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Myxoma

myxoma, myxomatous mitral valve
A myxoma New Latin from Greek 'muxa' for mucus is a myxoid tumor of primitive connective tissue1 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

Contents

  • 1 Location
    • 11 Atrial myxoma
  • 2 Types3
  • 3 Symptoms
  • 4 Treatment
  • 5 Epidemiology
  • 6 See also
  • 7 References
  • 8 External links

Locationedit

  • Atrial myxoma
  • Cutaneous myxoma
  • Odontogenic myxoma

Atrial myxomaedit

Myxomas are usually located in either the left or right atrium of the heart; about 86 percent occur in the left atrium2

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

Types3edit

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 Vimentin; variable staining with CD34, factor XIIIA, SMA1, MSA2 and S-100 20–30%
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, EMAclarification needed3, 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^ SMA, smooth muscle actin 2^ MSA, muscle-specific actin 3^ EMA, epithelial membrane antigen

Symptomsedit

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 myxoma45 High levels of IL-6 may be associated with a higher risk of embolism of the myxoma6

Symptoms of a cardiac myxoma include:7

  • Dyspnea on exertion
  • Paroxysmal nocturnal dyspnea
  • Fever
  • Weight loss see cachexia
  • Lightheadedness or syncope Loss of consciousness
  • Hemoptysis
  • Sudden death
  • Tachycardia or milder heartrate, ie 75 - 100 cycl/min

Treatmentedit

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

Epidemiologyedit

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 alsoedit

  • Myxoid tumor
  • Cutaneous myxoma
  • Carney complex
  • Myxomatosis
  • Primary tumors of the heart
  • Myxomatous degeneration

Referencesedit

  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:101161/01str19111435 
  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 1025174  PMID 8343326 doi:101136/hrt696565 
  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:101002/art1780330313 
  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:101159/000015180 
  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:1017996/anc21_61 Retrieved May 30, 2017 CS1 maint: Explicit use of et al link

External linksedit

  • 03-031b at Merck Manual of Diagnosis and Therapy Home Edition
  • Toronto General Hospital Department of Anesthesia VIRTUAL TEE pathology section: Cardiac Myxoma with video

myxoma, myxoma histology, myxoma surgery, myxoma tumor, myxoma tumor of the heart, myxoma virus, myxomatosis, myxomatosis in rabbits, myxomatous, myxomatous mitral valve


Myxoma Information about

Myxoma


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    Myxoma beatiful post thanks!

    29.10.2014


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