Close menu

Myxoma


Source: http://en.wikipedia.org/wiki/Myxoma
Updated: 2017-05-30T21:31Z
Myxoma
Atrial myxoma high mag.jpg
Micrograph of an atrial myxoma. H&E stain.
Classification and external resources
Specialty{{#statements:P1995}}
ICD-10D21.9
ICD-9-CM212.7
ICD-OM8840/0
OMIM255960
DiseasesDB30736
eMedicinemed/186
Patient UKMyxoma
MeSHD009232
[[[d:Lua error in Module:Wikidata at line 288: invalid escape sequence near '"^'.|edit on Wikidata]]]

A myxoma (New Latin from Greek 'muxa' for mucus) is a myxoid tumor of primitive connective tissue.[1] It is the most common primary tumor of the heart in adults, but can also occur in other locations.

Animated image of an MRI of the heart, showing a large myxoma plunging to and fro from atrium to ventricle across the mitral valve.
A Myxoma. A gelatinous tumor can be seen attached by a narrow pedicle to the atrial septum. The myxoma has an irregular surface and nearly fills the left atrium.

Location

Atrial myxoma

Myxomas are usually located in either the left or right atrium of the heart; about 86 percent occur in the left atrium.[2]

Myxomas are typically pedunculated, with a stalk that is attached to the interatrial septum. The most common location for attachment of the stalk is the fossa ovalis region of the interatrial septum.

An atrial myxoma may create an extra heart sound, audible to auscultation just after S2 It is most seen on echocardiography, as a pedunculated mass that is heterogeneous in appearance. A left atrial myxoma will cause an increase in pulmonary capillary wedge pressure.

The differential diagnosis include other cardiac tumors such as lipomas and rhabdomyomas (and rarely teratomas). These other tumors of the heart are typically not pedunculated, however, and are more likely to infiltrate the muscle of the heart. Cardiac magnetic resonance imaging (MRI) can help non-invasively diagnose cardiac tumors. However, diagnosis usually requires examination of a tissue sample by a pathologist.

Types[3]

MyxomaMarginVascular patternCellularityStromaStaining characteristicsRecurrence rateImage (see Histology)
Cutaneous myxoma or Superficial angiomyxomaPoor to moderately circumscribed, multilobularScattered thin-walled vesselsModerately cellular, bland spindled and stellate cells, variable inflammatory cell infiltrateAbundant mucin with clefts. Up to 30% have an associated epithelial componentVimentin; variable staining with CD34, factor XIIIA, SMA1, MSA2 and S-10020–30%
Cutaneous Myxoma
Intramuscular myxomaPoorly circumscribed merges with surrounding muscleHypovascular variant; hypervascular variantHypocellular variant; hypercellular variant; bland spindle cellsAbundant mucin with cystic spaces. Hypercellular variant has strands of collagenVimentin; variable staining with actin, desmin, CD34None
IntramuscularMyxoma.JPG
Juxta-articular myxomaPoorly circumscribed infiltrates surrounding tissueFocally vascularFocally hypercellular, peripheral spindle cells with occasional atypical cells and mitosesAbundant mucin, 89% of cases contain cystic spaces lined by fibrin or collagenVimentin; variable staining with actin, desmin, CD3434%
Aggressive angiomyxomaInfiltrativeUniformly distributed medium-sized blood vessels often with prominent hyalinizationLow to moderately cellular, evenly distributed round, spindled or stellate cellsLoose myxoid to focally collagenousVimentin, desmin, SMA1, MSA2, estrogen and progesterone receptor36–72%
AngiomyofibroblastomaWell circumscribedAbundant thin-walled blood vesselsAlternating hypercellular and hypocellular areas, perivascular condensations of spindled to epithelioid stromal cellsCollagenous to edematous with minimal mucinVimentin, desmin, CD34, estrogen and progesterone receptorNo recurrences reported, but rare cases of sarcomatous degeneration
Superficial acral fibromyxomaPushing to infiltrativeMild to moderately accentuated vasculatureModerately cellular, spindle and stellate cells with a storiform to fascicular pattern, variable mast cellsMyxoid to collagenousCD34, EMA[clarification needed]3, CD99Recurrence rare and primarily for incompletely excised lesions
Neurothekeoma (Nerve sheath myxoma)Well circumscribed, multilobularHypovascularModerately cellular, spindled cells in fascicles and whorlsNests of cells separated by collagenous bundlesS-100, EMA347% if incompletely excised
Neurothekeoma2.JPG

1.^ SMA, smooth muscle actin. 2.^ MSA, muscle-specific actin. 3.^ EMA, epithelial membrane antigen.

Symptoms

Symptoms associated with cardiac myxomas are typically due to the effect of the mass of the tumor obstructing the normal flow of blood within the chambers of the heart. Because pedunculated myxomas are somewhat mobile, symptoms may only occur when the patient is in a particular position.

Some symptoms of myxoma may be associated with the release of interleukin 6 (IL-6) by the myxoma.[4][5] High levels of IL-6 may be associated with a higher risk of embolism of the myxoma.[6]

Symptoms of a cardiac myxoma include:[7]

Treatment

Myxomas are usually removed surgically. The surgeon removes the myxoma, along with at least 5 surrounding millimeters of atrial septum. The septum is then repaired, using material from the pericardium.

Epidemiology

Cardiac myxomas predominantly appear in females in their 30s to 40s. Myxomas are the most common primary cardiac tumor affecting adults, accounting for one quarter to half of primary cardiac tumors seen in clinical practice. [8]

See also

References

  1. ^ "Myxoma" at Dorland's Medical Dictionary
  2. ^ Knepper LE, Biller J, Adams HP, Bruno A (1988). "Neurologic manifestations of atrial myxoma. A 12-year experience and review". Stroke. 19 (11): 1435–40. PMID 3188128. doi:10.1161/01.str.19.11.1435. 
  3. ^ Solitary superficial angiomyxoma: an infrequent but distinct soft tissue tumor. Satter. Journal of Cutaneous Pathology, Vol 36, Issue s1, pages 56-59.
  4. ^ Seino Y, Ikeda U, Shimada K (1993). "Increased expression of interleukin 6 mRNA in cardiac myxomas". Br Heart J. 69 (6): 565–7. PMC 1025174Freely accessible. PMID 8343326. doi:10.1136/hrt.69.6.565. 
  5. ^ Jourdan M, Bataille R, Seguin J, Zhang XG, Chaptal PA, Klein B (1990). "Constitutive production of interleukin-6 and immunologic features in cardiac myxomas". Arthritis Rheum. 33 (3): 398–402. PMID 1690543. doi:10.1002/art.1780330313. 
  6. ^ Wada A; Kanda t; Hayashi R; et al. (1993). "Cardiac myxoma metastasized to the brain: potential role of endogenous interleukin-6". Cardiology. 83 (3): 208–11. PMID 8281536. doi:10.1159/000015180. 
  7. ^ Fisher J. (1983). "Cardiac myxoma". Cardiovasc Rev Rep (4): 1195–9. 
  8. ^ Takahashi, Ayaka; et al. (May 2016). "Multimodal Cardiovascular Imaging of Cardiac Tumors". Annals of Nuclear Cardiology. 2 (1): 61–67. doi:10.17996/anc.2.1_61. Retrieved May 30, 2017. 

External links

Text is available under the Creative Commons Attribution-ShareAlike License; additional terms may apply. By using this site, you agree to the Terms of Use and Privacy Policy. Wikipedia is a registered trademark of the Wikimedia Foundation, Inc., a non-profit organization.

Also On Wow

    Advertisement