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, | **NAME = Nevi, Atrial myxoma, Myxoid neurofibroma, and Ephelides. | ||
**LAMB = Lentigines, Atrial myxomas, Mucocutaneous myxomas, Blue nevi. | **LAMB = Lentigines, Atrial myxomas, Mucocutaneous myxomas, Blue nevi. | ||
Revision as of 18:10, 7 January 2011
Cardiac tumours are rare buggers.
Most common (malignant)
- Metastases - most common cardiac tumour; 1:~100 primary tumours:secondary tumours.[1]
- Most common primary malignant tumour of the heart = angiosarcoma.[2]
Primary heart tumours
- Approximately 10% of resected (primary) cardiac tumours are malignant.[3]
- 90% are sarcomas.
- 10% are lymphomas.
In order of frequency:
- Myxoma.
- Lipoma.
- Papillary fibroelastoma.
Notes:
- If one is considering only valves - papillary fibroelastoma is No. 1.
Malignant heart tumours (in order of frequency):[1]
- Undifferentiated.
- Angiosarcoma.
- Leiomyosarcoma.
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.
- 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:[6]
- Lobular surface.
- Smooth surface.
Microscopic
Features:[6]
- Myxoid material - extra cellular (???) - key feature.
- Calcified elastic fibers - gamna bodies.
- Hemorrhage common.
- Often covered by endothelium.
Images:
- Atrial myxoma (wikimedia.org).
- Atrial myxoma - endothelial covering (wikimedia.org).
- Atrial myxoma - sharp border (wikimedia.org).
Fibroelastoma
General
- AKA papillary fibroelastoma.
- Usually an incidental finding.
Microscopic
Features:[7]
- Braching papillary fronds which are:
- Composed of collagen, and
- Avascular.
- +/-Elastic tissue.
- Surrounded by:
- Endothelium, and
- Mucopolysaccharide.
Micrographs:
- Papillary fibroelastoma - low magnification (commons.wikimedia.org).
- Papillary fibroelastoma - intermediate magnification (commons.wikimedia.org).
Rhabdomyoma
- Very rare.
- Benign.
- Associated with tuberous sclerosis.[8]
Rhabdomyosarcoma
See soft tissue tumours.
Lipoma
Main article: Adipocytic tumours
- Like lipomas elsewhere in the body.
- Usually location: left ventricle, subendocardial.[2]
DDx:
- Lipomatous hypertrophy.[9]
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
- ↑ 1.0 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.
- ↑ 2.0 2.1 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.
- ↑ Burke A (February 2008). "Primary malignant cardiac tumors". Semin Diagn Pathol 25 (1): 39-46. PMID 18350921.
- ↑ 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.
- ↑ 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.
- ↑ 6.0 6.1 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.
- ↑ http://www.pathologyoutlines.com/hearttumor.html
- ↑ 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.
- ↑ Miller, DV.; Tazelaar, HD. (Mar 2010). "Cardiovascular pseudoneoplasms.". Arch Pathol Lab Med 134 (3): 362-8. PMID 20196664.