Rhabdomyoma differential diagnosis: Difference between revisions

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! align="center" style="background:#4479BA; color: #FFFFFF;" + |Embolic Manifestations
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Embolic Manifestations
|-
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| rowspan="13" |Primary Cardiac Tumor
| rowspan="13" |
| rowspan="9" |Primary Benign
=== Primary Cardiac Tumor ===
|Myxoma
| rowspan="9" |
==== Primary Benign ====
|'''[[Myxoma]]'''
|
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* Mesenchymal
* [[Mesenchymal]]
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* Between third to sixth decade of life
* Between third to sixth decade of life
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* Left atrium (75%)
* [[Left atrium]] (75%)
* Right atrium (15-20%)
* [[Right atrium]] (15-20%)
* Arise from border of fossa ovalis
* Arise from border of [[fossa ovalis]]
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* 1-15 cm in diameter
* 1-15 cm in diameter
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* Polypoid
* Polypoid
* Smooth and lobulated
* Smooth and lobulated
* Villous and pappillary appearance associated with embolization
* Villous and pappillary appearance associated with [[embolization]]
|
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* Constitutional symptoms
* Constitutional symptoms
* Carney complex:
* [[Carney complex]]:
** Spotty skin pigmentation
** Spotty skin pigmentation
** Endocrinal tumors such as sertoli testicular tumors, pituitary adenomas, thyroid tumors
** [[Endocrine tumors|Endocrinal tumors]] such as [[Sertoli Cell Tumor|sertoli testicular tumors]], [[pituitary adenomas]], [[Thyroid tumor|thyroid tumors]]
** Schwanomma
** [[Schwannoma]]
* NAME syndrome:
* NAME syndrome:
** Nevi
** [[Nevi]]
** Atrial myxoma
** [[Atrial myxoma]]
** Myxoid neurofibroma
** Myxoid neurofibroma
** Ephelides
** Ephelides
* LAMB syndrome:
* LAMB syndrome:
** Lentigo (small brown patches on the skin)
** [[Lentigo]] (small brown patches on the [[skin]])
** Atrial myxoma
** [[Atrial myxoma]]
** Blue nevi
** [[Nevi|Blue nevi]]
|
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* Dyspnea
* [[Dyspnea]]
* Mitral regurgitation (LA)
* [[Mitral regurgitation]] (LA)
* Right heart failure (RA)
* [[Right heart failure]] (RA)
* Cardiomegaly
* [[Cardiomegaly]]
|
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* Pulmonary hypertension and pulmonary emboli (RA)
* [[Pulmonary hypertension]] and [[pulmonary emboli]] (RA)
|
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* Oval mobile left atrial lesion
* Oval mobile left [[atrial]] lesion
* Heterogenous enhancement
* Heterogenous enhancement
* Attachment to fossa ovalis
* Attachment to [[fossa ovalis]]
|-
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|Rhabdomyoma
|'''Rhabdomyoma'''
|
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* Striated Muscle
* Striated Muscle
|
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* Majority (80%) in infants (<12 months)
* Majority (80%) in [[infants]] (<12 months)
* Most common cardiac mass in childhood
* Most common cardiac mass in childhood
* 50-70% of all pediatric tumors
* 50-70% of all [[pediatric]] [[tumors]]
|
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* Ventricles
* [[Ventricles]]
* Interventricular septum
* [[Interventricular septum]]
|
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* 1-3 cm in size
* 1-3 cm in size
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* Firm
* Firm
* Circumscribed lobulated
* Circumscribed lobulated
* Majority multiple if associated with tuberous sclerosis (of those with no association, 50% are single)
* Majority multiple if associated with [[tuberous sclerosis]] (of those with no association, 50% are single)
|
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* Associated with tuberous sclerosis:
* Associated with [[tuberous sclerosis]]:
** Developmental delay
** [[Developmental delay]]
** Renal tumors (angiomyolipomas'')''
** Renal tumors ([[Angiomyolipoma|angiomyolipomas]]'')''
** Phakomas (white retinal spots)
** [[Phakomatoses|Phakomas]] (white retinal spots)
** Hypomelanic macules (ash leaf spots)
** Hypomelanic macules (ash leaf spots)
** Ungal fibromas
** Ungal fibromas
** Facial angiofibromas
** Facial [[Angiofibroma|angiofibromas]]
** Headache
** [[Headache]]
** Blurred vision
** [[Blurred vision]]
** Arrhythmias
** [[Cardiac arrhythmia|Arrhythmias]]
** Benign brain tumors (cortical tubers, subependymal nodules)  
** [[Benign]] [[Brain tumor|brain tumors]] ([[Cerebral cortex|cortical]] tubers, [[Subependymal zone|subependymal]] nodules)  
|
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* Outflow obstruction (both right and left ventricles)
* Outflow obstruction (both [[Ventricle|right and left ventricles]])
* Arrhythmias (ventricular pre-excitation, Wolff-Parkinson White syndrome)
* [[Cardiac arrhythmia|Arrhythmias]] (ventricular pre-excitation, [[Wolff-Parkinson-White syndrome|Wolff-Parkinson White syndrome]])
* Spontaneous regression in young cases (< 4 years); seldom regress in adult cases
* Spontaneous regression in young cases (< 4 years); seldom regress in adult cases
|
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* [[Pulmonary embolism]]
* [[Ischemic stroke]]
|
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* Multiple masses isointense to muscle tissue on T1 images
* Multiple masses isointense to [[muscle tissue]] on T1 images
* Hyperintense on T2 images
* Hyperintense on T2 images
|-
|-
|Fibroma
|'''[[Fibroma]]'''
|
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* Fibrous
* [[Fibrous]]
|
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* Children (1/3rd in infants)
* Children (1/3rd in infants)
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* Ventricles
* [[Ventricles]]
* Ventricular septum >Left ventricular free wall > Right ventricle > Atria
* [[Interventricular septum|Ventricular septum]] >[[Left ventricular|Left ventricular free wall]] > [[Right ventricle]] > [[Atria]]
* Central calcification
* Central [[calcification]]
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* Solitary  
* Solitary  
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* Cyanosis
* [[Asymptomatic]]
* [[Cyanosis]]
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* Atypical chest pain
* Atypical [[chest pain]]
* Heart failure
* [[Heart failure]]
* Cardiomegaly
* [[Cardiomegaly]]
* Biventricular hypertrophy
* Biventricular hypertrophy
* Atrioventricular nodal block
* [[Atrioventricular block|Atrioventricular nodal block]]
|
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* [[Pulmonary embolism]]
* [[Ischemic stroke]]
|
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* Solitary mass
* Solitary mass
* Low intensity on T2 weighted image
* Low intensity on T2 weighted image
|-
|-
|Fibroelastoma
|'''Fibroelastoma'''
|
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* Valvular endocardium
* Valvular [[endocardium]]
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|
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* Valvular endocardium (Adults-Aortic, children-Tricuspid)
* Valvular [[endocardium]] (Adults-[[Aortic valve|Aortic]], children-[[Tricuspid valve|Tricuspid]])
* Ventrucular surface of aortic and pulmonary valves and atrial surface of atriventricular valves
* [[Ventricular]] surface of [[aortic]] and [[pulmonary valves]] and [[atrial]] surface of [[atrioventricular valves]]
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* < 1 cm in diameter
* < 1 cm in diameter
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*  
*  
|
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* Asymptomatic
* [[Asymptomatic]]
* Association with hypertrophic obstructive cardiomyopathy (HOCM)
* Association with [[hypertrophic obstructive cardiomyopathy]] ([[Hypertrophic cardiomyopathy|HOCM]])
|
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* Subacute bacterial endocarditis
* [[Subacute bacterial endocarditis]]
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* Pulmonary embolism
* [[Pulmonary embolism]]
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* T1 and T2 weighted images show uniform intermediate signal intensity similar to myocardium
* T1 and T2 weighted images show uniform intermediate signal intensity similar to [[myocardium]]
* Homogeneous late gadolinium contrast enhancement
* Homogeneous late gadolinium contrast enhancement
|-
|-
|Hemangioma
|'''[[Hemangioma]]'''
|
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* Vascular
* [[Vascular]]
|
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* < 1 year to 70 years
* < 1 year to 70 years
* < 2 % of primary cardiac tumors
* < 2 % of primary cardiac tumors
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* Right Ventricle
* [[Ventricle|Right Ventricle]]
* Left ventricle
* [[Left ventricle]]
* Right atrium
* Right atrium
* Small percentage in interatrial septum and left atrium
* Small percentage in [[interatrial septum]] and [[left atrium]]
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* Polypoid
* Polypoid
* Encapsulated
* Encapsulated
|
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* Cyanosis
* [[Cyanosis]]
* Kasabach-Merritt syndrome (giant hemangiomas):
* [[Kasabach-Merritt syndrome]] (giant hemangiomas):
** Thrombosis
** [[Thrombosis]]
** Thrombocytopenia
** [[Thrombocytopenia]]
** Coagulopathy
** [[Coagulopathy]]
|
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* Pericardial effusions
* [[Pericardial effusions]]
* CHF
* [[CHF]]
* Arrhythmias
* [[Arrhythmias]]
|
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* Pulmonary embolism
* [[Pulmonary embolism]]
* Ischemic stroke
* [[Ischemic stroke]]
|
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* Intermediate density on T1 images
* Intermediate density on T1 images
* Hypointense on T2 images
* Hypointense on T2 images
* Multicystic enhancing lesion
* Multicystic enhancing lesion
* Involvement of epicardium or pericardium
* Involvement of [[epicardium]] or [[pericardium]]
|-
|-
|Lipoma
|'''[[Lipoma]]'''
|
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* Adipose
* [[Adipose]]
|
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* Between fourth to sixth decade of life
* Between fourth to sixth decade of life
|
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* Left ventricle (most commonly subendocardium)
* [[Left ventricle]] (most commonly subendocardium)
* Right atrium
* [[Right atrium]]
|
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* Sessile and small (specially subendocardial that protrude into cardiac chambers)
* Sessile and small (specially [[subendocardial]] that protrude into [[cardiac]] chambers)
* Broad pedicle
* Broad pedicle
* Growth into pericardial space
* Growth into [[pericardial space]]
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* Asymptomatic
* [[Asymptomatic]]
|
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* Dyspnea
* [[Dyspnea]]
* Local compression (subepicardial)
* Local compression (subepicardial)
* Arrhythmia
* [[Arrhythmia]]
|
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* Ischemic stroke
* [[Ischemic stroke]]
|
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* Epicardial on intramural lesion  
* [[Epicardial]] or intramural lesion  
* High intensity on T1 weighted image
* High intensity on T1 weighted image
* Drop out on fat saturation images
* Drop out on [[fat]] saturation images
|-
|-
|Paraganglioma
|'''[[Paraganglioma]]'''
|
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* Nervous (chromaffin cells of the sympathetic ganglia)
* [[Nervous system|Nervous]] ([[chromaffin cells]] of the [[sympathetic ganglia]])
|
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* Average age of presentation is 11-13 years
* Average age of presentation is 11-13 years
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*
*
|
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* Left atrium (under the aorta and the pulmonary artery)
* [[Left atrium]] (under the aorta and the [[pulmonary artery]])
* Interatrial septum
* [[Interatrial septum]]
* Left ventricle
* [[Left ventricle]]
* Right ventricular outflow tract
* [[Right ventricular outflow tract]]
|
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* 3-8 cm
* 3-8 cm
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* Hypervascular
* Hypervascular
|
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* Majority asymptomatic
* Majority [[asymptomatic]]
* May present with symptoms of catecholamine excess:
* May present with [[symptoms]] of [[catecholamine]] excess:
** Tachycardia
** [[Tachycardia]]
** Hypertension
** [[Hypertension]]
** Palipitations
** [[Palpitations]]
** Fever
** [[Fever]]
** Diaphoresis
** [[Diaphoresis]]
* Positive for chromogranin and synaptophysin
* Positive for [[chromogranin]] and [[synaptophysin]]
* Association with succinate dehydrogenase (SDH) mutation
* Association with [[succinate dehydrogenase]] ([[Succinate dehydrogenase|SDH]]) [[mutation]]
|
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* Dyspnea
* [[Dyspnea]]
* Valvular obstruction (murmurs)
* [[Valvular]] obstruction ([[murmurs]])
* Acute coronary syndrome (anginal pain)
* [[Acute coronary syndromes|Acute coronary syndrome]] ([[Angina|anginal pain]])
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* Enascement of coronary arteries by tumor
* Enascement of [[coronary arteries]] by [[tumor]]
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* Well defined lesion arising from atrial walls or septum
* Well defined lesion arising from [[Atrial|atrial walls]] or [[Interatrial septum|septum]]
* Bright on T2 weighted imaging
* Bright on T2 weighted imaging
|-
|-
|Atrioventricular Node Tumor
|'''Atrioventricular Node Tumor'''
|
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* Endodermal
* [[Endoderm|Endodermal]]
|
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* Average age of diagnosis is 38 years
* Average age of diagnosis is 38 years
* Female to male ratio 3:1
* Female to male ratio 3:1
|
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* Triangle of Koch in the AV nodal region of the atrial septum  
* [[Triangle of Koch]] in the [[AV node|AV nodal]] region of the [[atrial septum]]
|
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* 2 mm to 2 cm
* 2 mm to 2 cm
* Multiple
* Multiple
* Cystic
* Cystic
* Thickening of the atrial septum
* Thickening of the [[atrial septum]]
|
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* Most common cardiac tumor leading to sudden death
* Most common [[Cardiac tumors|cardiac tumor]] leading to sudden death
* Emery-Dreifuss muscular dystrophy
* [[Emery-Dreifuss muscular dystrophy]]
* Midline developmental defects along the central vertical body axis:
* Midline developmental defects along the central vertical body axis:
** Thyroglossal duct cysts
** [[Thyroglossal cyst|Thyroglossal duct cysts]]
** Cysts in the ovaries, breasts
** Cysts in the [[ovaries]], [[breasts]]
** Ventricular septal defect
** [[Ventricular septal defect]]
** Encephalocele
** [[Encephalocele]]
* Positive for:
* Positive for:
** Cytokeratin CAM5.2
** [[Cytokeratin]] CAM5.2
** Cytokeratin AE1/AE3
** [[Cytokeratin]] AE1/AE3
** Cytokeratin 34βE12
** [[Cytokeratin]] 34βE12
** Cytokeratin 5/6 (CK5/6)
** [[Cytokeratin]] 5/6 (CK5/6)
** Cytokeratin 7 (CK7)
** [[Cytokeratin]] 7 (CK7)
** Epithelial membrane antigen (EMA)
** Epithelial membrane antigen (EMA)
** Carcinoembryonic antigen (CEA)
** [[Carcinoembryonic antigen]] ([[CEA]])
** Carbohydrate antigen (CA)19.9, p63, bcl2, galectin 3
** [[CA 19-9|Carbohydrate antigen]] (CA)19.9, p63, [[bcl2]], [[Galectin-3|galectin 3]]
|
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* Palpitations
* [[Palpitations]]
* Dyspnea
* [[Dyspnea]]
* Chest pain
* [[Chest pain]]
* Diziness
* [[Dizziness|Diziness]]
* Syncope
* [[Syncope]]
* Complete heart block
* [[Complete heart block]]
* Partial AV block
* Partial [[Atrioventricular block|AV block]]
* Paroxysmal atrial arrhythmia
* [[Paroxysmal atrial tachycardia]]
* Spontaneous intermittent pre-excitation  
* Spontaneous intermittent pre-excitation  
|
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* Myocardial infarction
* [[Myocardial infarction]]
* Ischemic stroke
* [[Ischemic stroke]]
|
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* Hypointense cardiac mass on standard imaging
* Hypointense [[Cardiac mass causes|cardiac mass]] on standard imaging
* Hyperintensity on late gadolinium enhancement (LGE) images with heterogeneous contrast enhancement
* Hyperintensity on late gadolinium enhancement (LGE) images with heterogeneous contrast enhancement
|-
|-
|Lipomatous hypertrophy of the interatrial septum
|'''Lipomatous hypertrophy of the interatrial septum'''
|
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* Adipose
* Adipose
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|-
|-
| rowspan="4" |Primary Malignant
| rowspan="4" |
|Fibrosarcoma
==== Primary Malignant ====
|'''Fibrosarcoma'''
|
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* Fibrous
* Fibrous
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* Heterogenous or isointense to myocardium on T1 weighted images
* Heterogenous or isointense to myocardium on T1 weighted images
|-
|-
|Angiosarcoma
|'''Angiosarcoma'''
|
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* Vascular
* Vascular
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* Arterial phase enhancement
* Arterial phase enhancement
|-
|-
|Rhabdomyosarcoma
|'''Rhabdomyosarcoma'''
|
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* Mesenchymal
* Mesenchymal
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|-
|-
|Lymphoma
|'''Lymphoma'''
|
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* Lymphoid
* Lymphoid
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|
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|-
|-
|Secondary CardiacTumor
|
|Metastastatic Malignant
=== Secondary Cardiac Tumor ===
|Metastasis
|
==== Metastastatic Malignant ====
|'''Metastasis'''
|
|
* Skin (Melanoma)
* Skin (Melanoma)
Line 494: Line 504:
{|
{|
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;|Diseases
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
| colspan="6" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;|'''Clinical manifestations'''
| colspan="6" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Clinical manifestations'''
! colspan="7" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;|Para-clinical findings
! colspan="7" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Para-clinical findings
| colspan="1" rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;|'''Gold standard'''
| colspan="1" rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Gold standard'''
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;|Additional findings
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Additional findings
|-
|-
| colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;|'''Symptoms'''
| colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Symptoms'''
! colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;|Physical examination
! colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical examination
|-
|-
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;|Lab Findings
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab Findings
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;|Imaging
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;|Histopathology
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
|-  
|-  
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Symptom 1
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptom 1
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;|Symptom 2
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptom 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Symptom 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptom 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Physical exam 1
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical exam 1
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;|Physical exam 2
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical exam 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Physical exam 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical exam 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Lab 1
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab 1
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Lab 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Lab 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Imaging 1
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging 1
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Imaging 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Imaging 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging 3
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Rhabdomyoma
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Rhabdomyoma

Revision as of 16:23, 16 January 2019

Rhabdomyoma Microchapters

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Differentiating Rhabdomyoma from other Diseases

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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] Nima Nasiri, M.D.[3]

Overview

Rhabdomyoma is a benign tumor of striated muscle. Rhabdomyomas are rare and can be classified into cardiac type and extracardiac type.The most common primary benign pediatric tumor of the heart is cardiac rhabdomyoma which can be seen mainly in fetal life and children, second most common primary benign cardiac tumor in children is fibroma. Most tumors regress spontaneously, prognosis depends on the location of tumor and size. Cardiac rhabdomyoma is strongly associated with tuberous sclerosis.


Differentiating Rhabdomyoma from Other Diseases

Differential Diagnosis of Cardiac Rhabdomyoma

Cardiac rhabdomyoma 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]

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
  • Adipose
  • > 60 years
  • Limbus of the fossa ovalis (sparing fossa ovalis membrane)
  • > 2 cm
  • Interatrial septum maybe upto 7 cm in thickness
  • Mostly asymotmatic
  • May present with obstructive symptoms such as syncope in rare cases of extension into superior vena cava
  • Associated with:
    • Increased age
    • Obesity
    • Arrhythmias
  • Palpitations
  • Dyspnea
  • Fatigue
 -
  • Diffuse or nodular thickening of atrial walls
  • Hyperintense to normal myocardium
  • Hypointense to pericardial and posterior mediastinal fat

Primary Malignant

 Fibrosarcoma
  • Fibrous
  • Left sided (atrial mostly)
  • Sessile or pedunculated protruding masses in ventricular cavities
  • Soft
  • Lobulated
  • Gelatinous
  • Fever
  • Fatigue
  • Malaise
  • Weight loss
  • Cytoplasmatic positivity for a-SMA
  • CHF
  • Pericardial infiltration (effusions)
  • Pulmonary embolism
  • Ischemic stroke
  • Metastasis
  • Heterogenous or isointense to myocardium on T1 weighted images
Angiosarcoma
  • Vascular
  • Third to fifth decade of life
  • Right atrium
  • Broad-based
  • Internal hemmorrhage
  • Fever
  • Fatigue
  • Weight loss
  • Congestion
  • Superior vena cava syndrome
  • Embolic stroke
  • Endothelial marker D2-40
  • Dyspnea
  • CHF
  • Pericardial effusions
  • Cardiac tamponade
  • Pulmonary embolism
  • Ischemic stroke
  • Metastasis
  • Arterial phase enhancement
Rhabdomyosarcoma
  • Mesenchymal
  • Most common primary sarcoma of children
  • Average age of presentation is 20 years
  • Left sided (atrial mostly)
  • Multiple
  • Three types:
    • Embryonic
    • Pleomorphic
    • Alveolar
  • Fever
  • Fatigue
  • Weight loss
  • Metastasize to lung and lymph nodes
  • CHF
  • Arrhythmias
  • Pulmonary embolism
  • Ischemic stroke
  • Metastasis
  • Intermediate-to-hypointensity compared with muscle on T1 images
  • Hyperintense on T2-weighted imaging with heterogeneous contrast enhancement
Lymphoma
  • Lymphoid
  • First to ninth decade of life
  • Males > females
  • Right sided mostly
  • Majority solitary (1/3rd multiple)
  • Associations:
    • Chronic immunosupression
    • Epstein Barr virus
    • HIV
    • Diffuse large B cell lymphoma
  • May involve epicardium and pericardium
  • Pulmonary embolism
  • Ischemic stroke
  • Metastasis

Secondary Cardiac Tumor

Metastastatic Malignant

 Metastasis
  • Skin (Melanoma)
  • Lung carcinoma
  • Lymph (leukemia and lymphoma)
  • Breast carcinoma
  • Smooth muscle (Esophageal carcinoma)
  • Any age
  • Tumors metastasizing via hematogenous route present as multiple intramyocardial masses:
    • Melanoma
    • Leukemia
    • Sarcoma
  • Tumors metastasizing via venous system as right sided mass:
    • Renal cell carcinoma
    • Hepatocellular carcinoma
  • Lung tumor metastasizes to left atrium
  • Multiple
  • Fever
  • Fatigue
  • Weight loss
  • Dysphagia
  • Lymphadenopathy
  • Night sweats
  • Pericardial effusions
  • Dyspnea
  • Arrhytmia
  • Outflow obstruction
  • Pulmonary embolism
  • Ischemic stroke
  • Metastasis

Extra Cardiac Rhabdomyoma

  • Rhabdomyomas must be differentiated from other diseases, such as:[48][49][50]

On the basis , [symptom 2], and [symptom 3], Rhabdomyoma must be differentiated from Rhabdomyosarcoma, Tuberous sclerosis, Fibroma, Reticulohistiyocytoma, granular cell tumor, and hibernoma.

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histopathology
Symptom 1 Symptom 2 Symptom 3 Physical exam 1 Physical exam 2 Physical exam 3 Lab 1 Lab 2 Lab 3 Imaging 1 Imaging 2 Imaging 3
Rhabdomyoma
Rhabdomyosarcoma
Cardiac fibroma
granular cell tumor
Reticulohistyocytoma
Tuberous sclerosis

References

  1. ↑ Mankad R, Herrmann J (December 2016). "Cardiac tumors: echo assessment". Echo Res Pract. 3 (4): R65–R77. doi:10.1530/ERP-16-0035. PMC 5292983. PMID 27600455.
  2. ↑ Zaragoza-Macias E, Zaragosa-Macias E, Chen MA, Gill EA (February 2012). "Real time three-dimensional echocardiography evaluation of intracardiac masses". Echocardiography. 29 (2): 207–19. doi:10.1111/j.1540-8175.2011.01627.x. PMID 22283202.
  3. ↑ Larrieu AJ, Jamieson WR, Tyers GF, Burr LH, Munro AI, Miyagishima RT, Gerein AN, Allen P (March 1982). "Primary cardiac tumors: experience with 25 cases". J. Thorac. Cardiovasc. Surg. 83 (3): 339–48. PMID 7062746.
  4. ↑ Molina JE, Edwards JE, Ward HB (August 1990). "Primary cardiac tumors: experience at the University of Minnesota". Thorac Cardiovasc Surg. 38 Suppl 2: 183–91. doi:10.1055/s-2007-1014064. PMID 2237900.
  5. ↑ Tazelaar HD, Locke TJ, McGregor CG (October 1992). "Pathology of surgically excised primary cardiac tumors". Mayo Clin. Proc. 67 (10): 957–65. PMID 1434856.
  6. ↑ Sarjeant JM, Butany J, Cusimano RJ (2003). "Cancer of the heart: epidemiology and management of primary tumors and metastases". Am J Cardiovasc Drugs. 3 (6): 407–21. doi:10.2165/00129784-200303060-00004. PMID 14728061.
  7. ↑ St John Sutton MG, Mercier LA, Giuliani ER, Lie JT (June 1980). "Atrial myxomas: a review of clinical experience in 40 patients". Mayo Clin. Proc. 55 (6): 371–6. PMID 7382545.
  8. ↑ Pinede L, Duhaut P, Loire R (May 2001). "Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases". Medicine (Baltimore). 80 (3): 159–72. PMID 11388092.
  9. ↑ Reynen K (December 1995). "Cardiac myxomas". N. Engl. J. Med. 333 (24): 1610–7. doi:10.1056/NEJM199512143332407. PMID 7477198.
  10. ↑ Javed A, Zalawadiya S, Kovach J, Afonso L (March 2014). "Aortic valve myxoma at the extreme age: a review of literature". BMJ Case Rep. 2014. doi:10.1136/bcr-2013-202689. PMC 3962858. PMID 24642215.
  11. ↑ Lee VH, Connolly HM, Brown RD (August 2007). "Central nervous system manifestations of cardiac myxoma". Arch. Neurol. 64 (8): 1115–20. doi:10.1001/archneur.64.8.1115. PMID 17698701.
  12. ↑ Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VL (July 1985). "The complex of myxomas, spotty pigmentation, and endocrine overactivity". Medicine (Baltimore). 64 (4): 270–83. PMID 4010501.
  13. ↑ McAllister HA, Hall RJ, Cooley DA (February 1999). "Tumors of the heart and pericardium". Curr Probl Cardiol. 24 (2): 57–116. PMID 10028128.
  14. ↑ Klarich KW, Enriquez-Sarano M, Gura GM, Edwards WD, Tajik AJ, Seward JB (September 1997). "Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathologic correlation". J. Am. Coll. Cardiol. 30 (3): 784–90. PMID 9283541.
  15. ↑ Tamin SS, Maleszewski JJ, Scott CG, Khan SK, Edwards WD, Bruce CJ, Oh JK, Pellikka PA, Klarich KW (June 2015). "Prognostic and Bioepidemiologic Implications of Papillary Fibroelastomas". J. Am. Coll. Cardiol. 65 (22): 2420–9. doi:10.1016/j.jacc.2015.03.569. PMID 26046736.
  16. ↑ Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ (September 2003). "Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases". Am. Heart J. 146 (3): 404–10. doi:10.1016/S0002-8703(03)00249-7. PMID 12947356.
  17. ↑ Beghetti M, Gow RM, Haney I, Mawson J, Williams WG, Freedom RM (December 1997). "Pediatric primary benign cardiac tumors: a 15-year review". Am. Heart J. 134 (6): 1107–14. PMID 9424072.
  18. ↑ Smythe JF, Dyck JD, Smallhorn JF, Freedom RM (November 1990). "Natural history of cardiac rhabdomyoma in infancy and childhood". Am. J. Cardiol. 66 (17): 1247–9. PMID 2239731.
  19. ↑ Jacobs JP, Konstantakos AK, Holland FW, Herskowitz K, Ferrer PL, Perryman RA (November 1994). "Surgical treatment for cardiac rhabdomyomas in children". Ann. Thorac. Surg. 58 (5): 1552–5. PMID 7979700.
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  21. ↑ Basu S, Folliguet T, Anselmo M, Greengart A, Sabado M, Cunningham JN, Jacobowitz IJ (April 1994). "Lipomatous hypertrophy of the interatrial septum". Cardiovasc Surg. 2 (2): 229–31. PMID 8049952.
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  29. ↑ Webb DW, Thomas RD, Osborne JP (March 1993). "Cardiac rhabdomyomas and their association with tuberous sclerosis". Arch. Dis. Child. 68 (3): 367–70. PMC 1793857. PMID 8466239.
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  37. ↑ Yadav, Pradeep K.; Baquero, Giselle A.; Malysz, Jozef; Kelleman, John; Gilchrist, Ian C. (2014). "Cardiac Paraganglioma". Circulation: Cardiovascular Interventions. 7 (6): 851–856. doi:10.1161/CIRCINTERVENTIONS.114.001856. ISSN 1941-7640.
  38. ↑ Tahir M, Noor SJ, Herle A, Downing S (2009). "Right atrial paraganglioma: a rare primary cardiac neoplasm as a cause of chest pain". Tex Heart Inst J. 36 (6): 594–7. PMC 2801953. PMID 20069088.
  39. ↑ Hamilton BH, Francis IR, Gross BH, Korobkin M, Shapiro B, Shulkin BL, Deeb CM, Orringer MB (January 1997). "Intrapericardial paragangliomas (pheochromocytomas): imaging features". AJR Am J Roentgenol. 168 (1): 109–13. doi:10.2214/ajr.168.1.8976931. PMID 8976931.
  40. ↑ Shih, Wei-Jen; McCullough, Scott; Smith, Mary (1993). "Diagnostic imagings for primary cardiac fibrosarcoma". International Journal of Cardiology. 39 (2): 157–161. doi:10.1016/0167-5273(93)90028-F. ISSN 0167-5273.
  41. ↑ Arai T, Kurashima C, Wada S, Chida K, Ohkawa S (November 1998). "Histological evidence for cell proliferation activity in cystic tumor (endodermal heterotopia) of the atrioventricular node". Pathol. Int. 48 (11): 917–23. PMID 9832064.
  42. ↑ Wolf PL, Bing R (November 1965). "The smallest tumor which causes sudden death". JAMA. 194 (6): 674–5. PMID 5897246.
  43. ↑ Burke AP, Anderson PG, Virmani R, James TN, Herrera GA, Ceballos R (October 1990). "Tumor of the atrioventricular nodal region. A clinical and immunohistochemical study". Arch. Pathol. Lab. Med. 114 (10): 1057–62. PMID 2222148.
  44. ↑ Burke A, Tavora F (April 2016). "The 2015 WHO Classification of Tumors of the Heart and Pericardium". J Thorac Oncol. 11 (4): 441–52. doi:10.1016/j.jtho.2015.11.009. PMID 26725181.
  45. ↑ Tran, Thao T; Starnes, Vaughn; Wang, Xuedong; Getzen, James; Ross, Brian D (2009). "Cardiovascular magnetics resonance diagnosis of cystic tumor of the atrioventricular node". Journal of Cardiovascular Magnetic Resonance. 11 (1): 13. doi:10.1186/1532-429X-11-13. ISSN 1532-429X.
  46. ↑ Tatli, Servet; O'Gara, Patrick Thomas; Lambert, Jarvis; Kwong, Raymond; Byrne, John Gerald; Yucel, E. Kent (2004). "MRI of Atypical Lipomatous Hypertrophy of the Interatrial Septum". American Journal of Roentgenology. 182 (3): 598–600. doi:10.2214/ajr.182.3.1820598. ISSN 0361-803X.
  47. ↑ Saboo, Sachin S.; Krajewski, Katherine M.; Zukotynski, Katherine; Howard, Stephanie; Jagannathan, Jyothi P.; Hornick, Jason L.; Ramaiya, Nikhil (2012). "Imaging Features of Primary and Secondary Adult Rhabdomyosarcoma". American Journal of Roentgenology. 199 (6): W694–W703. doi:10.2214/AJR.11.8213. ISSN 0361-803X.
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  49. ↑ Miettinen M, Fetsch JF (April 2006). "Reticulohistiocytoma (solitary epithelioid histiocytoma): a clinicopathologic and immunohistochemical study of 44 cases". Am. J. Surg. Pathol. 30 (4): 521–8. PMID 16625100.
  50. ↑ Neuwirth H, Frasier B, Cochran ST (February 1989). "Genitourinary imaging and procedures by the emergency physician". Emerg. Med. Clin. North Am. 7 (1): 1–28. PMID 2645105.

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