ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (3)
Atrial myxoma
Other Resources UpToDate PubMed

Atrial myxoma

Contributors: Nishit Biniwale MD, Marie Crisel B. Erfe MD, Susan Burgin MD, Bipul Baibhav MD, FACC, FASE
Other Resources UpToDate PubMed

Synopsis

Cardiac myxoma is the most common benign primary tumor of the heart, representing 75% of all primary cardiac neoplastic masses. It is usually diagnosed in adults between the third and sixth decade of life and is more common in women. It can rarely occur in pediatric patients.

Location: 75%-80% of cardiac myxomas are located in the left atrium, 15%-20% are located in the right atrium, and 10% are either biatrial or in the ventricles. Myxomas most commonly develop from the interatrial septum but can also originate from the posterior and anterior atrial walls or the atrial appendage.

Clinical symptoms are variable and are depend on tumor size, location, and mobility. A classic triad of intracardiac obstruction, extracardiac embolism, and systemic / constitutional complaints has been described.

Most cases arise sporadically. Familial cases have been associated with the occurrence of multiple and atypical tumors. Complex familial tumors are part of a rare condition called the Carney complex. Carney complex may manifest with adrenocortical involvement, pituitary-related symptoms, abnormal pigmentation, or multiple neoplasias in the skin, thyroid, testicles, breast, and neural systems. Heterozygous mutation in the PRKAR1A gene, a protein kinase A regulatory subunit-1-alpha gene, on chromosome 17q22-24 has been linked to cases of intracardiac myxoma and to Carney complex type 1. Carney complex type 2 involves a locus at chromosome 2p16, although the gene responsible remains unknown.

Cardiac myxomas can grow rather quickly. Recurrence is thought to be due to inadequate resection, multicentric growth, inheritance, or metastasis and usually happens during the first 4 years postexcision. Sporadic cases almost never recur, while incidence of recurrence for familial cases, Carney complex, and multiple myxomas are 10%, 22%, and 33%, respectively.

Codes

ICD10CM:
D15.1 – Benign neoplasm of heart

SNOMEDCT:
233854003 – Atrial myxoma

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Intracardiac thrombus is the most common differential diagnosis. Cardiac magnetic resonance imaging (CMR) is helpful in discerning thrombus from myxoma.

Benign primary cardiac tumors:
  • Papillary fibroelastomas – Second most common benign cardiac tumor; occurs predominately in elderly patients (> 80 years). Usually < 1 cm in diameter, sessile pedunculated tumors attached to the mitral valve or semilunar valves; occurs exclusively on endocardium.
  • Rhabdomyomas – Most common cardiac tumor in children. Often multiple masses located in the ventricular myocardium; 50% of cases are associated with tuberous sclerosis.
  • Fibromas – Congenital tumors seen in children. Usually solitary, fibrous, and calcified; located in the myocardium.
  • Hemangiomas – Highly vascularized.
  • Lipomas – Encapsulated tumors usually originating from the epicardium.
  • Paragangliomas
  • Atrioventricular node tumor – Located at the base of the atrial septum.
Malignant primary cardiac tumors tend to be large, lobular, and broad based, with ill-defined borders, and show evidence of tissue invasion, necrosis, and calcifications:
  • Angiosarcoma – Highly invasive; usually with evidence of lung, brain, and/or liver metastasis upon diagnosis.
  • Rhabdomyosarcoma – Usually diagnosed in children; highly aggressive and may occur in any heart chamber.
  • Osteosarcoma – Bulky, sessile tumors usually located in the left atrium.
  • Leiomyosarcoma – Highly invasive; sessile masses with mucoid surface.
  • Lymphoma – Associated with lymphoproliferative conditions such as AIDS and with immunocompromised patients.
In addition:
  • Metastatic tumors – From direct extension from tumors in the mediastinum; hematogenous or lymphatic spread; more commonly affects the pericardium or myocardium.
  • Valvular vegetations
  • Right atrial masses – Prominent eustachian valve; Chiari network, aneurysm of the interatrial septum.

    Best Tests

    Subscription Required

    Management Pearls

    Subscription Required

    Therapy

    Subscription Required

    References

    Subscription Required

    Last Reviewed:03/09/2020
    Last Updated:03/17/2020
    Copyright © 2021 VisualDx®. All rights reserved.
    Atrial myxoma
    Atrial myxoma : Fatigue, Fever, Skin petechiae, Acral distribution, Heart murmur, Splinter hemorrhage, Arthralgia, Dyspnea, Erythematous macules
    Clinical image of Atrial myxoma
    Copyright © 2021 VisualDx®. All rights reserved.