Cardiac tumours

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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

General

  • Uncommon.
  • Clinical: may lead to cerebral infarction.[4]
  • Diagnosed by imaging.
  • May be familial, i.e. Carney complex (AKA NAME syndrome, AKA LAMB syndrome).[5]
    • NAME = Nevi, Atrial myxoma, Myxoid neurofibroma, and Ephelides (freckles[6]).
    • LAMB = Lentigines, Atrial myxomas, Mucocutaneous myxomas, Blue nevi.

Most common presentations:[4]

  • Dyspnea - 45%.
  • Neurologic symptoms 36%.

Gross

Location:[4]

  • Usually atrial.
  • Usually left side ~60%.[7]

Features:[8]

  • Lobular surface.
  • Smooth surface.

Microscopic

Features:[8]

  • Myxoid material - extra cellular - key feature.
  • Calcified elastic fibers - gamna bodies.
  • Hemorrhage common.
  • Often covered by endothelium.

Images:

Papillary fibroelastoma

  • AKA fibroelastoma.

General

  • Usually an incidental finding.

Gross

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

Microscopic

Features:[9]

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

Micrographs:

Cardiac rhabdomyoma

Rhabdomyosarcoma

See soft tissue tumours.

Lipoma

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

DDx:

  • Lipomatous hypertrophy.[11]

Cystic tumour of the atrioventricular nodal region

General

Gross

  • "Bump" in the triangle of Koch.
  • Cystic spaces.

Microscopic

Features:[13]

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

Images:

IHC

  • CEA +ve.
  • EMA +ve.

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. 4.0 4.1 4.2 Knepper LE, Biller J, Adams HP, Bruno A (November 1988). "Neurologic manifestations of atrial myxoma. A 12-year experience and review". Stroke 19 (11): 1435-40. PMID 3188128. http://stroke.ahajournals.org/cgi/reprint/19/11/1435.
  5. 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.
  6. URL: http://emedicine.medscape.com/article/1119293-overview. Accessed on: 7 January 2011.
  7. Grebenc, ML.; Rosado-de-Christenson, ML.; Green, CE.; Burke, AP.; Galvin, JR.. "Cardiac myxoma: imaging features in 83 patients.". Radiographics 22 (3): 673-89. PMID 12006696.
  8. 8.0 8.1 Grebenc ML, Rosado-de-Christenson ML, Green CE, Burke AP, Galvin JR (2002). "Cardiac myxoma: imaging features in 83 patients". Radiographics 22 (3): 673-89. PMID 12006696. http://radiographics.rsna.org/content/22/3/673.long.
  9. URL: http://www.pathologyoutlines.com/hearttumor.html. Accessed on: 16 May 2011.
  10. 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.
  11. Miller, DV.; Tazelaar, HD. (Mar 2010). "Cardiovascular pseudoneoplasms.". Arch Pathol Lab Med 134 (3): 362-8. PMID 20196664.
  12. 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.
  13. 13.0 13.1 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.