Rhabdomyoma

Rhabdomyoma

KEY FACTS

Terminology

• Hamartoma of developing cardiac myocytes
• Most common primary benign cardiac tumor in pediatric patients, highly associated with tuberous sclerosis
• 50% of solitary tumors and 100% of multiple tumors are associated with tuberous sclerosis

Imaging

• Most common cardiac tumor in pediatric patients, especially patients with tuberous sclerosis
• Almost always arise in ventricular myocardium; may prolapse into ventricular cavity
• Echocardiography: 1st-line test; echogenic mass
• CT: Isoattenuating to adjacent myocardium with minimal or no differential contrast enhancement
• Fatty lesions and pseudolesions may be associated in tuberous sclerosis
• MR: Homogeneous and well-circumscribed masses
- T1: Isointense; T2: Slightly hyperintense; perfusion: Hypointense; delayed enhancement: Isointense
• Echocardiography and MR: Complementary imaging modalities
• Echo: Better for small (< 5-mm) or completely intramural lesions
• MR: Better for intracavitary lesions and extracardiac extension

Top Differential Diagnoses

• Fibroma; myxoma; thrombus

Clinical Issues

• Almost all will spontaneously regress as child ages and can be managed conservatively
- Frequently associated with tuberous sclerosis
• Almost all will spontaneously regress
• Most will be followed to document regression, usually with echo but occasionally with MR if better seen by that modality

TERMINOLOGY

Synonyms

• Cardiac hamartoma

Definitions

• Hamartoma of developing cardiac myocytes
- Most common primary benign cardiac tumor in pediatric patients, highly associated with tuberous sclerosis
  - – 50% of patients with rhabdomyoma have tuberous sclerosis

IMAGING

General Features

• Best diagnostic clue
- Pediatric myocardial tumor with homogeneous signal, more common in patients with tuberous sclerosis
• Location
- Usually attached to the myocardium
- Intramyocardial or intracavitary
• Size
- Variable
• Morphology
- Generally well circumscribed
- Solitary or multiple
  - – 50% of solitary tumors and 100% of multiple tumors are associated with tuberous sclerosis
- Homogeneous; may have fatty degeneration

CT Findings

• Isoattenuating to adjacent myocardium with minimal or no differential contrast enhancement
• May demonstrate fatty degeneration
• Fatty lesions and pseudolesions may be associated in tuberous sclerosis
- Ventricular septum, free wall of left/right ventricle (LV/RV), papillary muscles
- Well circumscribed and focal

Only gold members can continue reading. Log In or Register to continue

Stay updated, free articles. Join our Telegram channel

Tags: CT and MR in Cardiology

Apr 6, 2020 | Posted by admin in CARDIOVASCULAR IMAGING | Comments Off

Full access? Get Clinical Tree

Get Clinical Tree app for offline access

Get Clinical Tree app for offline access