Difference between revisions of "Cardiac tumours"

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*Diagnosed by imaging.
*Diagnosed by imaging.
*May be familial, i.e. Carney complex ([[AKA]] NAME syndrome, [[AKA]] LAMB syndrome).<ref>{{Ref WMSP|135}}</ref>
*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.
**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.
**LAMB = Lentigines, Atrial myxomas, Mucocutaneous myxomas, Blue nevi.


Revision as of 19:30, 7 January 2011

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.

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.

Features:[7]

  • Lobular surface.
  • Smooth surface.

Microscopic

Features:[7]

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

Images:

Fibroelastoma

General

  • AKA papillary fibroelastoma.
  • Usually an incidental finding.

Microscopic

Features:[8]

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

Micrographs:

Rhabdomyoma

Rhabdomyosarcoma

See soft tissue tumours.

Lipoma

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

DDx:

  • Lipomatous hypertrophy.[10]

Cystic AV node tumour

General

  • Super rare.
  • Usually 1-2 mm.

Gross

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

Microscopic

Features:

  • Cystic spaces lined by epithelial cells.
  • +/-Psammoma bodies. (???)

IHC

  • CEA +ve.
  • EMA +ve.

Gross

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

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. ↑ 7.0 7.1 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.
  8. ↑ http://www.pathologyoutlines.com/hearttumor.html
  9. ↑ 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.
  10. ↑ Miller, DV.; Tazelaar, HD. (Mar 2010). "Cardiovascular pseudoneoplasms.". Arch Pathol Lab Med 134 (3): 362-8. PMID 20196664.
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