Myxoma

Source: Wikipedia, the free encyclopedia.
For the atrial subtype, see
Atrial myxoma. For the virus, see Myxoma virus
.
"Myxomatous" redirects here. Not to be confused with Myxomatosis.
Myxoma
atrial myxoma. H&E stain.
SpecialtyOncology Edit this on Wikidata

A myxoma (

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

Types

Table below:[2]

Myxoma Margin Vascular pattern Cellularity
Stroma
 Staining characteristics Recurrence rate Image (see Histology)
Cutaneous myxoma or Superficial angiomyxoma Poor to moderately circumscribed, multilobular Scattered thin-walled vessels Moderately cellular, bland spindled and stellate cells, variable inflammatory cell infiltrate Abundant mucin with clefts. Up to 30% have an associated epithelial component
S-100
 20–30%
Cutaneous Myxoma
Cutaneous Myxoma
Intramuscular myxoma Poorly circumscribed merges with surrounding muscle Hypovascular variant; hypervascular variant Hypocellular variant; hypercellular variant; bland spindle cells Abundant mucin with cystic spaces. Hypercellular variant has strands of collagen Vimentin; variable staining with actin, desmin, CD34 None
Juxta-articular myxoma Poorly circumscribed infiltrates surrounding tissue Focally vascular Focally hypercellular, peripheral spindle cells with occasional atypical cells and mitoses Abundant mucin, 89% of cases contain cystic spaces lined by fibrin or collagen Vimentin; variable staining with actin, desmin, CD34 34%
Aggressive
angiomyxoma
 Infiltrative Uniformly distributed medium-sized blood vessels often with prominent hyalinization Low to moderately cellular, evenly distributed round, spindled or stellate cells Loose myxoid to focally collagenous Vimentin, desmin, SMA1, MSA2, estrogen and progesterone receptor 36–72%
Angiomyofibroblastoma Well circumscribed Abundant thin-walled blood vessels Alternating hypercellular and hypocellular areas, perivascular condensations of spindled to epithelioid
stromal cells
 Collagenous to edematous with minimal mucin Vimentin, desmin, CD34, estrogen and progesterone receptor No recurrences reported, but rare cases of sarcomatous degeneration
Superficial acral fibromyxoma Pushing to infiltrative Mild to moderately accentuated vasculature Moderately cellular, spindle and stellate cells with a
storiform
to fascicular pattern, variable mast cells 		Myxoid to collagenous CD34,
EMA[clarification needed]3, CD99
 Recurrence rare and primarily for incompletely excised lesions
Neurothekeoma (Nerve sheath myxoma) Well circumscribed, multilobular Hypovascular Moderately cellular, spindled cells in fascicles and whorls Nests of cells separated by collagenous bundles S-100, EMA3 47% if incompletely excised

1.

epithelial membrane antigen
.

Symptoms and signs

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.[3][4] High levels of IL-6 may be associated with a higher risk of embolism of the myxoma.[5]

Symptoms of a cardiac myxoma include:[6]

Location

MRI
of the heart, showing a large myxoma plunging to and fro from atrium to ventricle across the mitral valve.

Ocular myxoma

Myxoma is a rare, benign stromal tumor of mesenchymal origin often confused with other conjunctival stromal tumors. Conjunctival myxomas are thought to originate in

ocular surface squamous neoplasia (OSSN), or amelanotic melanoma.[7]
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.

Atrial myxoma

Myxomas are usually located in either the

left atrium.[8]

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.[9]

An

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.[citation needed
]

The

pathologist.[citation needed
]

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.[citation needed]

Epidemiology

Cardiac myxomas predominantly appear in females in their 30s to 40s. Myxomas are the most common primary cardiac tumor affecting adults,[9] accounting for one quarter to half of primary cardiac tumors seen in clinical practice.[10]

See also

References

  1. ^ "Myxoma" at Dorland's Medical Dictionary
  2. S2CID 1528140
    .
  3. PMID 8343326
    .
  4. PMID 1690543
    .
  5. S2CID 22543670
    .
  6. ^ Fisher J. (1983). "Cardiac myxoma". Cardiovasc Rev Rep (4): 1195–9.
  7. PMID 30355886
    .
  8. PMID 3188128
    .
  9. ^
    ISBN 9783030478872
    .
  10. doi:10.17996/anc.2.1_61
    .

External links
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