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Fibrolamellar Hepatocellular Carcinoma (FHCC)

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0:01

So this patient is, uh, younger than

0:03

some of the other patients we've

0:04

seen so far in these, uh, vignettes.

0:06

This is a 23-year-old female who presents, um,

0:11

with abnormal liver function tests. Got some

0:13

other imaging that showed liver mass, and we've

0:15

been asked to do an MRI to further evaluate it.

0:18

So I'll scroll through first

0:20

sets of images for this patient.

0:22

These are going to be, uh, T2 and T1-weighted

0:25

images, and I'll just kind of scroll through

0:27

it up and down at first to give you a sense

0:29

of sort of what's going on in the liver.

0:32

And then I'll kind of focus in this area.

0:35

So here, uh, is the T2-weighted sequence.

0:37

This doesn't have any fat saturation with it.

0:39

This is a T1-weighted sequence with fat

0:42

saturation and without intravenous contrast.

0:46

And there's a really large lesion

0:48

that essentially is replacing

0:50

the entire right hepatic lobe.

0:52

So it's tough to even draw the

0:53

borders of this, but at least, I mean,

0:55

this whole thing here is abnormal.

0:57

And this whole thing here is abnormal.

0:59

Very heterogeneous in its appearance.

1:01

There's areas like over here

1:03

that are rather bright in signal.

1:05

There are areas, for example, over

1:07

here that have a sort of star-shaped

1:09

appearance that is, uh, darker in signal.

1:13

On the T1-weighted sequences,

1:14

again, heterogeneous.

1:15

Some areas that are a little bit

1:17

brighter, others that are darker.

1:19

Kind of very difficult to, uh, figure

1:21

out the boundaries of this lesion.

1:23

Again, if we scroll through this lesion,

1:25

we can see that certainly the right hepatic

1:27

lobe is replaced, but there are probably

1:28

lesions in the left hepatic lobe as well,

1:30

as can be seen over here and over here

1:32

that have similar imaging features to the

1:34

dominant mass in the right hepatic lobe.

1:36

Now, for this particular study, we didn't

1:38

get T1-weighted images, uh, in and out of

1:41

phase to look for the presence of lipid, but

1:43

there was no suggestion of lipid based on some

1:46

of the imaging findings that, uh, we do have.

1:48

So we move on ahead to our post-contrast images.

1:51

This is a T1 FAT SAT.

1:54

That post-contrast image in the

1:56

arterial phase, portal venous phase,

1:58

and this is the equilibrium phase.

2:00

And again, this is a large lesion, so

2:02

it's hard to show its entirety with just

2:04

a snapshot, but certainly, uh, all this

2:06

here is abnormal in the right hepatic lobe.

2:08

There's very heterogeneous

2:10

arterial hyperenhancement.

2:12

Certainly, by the time you get to the equilibrium

2:14

phase, you can argue that the areas that

2:16

are arterially enhancing are washing out.

2:18

Maybe there's areas of capsule or

2:20

pseudo-capsule associated with it.

2:22

And this central portion that was rather dark

2:24

on the T2-weighted images, we notice, is also

2:27

dark post-contrast and never really fills in.

2:30

It remains dark on the arterial phase, portal

2:33

venous phase, and equilibrium phase images.

2:37

So what we're really left with is a very large

2:39

lesion that has, you know, very heterogeneous

2:43

signal on the, uh, T2 and T1-weighted images.

2:47

It has this sort of T2 dark and non-enhancing

2:52

central portion of it.

2:56

Some people may even term that

2:57

a scar around the center of it.

3:00

There are areas that demonstrate arterial hyper

3:03

enhancement, there are areas that wash out,

3:05

and there may be areas that have a capsule

3:08

around portions of this lesion.

3:10

Now, this person had a history of cirrhosis, was

3:13

at risk for developing hepatocellular carcinoma.

3:16

Based on what we have, we could call

3:18

this a LI-RADS 5 lesion because of the

3:22

size, arterial hyperenhancement, and

3:23

washout, and move on to treatment.

3:26

However, LI-RADS does not apply

3:29

to this patient because this

3:30

patient has no risk factors.

3:32

for cirrhosis, does not have

3:35

hepatitis B infection, nor does this

3:37

patient have a history of prior HCC.

3:40

The LI-RADS only applies to those

3:43

patients in those categories.

3:45

This happens to be a very unfortunate young

3:47

patient who is otherwise healthy, who happens

3:50

to have a large mass that has some imaging

3:53

features of HCC, but we cannot use LI-RADS in

3:55

this setting for the reasons that are mentioned.

3:58

So this warrants a biopsy, this was

4:00

biopsied, and this turned out to be a

4:02

histological subtype of HCC that is known

4:07

as fibrolamellar hepatocellular carcinoma.

4:12

Now, the important thing to know about

4:14

this particular subtype is that it tends

4:17

to happen in younger patients, typically

4:21

in the second to third decades of life.

4:24

And, uh, as mentioned, patients

4:26

tend to be otherwise healthy.

4:29

No known history of cirrhosis typically

4:32

associated with this finding.

4:35

So, LI-RADS does not apply in this setting.

4:38

When they do present, the

4:39

lesions can be rather large.

4:40

They can range from 5 to 20 centimeters.

4:43

On average, they can be around 13 centimeters.

4:46

These are large lesions.

4:48

And for hepatocellular carcinomas, we can

4:50

often use alpha-fetoprotein as tumor markers.

4:54

If it's elevated, it tells us that there

4:56

may be the presence of this disease.

4:58

However, with fibrolamellar hepatocellular

5:01

carcinomas, usually these levels are

5:03

normal, or they're only minimally elevated,

5:06

despite the large size of the mass.

5:10

Overall, prognosis tends to be a

5:12

little bit better for this subtype than

5:15

other HCCs, although, you know, this

5:17

tends to also be quite aggressive, uh,

5:20

locally, and it frequently metastasizes.

5:23

So, not a great prognosis overall

5:26

due to those reasons.

5:28

So, this was a patient, healthy patient,

5:29

large liver lesion, some imaging features

5:31

of HCC, in which LI-RADS does not apply.

5:34

And this was biopsied, and this turned out

5:36

to be a histological subtype that tends

5:38

to be seen in young, healthy patients,

5:41

which may have a slightly better

5:43

prognosis than other HCCs of this size.

Report

Faculty

Mahan Mathur, MD

Associate Professor, Division of Body Imaging; Vice Chair of Education, Dept of Radiology and Biomedical Imaging

Yale School of Medicine

Tags

Neoplastic

MRI

Liver

Gastrointestinal (GI)

Body

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