From the Guidelines
The false statement about atrial myxoma is A) In most cases, it is autosomal dominant. Atrial myxoma is typically sporadic, occurring randomly in about 90-95% of cases rather than being inherited in an autosomal dominant pattern. Only 5-10% of cases are familial, occurring as part of Carney complex, which is indeed inherited in an autosomal dominant fashion. The other statements are true characteristics of atrial myxoma. Atrial myxomas can be associated with Raynaud's syndrome due to vasospasm triggered by tumor-secreted cytokines. They are also associated with connective tissue diseases, with some patients exhibiting symptoms resembling lupus or other autoimmune disorders. Embolic phenomena in the peripheries are a common and dangerous complication of atrial myxomas, as tumor fragments or thrombi formed on the tumor surface can break off and travel to various organs, causing stroke, limb ischemia, or other embolic manifestations 1. According to the most recent guidelines, surgical excision of atrial myxomas is recommended to prevent embolic events 1. The majority of cardiac tumors, including myxomas, are found incidentally and carry the threat of stroke or recurrent stroke, highlighting the importance of early detection and treatment 1. In terms of genetic inheritance, while some cardiac conditions are inherited in an autosomal dominant pattern, atrial myxoma is not typically one of them, with most cases being sporadic 1. Overall, the management of atrial myxoma focuses on preventing embolic complications and addressing associated conditions such as connective tissue diseases or Raynaud's syndrome.
From the Research
Atrial Myxoma Characteristics
- Atrial myxoma is the most common benign cardiac tumor 2.
- It can present with constitutional, embolic, and obstructive effects 2.
- The tumor can cause embolic phenomena in the peripheries, leading to serious consequences 2.
Genetic and Syndromic Associations
- Familial atrial myxomas may present as a component of the Carney complex, an autosomal dominant multiple neoplasia and lentiginosis syndrome [(3,4,5)].
- The Carney complex is characterized by spotty skin pigmentation, cardiac and cutaneous myxoma, and endocrine overactivity 4.
- Approximately 7% of cardiac myxomas are related to the Carney complex 5.
False Statement
- The statement "In most cases, it is autosomal dominant" is not entirely accurate, as while some cases of atrial myxoma are associated with the autosomal dominant Carney complex, not all cases are [(3,4,5)].
- Atrial myxoma can be associated with Raynaud's syndrome, connective tissue disease, and embolic phenomena in the peripheries, making option A the false statement [(2,4,5,6)].
Clinical Manifestations
- Atrial myxoma can present with a variety of symptoms, including shortness of breath, cough, and thromboembolic features [(2,6)].
- The diagnosis may be missed or delayed due to subclinical cardiac manifestations 6.