atrioventricular node: an unexpected finding in an explanted heart. | journal = Cardiovasc Pathol | volume = 19 | issue = 3 | pages = e75-8 | month =  | year =  | doi = 10.1016/j.carpath.2008.10.011 | PMID = 19144541 }}</ref>
*Antemortem diagnosis extremely rare - only four reported in literature as of 2010.<ref name=pmid19144541>{{Cite journal  | last1 = Sharma | first1 = G. | last2 = Linden | first2 = MD. | last3 = Schultz | first3 = DS. | last4 = Inamdar | first4 = KV. | title = Cystic tumor of the atrioventricular node: an unexpected finding in an explanted heart. | journal = Cardiovasc Pathol | volume = 19 | issue = 3 | pages = e75-8 | month =  | year =  | doi = 10.1016/j.carpath.2008.10.011 | PMID = 19144541 }}</ref>


Clinical:
Clinical:<ref name=pmid19144541/>
*Conduction abnormalities.
*Conduction abnormalities.
**Complete heart block.
**Complete heart block.
*Usually women.
*Usually women - mean age 38 years.
 
Treatment:
*Excision + pacemaker.<ref name=pmid19237400>{{Cite journal  | last1 = Guo | first1 = J. | last2 = Zuo | first2 = S. | last3 = Lin | first3 = C. | last4 = Ji | first4 = Y. | title = Surgical treatment of a giant cystic tumor of the atrioventricular nodal region. | journal = Interact Cardiovasc Thorac Surg | volume = 8 | issue = 5 | pages = 592-3 | month = May | year = 2009 | doi = 10.1510/icvts.2008.191866 | PMID = 19237400 }}</ref><ref name=pmid16195634>{{Cite journal  | last1 = Saito | first1 = S. | last2 = Kobayashi | first2 = J. | last3 = Tagusari | first3 = O. | last4 = Bando | first4 = K. | last5 = Niwaya | first5 = K. | last6 = Nakajima | first6 = H. | last7 = Yamagishi | first7 = M. | last8 = Yagihara | first8 = T. | last9 = Kitamura | first9 = S. | title = Successful excision of a cystic tumor of the atrioventricular nodal region. | journal = Circ J | volume = 69 | issue = 10 | pages = 1293-4 | month = Oct | year = 2005 | doi =  | PMID = 16195634 }}</ref>


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


==Microscopic==
==Microscopic==

Latest revision as of 23:44, 22 July 2016

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

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

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

  • CTAVNR - very low mag. (WC)

  • CTAVNR - high mag. (WC)

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.
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