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Difference between revisions of "Cardiac myxoma"
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Atrial_myxoma_edge_high_mag.jpg | |||
| Width = | |||
| Caption = Atrial myxoma. [[H&E stain]]. | |||
| Micro = myxoid material (extra cellular), myxoma cells (stellate, polygonal or spindled morphology; +/-multinucleated, inconspicuous [[nucleoli]]; abundant cytoplasm), calcified elastic fibers (''gamna bodies''), +/-hemorrhage, +/-endothelial covering, +/-ossification, +/-fibrosis | |||
| Subtypes = | |||
| LMDDx = [[myxofibrosarcoma]], [[myxoid liposarcoma]], [[papillary fibroelastoma]] | |||
| Stains = | |||
| IHC = | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = usu. atrium, usu. left side | |||
| Grossing = | |||
| Site = [[heart]] | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = +/-dyspnea, +/-neurologic symptoms | |||
| Prevalence = most common heart tumour, generally uncommon | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = | |||
| Other = | |||
| ClinDDx = other heart tumours | |||
}} | |||
'''Cardiac myxoma''' is the most common [[Cardiac tumours|tumour of the heart]]. They are typical in the atrium; thus, a synonym is '''atrial myxoma'''. | |||
==General== | |||
*Uncommon. | |||
*Clinical: may lead to cerebral infarction.<ref name=pmid3188128>{{cite journal |author=Knepper LE, Biller J, Adams HP, Bruno A |title=Neurologic manifestations of atrial myxoma. A 12-year experience and review |journal=Stroke |volume=19 |issue=11 |pages=1435-40 |year=1988 |month=November |pmid=3188128 |doi= |url=http://stroke.ahajournals.org/cgi/reprint/19/11/1435}}</ref> | |||
*Diagnosed by imaging. | |||
*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 (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. | |||
Most common presentations:<ref name=pmid3188128/> | |||
*Dyspnea - 45%. | |||
*Neurologic symptoms 36%. | |||
==Gross== | |||
Location:<ref name=pmid3188128/> | |||
*Usually atrial. | |||
*Usually left side ~60%.<ref name=pmid12006696>{{Cite journal | last1 = Grebenc | first1 = ML. | last2 = Rosado-de-Christenson | first2 = ML. | last3 = Green | first3 = CE. | last4 = Burke | first4 = AP. | last5 = Galvin | first5 = JR. | title = Cardiac myxoma: imaging features in 83 patients. | journal = Radiographics | volume = 22 | issue = 3 | pages = 673-89 | month = | year = | doi = | PMID = 12006696 }}</ref> | |||
Features:<ref name=pmid12006696>{{cite journal |author=Grebenc ML, Rosado-de-Christenson ML, Green CE, Burke AP, Galvin JR |title=Cardiac myxoma: imaging features in 83 patients |journal=Radiographics |volume=22 |issue=3 |pages=673-89 |year=2002 |pmid=12006696 |doi= |url=http://radiographics.rsna.org/content/22/3/673.long}}</ref> | |||
*Lobular surface. | |||
*Smooth surface. | |||
==Microscopic== | |||
Features:<ref name=pmid12006696/> | |||
*[[myxoid stroma|Myxoid]] material - extra cellular - '''key feature'''. | |||
*Myxoma cells:<ref name=pmid16508920>{{Cite journal | last1 = Orlandi | first1 = A. | last2 = Ciucci | first2 = A. | last3 = Ferlosio | first3 = A. | last4 = Genta | first4 = R. | last5 = Spagnoli | first5 = LG. | last6 = Gabbiani | first6 = G. | title = Cardiac myxoma cells exhibit embryonic endocardial stem cell features. | journal = J Pathol | volume = 209 | issue = 2 | pages = 231-9 | month = Jun | year = 2006 | doi = 10.1002/path.1959 | PMID = 16508920 }}</ref> | |||
**Stellate, polygonal or spindled morphology. | |||
**+/-Multinucleated. | |||
**Inconspicuous [[nucleoli]]. | |||
**Abundant cytoplasm. | |||
*Calcified elastic fibers - ''gamna bodies''. | |||
*Hemorrhage common. | |||
*Often covered by endothelium. | |||
*+/-Ossification. | |||
*+/-Fibrosis. | |||
DDx:<ref>{{Ref DCHH|79}}</ref> | |||
*[[Papillary fibroelastoma]] - not really in the histomorphologic [[differential diagnosis]]. | |||
*[[Myxofibrosarcoma]] - nuclear pleomorphism. | |||
*[[Myxoid liposarcoma]] - chickenwire vasculature. | |||
===Images=== | |||
<gallery> | |||
Image:Atrial_myxoma_high_mag.jpg | Atrial myxoma - high mag. (WC) | |||
Image:Atrial_myxoma_edge_high_mag.jpg | Atrial myxoma - endothelial covering - high mag. (WC) | |||
Image:Atrial_myxoma_edge_low_mag.jpg | Atrial myxoma - sharp border - low mag. (WC) | |||
</gallery> | |||
www: | |||
*[http://radiographics.rsna.org/content/22/3/673/F3.expansion.html Atrial myxoma (rsna.org)].<ref name=pmid12006696/> | |||
*[http://radiographics.rsna.org/content/22/3/673/F4.expansion.html Gamna bodies (rsna.org)].<ref name=pmid12006696/> | |||
==Sign out== | |||
<pre> | |||
MASS, LEFT ATRIUM, EXCISION: | |||
- MYXOMA. | |||
</pre> | |||
===Micro=== | |||
The sections show paucicellular myxoid material containing polygonal and spindled cells with eosinophilic myxoid cytoplasm, bland nuclei, inconspicuous nucleoli and focal multinucleation (myxoma cells). Hemosiderin-laden macrophages, calcified elastic fibres (gamna bodies) and scattered inflammatory cells are also present. There is no nuclear atypia. Mitotic activity is not evident. Several sections show fresh hemorrhage. The edge has a fibrotic rim and appears to be covered by endothelium. No cardiac muscle is identified. | |||
==See also== | |||
*[[Cardiac tumours]]. | |||
*[[Myxoid lesions]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Cardiovascular pathology]] | |||
[[Category:Cardiac tumours]] | |||