Myxoma differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]Cafer Zorkun, M.D., Ph.D. [3] Ahmad Al Maradni, M.D. [4] Maria Fernanda Villarreal, M.D. [5]

Overview

Cardiac myxoma should be differentiated from other benign and malignant primary heart tumors including papillary fibroelastoma, lipoma, rhabdomyoma, and cardiac metastasis. Primarily, cardiac myxoma should be differentiated from other diseases that cause mitral valve obstruction and embolic events, such as thrombi or endocarditis.

Differentiating Myxoma from other Diseases

Cardiac myxoma should be differentiated from other cardiac tumors that present as a cardiac mass. The following are the differentials:[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43][44][45][46][47][48]

Site of Tumor Malignant Potential Type of Tumor Tissue of Origin Age of Presentation Location Morphology Signs and Symptoms MRI Findings
Systemic Manifestations Cardiac Manifestations Embolic Manifestations
Primary Cardiac Tumor Primary Benign Myxoma
  • Between third to sixth decade of life
  • 1-15 cm in diameter
  • Pedunculated
  • Polypoid
  • Smooth and lobulated
  • Villous and pappillary appearance associated with embolization
Rhabdomyoma
  • Striated Muscle
  • 1-3 cm in size
  • Yellow-gray color
  • Firm
  • Circumscribed lobulated
  • Majority multiple if associated with tuberous sclerosis (of those with no association, 50% are single)
  • Multiple masses isointense to muscle tissue on T1 images
  • Hyperintense on T2 images
Fibroma
  • Children (1/3rd in infants)
  • Solitary
  • Solitary mass
  • Low intensity on T2 weighted image
Fibroelastoma
  • < 1 cm in diameter
  • Solitary
  • Papillary
  • Flower-like appearance with multiple attachments to valve
  • Short pedicle
  • T1 and T2 weighted images show uniform intermediate signal intensity similar to myocardium
  • Homogeneous late gadolinium contrast enhancement
Hemangioma
  • < 1 year to 70 years
  • < 2 % of primary cardiac tumors
  • Polypoid
  • Encapsulated
  • Intermediate density on T1 images
  • Hypointense on T2 images
  • Multicystic enhancing lesion
  • Involvement of epicardium or pericardium
Lipoma
  • Between fourth to sixth decade of life
  • Epicardial or intramural lesion
  • High intensity on T1 weighted image
  • Drop out on fat saturation images
Paraganglioma
  • Average age of presentation is 11-13 years
  • 3-8 cm
  • Well-defined
  • Broad base
  • Encapsulated
  • Heterogeneous
  • Hypervascular
Atrioventricular Node Tumor
  • Average age of diagnosis is 38 years
  • Female to male ratio 3:1
  • Hypointense cardiac mass on standard imaging
  • Hyperintensity on late gadolinium enhancement (LGE) images with heterogeneous contrast enhancement
Lipomatous hypertrophy of the interatrial septum
  • > 60 years
-
Primary Malignant Fibrosarcoma
  • 20 to 80 years
  • Sessile or pedunculated protruding masses in ventricular cavities
  • Soft
  • Lobulated
  • Gelatinous
  • Heterogenous or isointense to myocardium on T1 weighted images
Angiosarcoma
  • 30 to 50 years
  • Arterial phase enhancement
Rhabdomyosarcoma
  • Most common primary sarcoma of children
  • Average age of presentation is 20 years
  • Multiple
  • Three types:
    • Embryonic
    • Pleomorphic
    • Alveolar
  • Intermediate-to-hypointensity compared with muscle on T1 images
  • Hyperintense on T2-weighted imaging with heterogeneous contrast enhancement
Lymphoma
  • 10 to 90 years
  • Males > females
  • Right sided mostly
  • Majority solitary (1/3rd multiple)
  • Hypointense on T1-weighted images and hyperintense on T2-weighted images
Secondary Cardiac Tumor Metastastatic Malignant Metastasis
  • Any age
  • Multiple


  • Differential diagnosis of intracardiac masses requires histological analysis and a direct assessment of the specific location (endocardial, myocardial, and pericardial). The table below summarizes the findings that differentiate cardiac myxoma from other conditions that are also intracavitary cardiac masses and cause embolic events:
Disease Findings
Papillary fibroelastoma Is a rare benign primary tumor of the heart that typically involves one of the valves of the heart. Presents with syncope, chest pain, myocardial infarction, stroke and sudden cardiac death. They are the third most common type of primary tumor of the heart. There is a slightly increased male predilection.[49][50]
Lipoma Also known as "Lipomatous hypertrophy (LHIS)" is a very rare benign primary cardiac neoplasm. It is the proliferation of mature adipocytes. Lipomas are typically found in adult patients but can affect all ages. A common clinical feature is arrythmias.[51]
Rhabdomyoma Is the most common primary cardiac tumor in children. It is conformed from hamartomatous accumulation of the striated cardiomyocytes. Rhabdomyomas occur with more frequency in the myocardium of the left ventricle or in the interventricular septum. Is commonly associated with tuberous sclerosis, and congenital defects of the heart.[52]
Thrombus Intracardiac thrombi, can occur when there is an elevated risk of blood clots developing, such as: atrial fibrillation (atrial thrombi predominate), mitral stenosis, heart valve replacement and myocardial infarction (ventricular thrombus formation).[53]
Metastasis Secondary malignant tumor of the heart, arising by lymphatic or hematogenous spread of a primary neoplasm. Presents with dyspnea, congestive heart failure, hypotension and malignant pericardial effusion. Any primary malignancy may metastasize to the heart, however, lung cancer is among the most common.[54]

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  49. Papillary fibroelastoma of the heart. Dr Craig Hacking et al http://radiopaedia.org/articles/papillary-fibroelastoma-of-the-heart Accessed on November 24, 2015
  50. Papillary fibroelastoma .Wikipedia https://en.wikipedia.org/wiki/Papillary_fibroelastoma Accessed on November 24, 2015
  51. Lipoma. Dr Craig Hacking et al . Radiopedia. http://radiopaedia.org/articles/cardiac-lipoma Accessed on November 24, 2015
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