CUA BPR 2020: Diagnosis and management of sporadic AML

Question 1

what are components of AML

Answer 1

blood vessels, smooth muscle and adipose tissue

Question 2

are the majority of AMLs asymptomatic?

Answer 2

yes, followed by presentation wit hemorrhage

Question 3

what are hereditary conditions associated with AML?

Answer 3

TSC, LAM( lymphangioleiomyomatosis

Question 4

is there any indication in treatment of sporadic AMLs with mTOR inhibitors?

Answer 4

NO, NOT for Sporadic

Question 5

What imaging tests are necessary to confirm the diagnosis of AMLs

Answer 5

unenhanced CT(intertumoral fat, <10HU) , contrast-enhanced CT(homogeneous enhancement, delayed washout, high intrinsic attenuation) , or MRI(fat hyperintense of T1 and hypointense on T2), chemical shift fat suppression can help identify AML. Percutaneous biopsy should be considered if neither CT nor MRI are diagnostic.
**on US hyperreflective lesion with acoustic shadowing

Question 6

What is the natural history of AMLs?

Answer 6

mostly asymptomatic on FU, majority stable in size, hemorrhage uncommon. lesions bigger than 4 cm more likely to be symptomatic + hemorrhage ( evidence is low level for this)

Question 7

What is the optimum follow up protocol for AMLs under observation?

Answer 7

biannual or annual
basis, but consideration should be given to decreasing frequency once stability has been
established. A decision for the cessation of monitoring should involve a discussion between
provider and patient, weighing risks and benefits.

Question 8

What are the indications for intervention?

Answer 8

Symptomatic
Treatment for asymptomatic AML >4cm should
be discussed
with the understanding that the absolute risks of hemorrhage are lower than
women of childbearing age, (increased risk due to estrogen receptor expression by AML)
and patient preferences.

Question 9

What interventions are available and preferred?

Answer 9

Angio-embolization
Nephrectomy
mTOR
observation

Question 10

What is the management of acutely bleeding AMLs?

Answer 10

Transcatheter embolization should be the first-line treatment for acutely bleeding AML.

Question 11

what type of AML can display malignant behavior?

Answer 11

Epithelioid AML( absence of adipocytes and abnormal vessels). (18-49% estimated to be malignant). 
they dont have fat so are diagnosed as RCC on imaging.

Question 12

what is the treatment of EAML

Answer 12

surgery