For the atrial subtype, see Atrial myxoma. For the virus, see Myxoma virus.
"Myxomatous" redirects here. It is not to be confused with Myxomatosis.
| Myxoma |
|
Micrograph of an atrial myxoma. H&E stain.
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| Classification and external resources |
| Specialty |
oncology |
| ICD-10 |
D21.9 |
| ICD-9-CM |
212.7 |
| ICD-O |
M8840/0 |
| OMIM |
255960 |
| DiseasesDB |
30736 |
| eMedicine |
med/186 |
| MeSH |
D009232 |
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.
Contents
- 1 Location
- 2 Types[3]
- 3 Symptoms
- 4 Treatment
- 5 See also
- 6 References
- 7 External links
Location
- Atrial myxoma
- Cutaneous myxoma
- Odontogenic myxoma
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]
| 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, 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.^ 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]
- Dyspnea on exertion
- Paroxysmal nocturnal dyspnea
- Fever
- Weight loss (see cachexia)
- Lightheadedness or syncope (Loss of consciousness)
- Hemoptysis
- Sudden death
- Tachycardia or milder heartrate, i.e. 75 - 100 cycl/min
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.
See also
- Myxoid tumor
- Cutaneous myxoma
- Carney complex
- Myxomatosis
- Primary tumors of the heart
- Myxomatous degeneration
References
- ^ "Myxoma" at Dorland's Medical Dictionary
- ^ 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. doi:10.1161/01.str.19.11.1435. PMID 3188128.
- ^ Solitary superficial angiomyxoma: an infrequent but distinct soft tissue tumor. Satter. Journal of Cutaneous Pathology, Vol 36, Issue s1, pages 56-59.
- ^ Seino Y, Ikeda U, Shimada K. (1993). "Increased expression of interleukin 6 mRNA in cardiac myxomas". Br Heart J 69 (6): 565–7. doi:10.1136/hrt.69.6.565. PMC 1025174. PMID 8343326.
- ^ 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. doi:10.1002/art.1780330313. PMID 1690543.
- ^ 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. doi:10.1159/000015180. PMID 8281536.
- ^ Fisher J. (1983). "Cardiac myxoma". Cardiovasc Rev Rep (4): 1195–9.
External links
- -435814399 at GPnotebook
- 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)
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Connective/soft tissue tumors and sarcomas (ICD-O 8800–9059) (C45–C49/D17–D21, 171/214–215)
|
|
| Not otherwise specified |
- Soft-tissue sarcoma
- Desmoplastic small-round-cell tumor
|
|
| Connective tissue neoplasm |
| Fibromatous |
| Fibroma/fibrosarcoma: |
- Dermatofibrosarcoma protuberans
- Desmoplastic fibroma
|
|
| Fibroma/fibromatosis: |
- Aggressive infantile fibromatosis
- Aponeurotic fibroma
- Collagenous fibroma
- Diffuse infantile fibromatosis
- Familial myxovascular fibromas
- Fibroma of tendon sheath
- Fibromatosis colli
- Infantile digital fibromatosis
- Juvenile hyaline fibromatosis
- Plantar fibromatosis
- Pleomorphic fibroma
- Oral submucous fibrosis
|
|
| Histiocytoma/histiocytic sarcoma: |
- Benign fibrous histiocytoma
- Malignant fibrous histiocytoma
- Atypical fibroxanthoma
- Solitary fibrous tumor
|
|
|
| Myxomatous |
- Myxoma/myxosarcoma
- Cutaneous myxoma
- Superficial acral fibromyxoma
- Angiomyxoma
- Ossifying fibromyxoid tumour
|
|
| Fibroepithelial |
- Brenner tumour
- Fibroadenoma
- Phyllodes tumor
|
|
| Synovial-like |
- Synovial sarcoma
- Clear-cell sarcoma
|
|
|
| Lipomatous |
- Lipoma/liposarcoma
- Myelolipoma
- Myxoid liposarcoma
- PEComa
- Chondroid lipoma
- Intradermal spindle cell lipoma
- Pleomorphic lipoma
- Lipoblastomatosis
- Spindle cell lipoma
- Hibernoma
|
|
| Myomatous |
| general: |
|
|
| smooth muscle: |
|
|
| skeletal muscle: |
- Rhabdomyoma/rhabdomyosarcoma: Embryonal rhabdomyosarcoma
- Alveolar rhabdomyosarcoma
|
|
- Leiomyoma
- Angioleiomyoma
- Angiolipoleiomyoma
- Genital leiomyoma
- Leiomyosarcoma
- Multiple cutaneous and uterine leiomyomatosis syndrome
- Multiple cutaneous leiomyoma
- Neural fibrolipoma
- Solitary cutaneous leiomyoma
- STUMP
|
|
|
| Complex mixed and stromal |
- Adenomyoma
- Pleomorphic adenoma
- Mixed Müllerian tumor
- Mesoblastic nephroma
- Wilms' tumor
- Malignant rhabdoid tumour
- Clear-cell sarcoma of the kidney
- Hepatoblastoma
- Pancreatoblastoma
- Carcinosarcoma
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|
| Mesothelial |
- Mesothelioma
- Adenomatoid tumor
|
|
|
Index of muscle
|
|
| Description |
- Anatomy
- head
- neck
- arms
- chest and back
- diaphragm
- abdomen
- genital area
- legs
- Muscle tissue
- Physiology
|
|
| Disease |
- Myopathy
- Soft tissue
- Connective tissue
- Congenital
- abdomen
- muscular dystrophy
- Neoplasms and cancer
- Injury
- Symptoms and signs
|
|
| Treatment |
- Procedures
- Drugs
- anti-inflammatory
- muscle relaxants
|
|
|
|
Heart neoplasia (C38.0/D15.1, 164.1/212.7)
|
|
| Primary |
- Papillary fibroelastoma
- Rhabdomyoma
- Angiosarcoma
- Teratoma
- Cystic tumour of the atrioventricular nodal region
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| Other |
|
|
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Index of the heart
|
|
| Description |
- Anatomy
- Physiology
- Development
|
|
| Disease |
- Injury
- Congenital
- Neoplasms and cancer
- Other
- Symptoms and signs
- Blood tests
|
|
| Treatment |
- Procedures
- Drugs
- glycosides
- other stimulants
- antiarrhythmics
- vasodilators
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