Difference between revisions of "Cystic tumour of the atrioventricular nodal region"
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'''Cystic tumour of the atrioventricular nodal region''' is a rare cardiac tumour that can lead to [[sudden cardiac death]]. | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | |||
| Image = Cystic tumour of the atrioventricular nodal region - very low mag.jpg | |||
| Width = | |||
| Caption = Cystic tumour of the atrioventricular nodal region. [[H&E stain]]. | |||
| Synonyms = cystic tumour of the atrioventricular node | |||
| Micro = cystic spaces lined by single layer of bland epithelial cells, +/-focal mononuclear inflammation, +/-[[psammoma bodies]] | |||
| Subtypes = | |||
| LMDDx = cystic metastatic [[carcinoma]] | |||
| Stains = | |||
| IHC = CEA +ve, EMA +ve, calretinin -ve, WT1 -ve | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = very small cysts in region of [[AV node]] | |||
| Grossing = see ''[[Heart#Conducting_system|conducting system of heart]]'' | |||
| Staging = | |||
| Site = [[heart]] - see ''[[cardiac tumours]]'' | |||
| Assdx = [[sudden cardiac death]] | |||
| Syndromes = | |||
| Clinicalhx = usu. women - young | |||
| Signs = conduction abnormalities, +/-bradycardia (due to complete heart block) | |||
| Symptoms = | |||
| Prevalence = very rare | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = good if caught in time | |||
| Other = | |||
| ClinDDx = other [[cardiac tumours]] | |||
| 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]]. | |||
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 ''[[mesothelioma]]s''.<ref name=pmid16254122>{{Cite journal | last1 = Evans | first1 = CA. | last2 = Suvarna | first2 = SK. | title = Cystic atrioventricular node tumour: not a mesothelioma. | journal = J Clin Pathol | volume = 58 | issue = 11 | pages = 1232 | month = Nov | year = 2005 | doi = | PMID = 16254122 }}</ref><ref name=pmid19144541/> | |||
==General== | ==General== | ||
* | *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 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:<ref name=pmid19144541/> | |||
*Conduction abnormalities. | |||
**Complete heart block. | |||
*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''. | ||
*Cystic spaces. | *Usually 1-2 mm. | ||
*Cystic spaces - often not apparent at gross. | |||
==Microscopic== | ==Microscopic== | ||
Features:<ref name=pmid10722558>{{Cite journal | last1 = Paniagua | first1 = JR. | last2 = Sadaba | first2 = JR. | last3 = Davidson | first3 = LA. | last4 = Munsch | first4 = CM. | title = Cystic tumour of the atrioventricular nodal region: report of a case successfully treated with surgery. | journal = Heart | volume = 83 | issue = 4 | pages = E6 | month = Apr | year = 2000 | doi = | PMID = 10722558 }}</ref> | Features:<ref name=pmid10722558>{{Cite journal | last1 = Paniagua | first1 = JR. | last2 = Sadaba | first2 = JR. | last3 = Davidson | first3 = LA. | last4 = Munsch | first4 = CM. | title = Cystic tumour of the atrioventricular nodal region: report of a case successfully treated with surgery. | journal = Heart | volume = 83 | issue = 4 | pages = E6 | month = Apr | year = 2000 | doi = | PMID = 10722558 }}</ref> | ||
*Cystic spaces lined single layer of epithelial cells. | *Cystic spaces lined with single layer of epithelial cells. | ||
*+/-Focal mononuclear inflammation. | *+/-Focal mononuclear inflammation. | ||
*+/-[[Psammoma bodies]]. | *+/-[[Psammoma bodies]]. | ||
DDx: | |||
*Cystic [[metastatic carcinoma]]. | |||
===Images=== | ===Images=== | ||
| Line 21: | Line 69: | ||
Image:Cystic_tumour_of_the_atrioventricular_nodal_region_-_high_mag.jpg | CTAVNR - high mag. (WC) | Image:Cystic_tumour_of_the_atrioventricular_nodal_region_-_high_mag.jpg | CTAVNR - high mag. (WC) | ||
</gallery> | </gallery> | ||
==IHC== | ==IHC== | ||
Features:<ref name=pmid16254122/><ref name=pmid23253729>{{Cite journal | last1 = Pan | first1 = Y. | last2 = Chen | first2 = JL. | last3 = Li | first3 = ZJ. | last4 = Zhao | first4 = LX. | last5 = Wang | first5 = M. | last6 = Li | first6 = YK. | last7 = Tian | first7 = LB. | last8 = Guo | first8 = H. | title = Cystic tumour of the atrioventricular node: a case report and review of the literature. | journal = Chin Med J (Engl) | volume = 125 | issue = 24 | pages = 4514-6 | month = Dec | year = 2012 | doi = | PMID = 23253729 }}</ref> | |||
*CEA +ve. | *CEA +ve. | ||
*[[EMA]] +ve. | *[[EMA]] +ve. | ||
*[[CK7]] +ve. | |||
*Calretinin -ve. | |||
*WT1 -ve. | |||
==See also== | ==See also== | ||
*[[Cardiac tumours]]. | *[[Cardiac tumours]]. | ||
*[[Cardiac arrhythmias]]. | |||
*[[AV node]]. | |||
==References== | ==References== | ||
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
- Very rare.[3]
- May cause sudden cardiac death.[4]
- Antemortem diagnosis extremely rare - only four reported in literature as of 2010.[2]
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:
- Cystic metastatic carcinoma.
Images
CTAVNR - very low mag. (WC)
CTAVNR - high mag. (WC)
IHC
See also
References
- ↑ 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.
- ↑ 2.0 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.
- ↑ 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.
- ↑ 4.0 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.