Cardiac tumours
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:
Notes:
- If one is considering only valves - papillary fibroelastoma is No. 1.
Malignant heart tumours (in order of frequency):[1]
- Undifferentiated.
- Angiosarcoma.
- 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:
- Atrial myxoma (wikimedia.org).
- Atrial myxoma - endothelial covering (wikimedia.org).
- Atrial myxoma - sharp border (wikimedia.org).
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:
- Papillary fibroelastoma - low magnification (commons.wikimedia.org).
- Papillary fibroelastoma - intermediate magnification (commons.wikimedia.org).
Cardiac rhabdomyoma
Main article: Rhabdomyoma
- Very rare.
- Benign.
- Associated with tuberous sclerosis.[10]
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.[11]
Cystic tumour of the atrioventricular nodal region
General
- Super rare.[12]
- Usually 1-2 mm.
- May cause sudden cardiac death.[13]
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
- ↑ 1.0 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.
- ↑ Burke A (February 2008). "Primary malignant cardiac tumors". Semin Diagn Pathol 25 (1): 39-46. PMID 18350921.
- ↑ 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.
- ↑ 4.0 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.
- ↑ URL: http://emedicine.medscape.com/article/1119293-overview. Accessed on: 7 January 2011.
- ↑ 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.
- ↑ 8.0 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.
- ↑ URL: http://www.pathologyoutlines.com/hearttumor.html. Accessed on: 16 May 2011.
- ↑ 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.
- ↑ 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.
- ↑ 13.0 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.