Difference between revisions of "Cardiac tumours"

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'''[[Heart|Cardiac]] tumours''' are rare buggers.  They provide some work for cardiac surgeons.
'''[[Heart|Cardiac]] tumours''' are rare buggers.  They provide some work for cardiac surgeons.


==Most common (malignant)==
=Most common malignant=
*Metastases - most common cardiac tumour; 1:~100 primary tumours:secondary tumours.<ref name=pmid20472615>{{Cite journal  | last1 = Castillo | first1 = JG. | last2 = Silvay | first2 = G. | title = Characterization and management of cardiac tumors. | journal = Semin Cardiothorac Vasc Anesth | volume = 14 | issue = 1 | pages = 6-20 | month = Mar | year = 2010 | doi = 10.1177/1089253210362596 | PMID = 20472615 }}</ref>
*[[Metastases]] - most common cardiac tumour; 1:~100 primary tumours:secondary tumours.<ref name=pmid20472615>{{Cite journal  | last1 = Castillo | first1 = JG. | last2 = Silvay | first2 = G. | title = Characterization and management of cardiac tumors. | journal = Semin Cardiothorac Vasc Anesth | volume = 14 | issue = 1 | pages = 6-20 | month = Mar | year = 2010 | doi = 10.1177/1089253210362596 | PMID = 20472615 }}</ref>


==Primary heart tumours==
=Primary heart tumours=
*Approximately 10% of resected (primary) cardiac tumours are malignant.<ref name=pmid18350921>{{cite journal |author=Burke A |title=Primary malignant cardiac tumors |journal=Semin Diagn Pathol |volume=25 |issue=1 |pages=39-46 |year=2008 |month=February |pmid=18350921 |doi= |url=}}</ref>
*Approximately 10% of resected (primary) cardiac tumours are malignant.<ref name=pmid18350921>{{cite journal |author=Burke A |title=Primary malignant cardiac tumors |journal=Semin Diagn Pathol |volume=25 |issue=1 |pages=39-46 |year=2008 |month=February |pmid=18350921 |doi= |url=}}</ref>
**90% are sarcomas.
**90% are sarcomas.
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In order of frequency:
In order of frequency:
#Myxoma.
#[[Cardiac myxoma|Myxoma]].
#[[Lipoma]].
#[[Lipoma]].
#Papillary fibroelastoma.
#[[Papillary fibroelastoma]].


Notes:
Notes:
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Malignant heart tumours (in order of frequency):<ref name=pmid20472615>{{Cite journal  | last1 = Castillo | first1 = JG. | last2 = Silvay | first2 = G. | title = Characterization and management of cardiac tumors. | journal = Semin Cardiothorac Vasc Anesth | volume = 14 | issue = 1 | pages = 6-20 | month = Mar | year = 2010 | doi = 10.1177/1089253210362596 | PMID = 20472615 }}</ref>
Malignant heart tumours (in order of frequency):<ref name=pmid20472615>{{Cite journal  | last1 = Castillo | first1 = JG. | last2 = Silvay | first2 = G. | title = Characterization and management of cardiac tumors. | journal = Semin Cardiothorac Vasc Anesth | volume = 14 | issue = 1 | pages = 6-20 | month = Mar | year = 2010 | doi = 10.1177/1089253210362596 | PMID = 20472615 }}</ref>
#Undifferentiated.
#Undifferentiated.
#Angiosarcoma.
#[[Angiosarcoma]].
#Leiomyosarcoma.
#[[Leiomyosarcoma]].


Note:
Note:
*According to WMSP:<ref name=Ref_WMSP135>{{Ref WMSP|135}}</ref> Most common primary malignant tumour of the heart = angiosarcoma.
*According to WMSP:<ref name=Ref_WMSP135>{{Ref WMSP|135}}</ref> Most common primary malignant tumour of the heart = angiosarcoma.


==Myxoma==
=Specific entities=
==Cardiac myxoma==
{{Main|Cardiac myxoma}}
This includes the common ''atrial myxoma''.
 
==Papillary fibroelastoma==
*[[AKA]] ''fibroelastoma''.
**Should '''not''' be confused with ''[[elastofibroma]]''.
*[[AKA]] ''giant Lambl excrescence''.<ref name=pmid9767897>{{Cite journal  | last1 = Loire | first1 = R. | last2 = Pinède | first2 = L. | last3 = Donsbeck | first3 = AV. | last4 = Nighoghossian | first4 = N. | last5 = Perinetti | first5 = M. | title = [Papillary fibroelastoma of the heart (giant Lambl excrescence). Clinical-anatomical study on 10 surgically treated patients]. | journal = Presse Med | volume = 27 | issue = 16 | pages = 753-7 | month = Apr | year = 1998 | doi =  | PMID = 9767897 }}</ref>
 
===General===
===General===
*Uncommon.
*Usually an incidental finding.
*Clinical: may lead to cerebral infarction.<ref name=pmid3188128>{{cite journal |author=Knepper LE, Biller J, Adams HP, Bruno A |title=Neurologic manifestations of atrial myxoma. A 12-year experience and review |journal=Stroke |volume=19 |issue=11 |pages=1435-40 |year=1988 |month=November |pmid=3188128 |doi= |url=http://stroke.ahajournals.org/cgi/reprint/19/11/1435}}</ref>
*Diagnosed by imaging.
*May be familial, i.e. Carney complex ([[AKA]] NAME syndrome, [[AKA]] LAMB syndrome).<ref>{{Ref WMSP|135}}</ref>
**NAME = Nevi, Atrial myxoma, Myxoid neurofibroma, and Ephelides (freckles<ref>URL: [http://emedicine.medscape.com/article/1119293-overview http://emedicine.medscape.com/article/1119293-overview]. Accessed on: 7 January 2011.</ref>).
**LAMB = Lentigines, Atrial myxomas, Mucocutaneous myxomas, Blue nevi.
 
Most common presentations:<ref name=pmid3188128/>
*Dyspnea - 45%.
*Neurologic symptoms 36%.


===Gross===
===Gross===
Location:<ref name=pmid3188128/>
*Friable appearing.
*Usually atrial.
*Yellow.
*Usually left side.
*Typically on free edge.
 
Features:<ref name=pmid12006696>{{cite journal |author=Grebenc ML, Rosado-de-Christenson ML, Green CE, Burke AP, Galvin JR |title=Cardiac myxoma: imaging features in 83 patients |journal=Radiographics |volume=22 |issue=3 |pages=673-89 |year=2002 |pmid=12006696 |doi= |url=http://radiographics.rsna.org/content/22/3/673.long}}</ref>
*Lobular surface.
*Smooth surface.
 
===Microscopic===
Features:<ref name=pmid12006696/>
*Myxoid material - extra cellular (???) - '''key feature'''.
*Calcified elastic fibers - ''gamna bodies''.
*Hemorrhage common.
*Often covered by endothelium.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Atrial_myxoma_high_mag.jpg Atrial myxoma (wikimedia.org)].
*[http://commons.wikimedia.org/wiki/File:Atrial_myxoma_edge_high_mag.jpg Atrial myxoma - endothelial covering (wikimedia.org)].
*[http://commons.wikimedia.org/wiki/File:Atrial_myxoma_edge_low_mag.jpg Atrial myxoma - sharp border (wikimedia.org)].


==Fibroelastoma==
Image:
===General===
*[http://www.e-heart.org/Photos/09_Cardiac_Tumors_Photos/%C2%A9%20Papillary%20fibroelastoma%20-%20Gross.jpg Papillary fibroelastoma (e-heart.org)].<ref>URL: [http://www.e-heart.org/pages/09_Cardiac_Tumors/09_Cardiac_Tumors_Primary_Benign_Cardiac_Papillary%20Fibroelastoma_001.htm http://www.e-heart.org/pages/09_Cardiac_Tumors/09_Cardiac_Tumors_Primary_Benign_Cardiac_Papillary%20Fibroelastoma_001.htm]. 5 March 2013.</ref>
*AKA ''papillary fibroelastoma''.
*Usually an incidental finding.


===Microscopic===
===Microscopic===
Features:<ref>[http://www.pathologyoutlines.com/hearttumor.html http://www.pathologyoutlines.com/hearttumor.html]</ref>
Features:<ref>URL: [http://www.pathologyoutlines.com/hearttumor.html http://www.pathologyoutlines.com/hearttumor.html]. Accessed on: 16 May 2011.</ref>
*Braching papillary fronds which are:
*Braching papillary fronds which are:
**Composed of collagen, and
**Composed of collagen, and
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**Mucopolysaccharide.
**Mucopolysaccharide.


Micrographs:
====Images====
*[http://commons.wikimedia.org/wiki/File:Papillary_fibroelastoma.jpg Papillary fibroelastoma - low magnification (commons.wikimedia.org)].
<gallery>
*[http://commons.wikimedia.org/wiki/File:Papillary_fibroelastoma2.jpg Papillary fibroelastoma - intermediate magnification (commons.wikimedia.org)].
Image:Papillary_fibroelastoma.jpg | Papillary fibroelastoma - low mag. (WC)
Image:Papillary_fibroelastoma2.jpg | Papillary fibroelastoma - intermed. mag. (WC)
</gallery>


==Rhabdomyoma==
==Cardiac rhabdomyoma==
{{Main|Rhabdomyoma}}
*Very rare.
*Very rare.
*Benign.
*Benign.
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==Rhabdomyosarcoma==
==Rhabdomyosarcoma==
See ''[[soft tissue tumours]]''.
See ''[[rhabdomyosarcoma]]''.


==Lipoma==
==Lipoma==
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*Lipomatous hypertrophy.<ref name=pmid20196664>{{Cite journal  | last1 = Miller | first1 = DV. | last2 = Tazelaar | first2 = HD. | title = Cardiovascular pseudoneoplasms. | journal = Arch Pathol Lab Med | volume = 134 | issue = 3 | pages = 362-8 | month = Mar | year = 2010 | doi =  | PMID = 20196664 }}</ref>
*Lipomatous hypertrophy.<ref name=pmid20196664>{{Cite journal  | last1 = Miller | first1 = DV. | last2 = Tazelaar | first2 = HD. | title = Cardiovascular pseudoneoplasms. | journal = Arch Pathol Lab Med | volume = 134 | issue = 3 | pages = 362-8 | month = Mar | year = 2010 | doi =  | PMID = 20196664 }}</ref>


==Cystic AV node tumour==
==Cystic tumour of the atrioventricular nodal region==
===General===
{{Main|Cystic tumour of the atrioventricular nodal region}}
*Super rare.
*Usually 1-2 mm.
 
===Gross===
*"Bump" in the ''triangle of Koch''.
*Cystic spaces.


===Microscopic===
==Angiosarcoma==
Features:
[[File: Angiosarc heart 1 sl 1.png| Angiosarcoma of right atrium]]
*Cystic spaces lined by epithelial cells.
[[File: Angiosarc heart 1 sl 2.png| Angiosarcoma of right atrium]]
*+/-Psammoma bodies. (???)
[[File: Angiosarc heart 1 sl 3.png| Angiosarcoma of right atrium]]
[[File: Angiosarc heart 1 sl 4.png| Angiosarcoma of right atrium]]
[[File: Angiosarc heart 1 sl 5.png| Angiosarcoma of right atrium]]
[[File: Angiosarc heart 1 sl 6.png| Angiosarcoma of right atrium]]


===IHC===
Angiosarcoma of right atrium. A. Luminal clot tops tumor with blood filled spaces. B. Tumor replaces myocardium. C. Irregularly branched vessels, sometimes associated with extravasated erythrocytes. D. Irregularly aggregated and oriented spindle cells. E. Cells vary in size and shape much more so than an organizing thrombus or hemangioma. Note luminal tufts (green arrows), clustered large cancer nuclei with nucleoli (blue arrows). F. Cytoplasmic vacuoles, some with erythrocytes (arrows).
*CEA +ve.
*EMA +ve.
 
===Gross===
*Friable appearing.
*Yellow.
*Typically on free edge.


==See also==
=See also=
*[[Heart]].
*[[Heart]].
*[[Valvular heart disease]].
*[[Valvular heart disease]].


==References==
=References=
{{reflist|2}}
{{reflist|2}}


[[Category:Cardiovascular pathology]]
[[Category:Cardiovascular pathology]]
[[Category:Cardiac tumours]]

Latest revision as of 19:04, 24 March 2019

Cardiac tumours are rare buggers. They provide some work for cardiac surgeons.

Most common malignant

  • Metastases - most common cardiac tumour; 1:~100 primary tumours:secondary tumours.[1]

Primary heart tumours

  • Approximately 10% of resected (primary) cardiac tumours are malignant.[2]
    • 90% are sarcomas.
    • 10% are lymphomas.

In order of frequency:

  1. Myxoma.
  2. Lipoma.
  3. Papillary fibroelastoma.

Notes:

  • If one is considering only valves - papillary fibroelastoma is No. 1.

Malignant heart tumours (in order of frequency):[1]

  1. Undifferentiated.
  2. Angiosarcoma.
  3. Leiomyosarcoma.

Note:

  • According to WMSP:[3] Most common primary malignant tumour of the heart = angiosarcoma.

Specific entities

Cardiac myxoma

Main article: Cardiac myxoma

This includes the common atrial myxoma.

Papillary fibroelastoma

General

  • Usually an incidental finding.

Gross

  • Friable appearing.
  • Yellow.
  • Typically on free edge.

Image:

Microscopic

Features:[6]

  • Braching papillary fronds which are:
    • Composed of collagen, and
    • Avascular.
  • +/-Elastic tissue.
  • Surrounded by:
    • Endothelium, and
    • Mucopolysaccharide.

Images

  • Papillary fibroelastoma - low mag. (WC)

  • Papillary fibroelastoma - intermed. mag. (WC)

Cardiac rhabdomyoma

Main article: Rhabdomyoma

Rhabdomyosarcoma

See rhabdomyosarcoma.

Lipoma

Main article: Adipocytic tumours
  • Like lipomas elsewhere in the body.
  • Usually location: left ventricle, subendocardial.[3]

DDx:

  • Lipomatous hypertrophy.[8]

Cystic tumour of the atrioventricular nodal region

Main article: Cystic tumour of the atrioventricular nodal region

Angiosarcoma

Angiosarcoma of right atrium Angiosarcoma of right atrium Angiosarcoma of right atrium Angiosarcoma of right atrium Angiosarcoma of right atrium Angiosarcoma of right atrium

Angiosarcoma of right atrium. A. Luminal clot tops tumor with blood filled spaces. B. Tumor replaces myocardium. C. Irregularly branched vessels, sometimes associated with extravasated erythrocytes. D. Irregularly aggregated and oriented spindle cells. E. Cells vary in size and shape much more so than an organizing thrombus or hemangioma. Note luminal tufts (green arrows), clustered large cancer nuclei with nucleoli (blue arrows). F. Cytoplasmic vacuoles, some with erythrocytes (arrows).

See also

References

  1. ↑ 1.0 1.1 Castillo, JG.; Silvay, G. (Mar 2010). "Characterization and management of cardiac tumors.". Semin Cardiothorac Vasc Anesth 14 (1): 6-20. doi:10.1177/1089253210362596. PMID 20472615.
  2. ↑ Burke A (February 2008). "Primary malignant cardiac tumors". Semin Diagn Pathol 25 (1): 39-46. PMID 18350921.
  3. ↑ 3.0 3.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 135. ISBN 978-0781765275.
  4. ↑ Loire, R.; Pinède, L.; Donsbeck, AV.; Nighoghossian, N.; Perinetti, M. (Apr 1998). "[Papillary fibroelastoma of the heart (giant Lambl excrescence). Clinical-anatomical study on 10 surgically treated patients].". Presse Med 27 (16): 753-7. PMID 9767897.
  5. ↑ URL: http://www.e-heart.org/pages/09_Cardiac_Tumors/09_Cardiac_Tumors_Primary_Benign_Cardiac_Papillary%20Fibroelastoma_001.htm. 5 March 2013.
  6. ↑ URL: http://www.pathologyoutlines.com/hearttumor.html. Accessed on: 16 May 2011.
  7. ↑ Yinon, Y.; Chitayat, D.; Blaser, S.; Seed, M.; Amsalem, H.; Yoo, SJ.; Jaeggi, ET. (Aug 2010). "Fetal cardiac tumors: a single-center experience of 40 cases.". Prenat Diagn. doi:10.1002/pd.2590. PMID 20721876.
  8. ↑ Miller, DV.; Tazelaar, HD. (Mar 2010). "Cardiovascular pseudoneoplasms.". Arch Pathol Lab Med 134 (3): 362-8. PMID 20196664.
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