Myxoma

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Classification according to ICD-10
D15 Benign neoplasm of other and unspecified intrathoracic organs
D15.1 heart
ICD-O M8840 / 0
D21 Other benign neoplasms of connective and other soft tissues
ICD-10 online (WHO version 2019)
Right atrial myxoma after resection

The term myxoma describes a rare, benign tumor that has only a sparse vascular supply and contains spindle-shaped, fibroblastic cells. There are basically two types of myxoma: intramuscular and juxta-articular .

The best known is the myxoma of the heart, which is usually found in the left atrium ( atrial myxoma ) and leads to cardiac arrhythmias and blood clots ( embolism ). The Carney complex is a hereditary form of myxoma . Characteristic here is the appearance of myxomas not only in the heart, but also in the skin, breast, nerve tissue and thyroid gland.

Intramuscular myxoma

The intramuscular myxoma occurs mainly in adulthood (peak 40 and 70 years) in women in the large skeletal muscles near the trunk and appears there as a clearly defined tumor mass.

Juxta-articular myxoma

The juxta-articular myxoma occurs mainly in middle age near the large joints. Its cause is unknown. It usually does not cause any clinical symptoms and is usually found as an incidental finding in the histological work-up after synovectomy .

Myxoma of the heart

Large atrial myxoma extending mainly into the left atrium and haemodynamic effect.

Occurrence

In autopsy series, a myxoma is found in up to 0.3%. Women are about three times as likely to be affected as men. The maximum age is between 40 and 60 years of age.

localization

The tumor can occur in all four heart cavities, 85% of which is in the left atrium.

Symptoms

If the myxoma is in the left atrium, it can cause signs of mitral stenosis or mitral regurgitation , depending on whether it is obstructing or destroying the valve . If a pedunculated tumor only occasionally obstructs the mitral valve, this can lead to short-term collapse states. In addition, the appearance of a consuming systemic disease: fever, weight loss, cachexia , fatigue, anemia . Cardiac arrhythmias can present themselves to the patient as palpitations (heart stumbling). Otherwise, the myxoma often only becomes noticeable through embolism (see below).

pathology

Macroscopically, it is a spherical, polypoid or shaggy lump with a soft gelatinous consistency. Although the tumor can be confused histologically with a thrombus in organization, it is pathogenetically derived from a true neoplasm of undifferentiated endocardial mesenchymal cells . The tumors are on average 5–6 cm in size and usually have peduncles attached to the endocardium (rarely broad-based).

Complications

Cardiac arrhythmias occur most frequently (> 50%). Blood clots ( thrombus ) that form on the myxoma and can loosen are also dangerous . The blood clot is washed out with the blood (embolism in 25% of patients) and can lead to circulatory disorders in the brain ( stroke ) or to vascular occlusions in the legs. Acute pulmonary edema as a result of left heart failure and sudden cardiac death can occur in pulmonary embolism .

A myxoma can also obstruct the outflow of the pulmonary veins and develop pulmonary hypertension as a result .

treatment

The focus is on treating life-threatening complications such as cardiac arrhythmias and embolisms . In order to prevent further blood clots (thrombi) from forming, blood clotting is slowed down with medication ( anticoagulation ).

The myxoma is usually removed as part of a heart operation. A temporary cardiac arrest and the use of a heart-lung machine are necessary. During the operation, the myxoma and part of the heart wall are cut out. The hole in the heart wall is closed using a so-called patch plastic.

literature

Individual evidence

  1. ^ A b W. Mohr: Joint pathology: Historical bases, causes and developments of joint diseases and their pathomorphology. Springer, 2000, ISBN 3-642-57071-2 , p. 505. (online)
  2. ↑ a b c A. Dominikus et al.: Imaging diagnosis and therapy of soft tissue tumors: with pathological classification, nuclear medicine, interventional therapy. Georg Thieme Verlag, 2008, ISBN 978-3-13-143131-8 , pp. 55-56. (on-line)
  3. ↑ J. Bertherat: Carney complex (CNC). In: Orphanet J Rare Dis. 2006 Jun 6; 1, p. 21. PMID 16756677 , PMC 1513551 (free full text).
  4. ↑ Macroscopic image of an atrial myxoma on Patho Pic
  5. ^ Herbert Reindell , Helmut Klepzig: Diseases of the heart and the vessels. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition, ibid. 1961, p. 579 ( functional disorders and diseases that lead to an increase in pressure in the small circulation ).

Web links

Wiktionary: Myxom  - explanations of meanings, word origins, synonyms, translations