Difference between revisions of "Cardiac tumours"

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==Papillary fibroelastoma==
==Papillary fibroelastoma==
*[[AKA]] ''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>
*[[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>


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==Rhabdomyosarcoma==
==Rhabdomyosarcoma==
See ''[[soft tissue tumours]]''.
See ''[[rhabdomyosarcoma]]''.


==Lipoma==
==Lipoma==
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==Cystic tumour of the atrioventricular nodal region==
==Cystic tumour of the atrioventricular nodal region==
===General===
{{Main|Cystic tumour of the atrioventricular nodal region}}
*Super 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>
*Usually 1-2 mm.
*May cause [[Sudden_natural_death|sudden cardiac death]].<ref name=pmid10722558/>
 
===Gross===
*"Bump" in the ''triangle of Koch''.
*Cystic spaces.


===Microscopic===
==Angiosarcoma==
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>
[[File: Angiosarc heart 1 sl 1.png| Angiosarcoma of right atrium]]
*Cystic spaces lined single layer of epithelial cells.
[[File: Angiosarc heart 1 sl 2.png| Angiosarcoma of right atrium]]
*+/-Focal mononuclear inflammation.
[[File: Angiosarc heart 1 sl 3.png| Angiosarcoma of right atrium]]
*+/-[[Psammoma bodies]].
[[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]]


====Images====
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).
<gallery>
Image:Cystic_tumour_of_the_atrioventricular_nodal_region_-_very_low_mag.jpg | CTAVNR - very low mag. (WC)
Image:Cystic_tumour_of_the_atrioventricular_nodal_region_-_high_mag.jpg | CTAVNR - high mag. (WC)
</gallery>
===IHC===
*CEA +ve.
*EMA +ve.


=See also=
=See also=

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

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

Cardiac rhabdomyoma

Rhabdomyosarcoma

See rhabdomyosarcoma.

Lipoma

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

DDx:

  • Lipomatous hypertrophy.[8]

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.