0:01

This was an infant who presented at Day of Life six,

0:05

who at prenatal ultrasound had a question

0:08

of a cystic left kidney.

0:11

So they came for postnatal renal ultrasound

0:13

to further evaluate that left kidney.

0:16

On the very first image,

0:17

we can see there's something abnormal.

0:19

So we see normal part of normal liver

0:23

and then we see part of

0:24

what looks like a normal right kidney.

0:26

But then we have this heterogeneous mass lesion

0:29

above the right kidney, which is kind of funny

0:31

because this patient presented with concern

0:34

for contralateral left sided abnormality.

0:37

So here we have normal right kidney,

0:39

but again, this abnormal heterogeneous lesion in

0:42

the senal space.

0:44

Interestingly, we see part of one limb of the adrenal gland.

0:48

Um, this does not appear

0:50

to be arising from the left kidney itself

0:52

because we do not have a claw sign of this left kidney.

0:56

Um, sorry, right kidney.

0:58

This looks completely separate from the right kidney.

1:01

We don't have a claw of tissue.

1:03

And circling this, we see only part of normal adrenal gland.

1:07

So this is gonna be an adrenal primary lesion.

1:10

We see some normal portion of hypertrophy,

1:13

normal hypertrophied adrenal gland,

1:16

but then we have this heterogeneous mass lesion, um,

1:19

occupying the other limb of the adrenal gland.

1:23

So the question is, is this gonna be a solid lesion?

1:26

Can we find internal flow

1:27

or is this just an avascular adrenal hematoma related

1:31

to birth injury?

1:34

And so we'll put color doppler flow

1:36

to be able to distinguish that.

1:38

And unfortunately this, um, uh,

1:41

heterogeneous mass lesion arising from the right adrenal

1:43

gland does appear to have internal vascularity.

1:48

So right away, just based on these few images we've seen,

1:51

this is gonna be concerning for congenital neuroblastoma.

1:55

Um, let's see what other images we have.

1:58

Yeah, this is definitely real flow to this

2:02

SRE mass lesion involving the right adrenal gland.

2:05

So this is highly concerning for congenital neuroblastoma.

2:09

So from here we're gonna recommend that our clinicians, uh,

2:12

number one, refer or involve the pediatric

2:15

or pediatric oncology colleagues that they get, um,

2:18

urine catecholamine levels.

2:20

Um, in this case, I'll tell you that they were elevated.

2:23

I'll spoil the surprise.

2:25

And then, um, several other things need to happen.

2:28

Number one, um, usually when you have a tumor

2:31

that presents at such a young age,

2:34

the prognosis is, uh, worse.

2:37

But neuroblastoma is the opposite.

2:39

When you have an infant that presents either

2:41

with congenital neuroblastoma

2:43

or any infant prior to the age of 18 months,

2:47

they actually typically have a better prognosis.

2:49

So that is called stage, um, MS in the, um,

2:55

INSS staging system.

2:57

Um, so this patient will go on number one

3:00

to soft tissue sampling of this lesion to confirm

3:03

that is neuroblastoma,

3:05

but then also to do molecular analysis of this tumor.

3:08

They'll look for en IC amplification to be able

3:10

to additionally help them prognosticate in this tumor.

3:14

And then the last thing that will happen is staging

3:16

with MIBG.

3:18

Um, so looking for additional areas of uptake to be sure

3:22

that this patient doesn't need to be upstaged, um, uh, to,

3:26

uh, a higher stage of neuroblastoma continuing

3:30

through this kidney, uh, renal ultrasound just to make sure

3:32

that whatever cystic structure they saw in the left kidney

3:35

prenatally is either real or not real.

3:38

We have a normal urinary bladder, normal, uh,

3:41

heterogeneous content in the rectum.

3:43

This is a normal thickness

3:45

of a urinary bladder in an infant.

3:46

It's just not super well distended.

3:49

Oh no, the left kidney was indeed abnormal.

3:52

So that right adrenal neuroblastoma

3:54

was an incidental finding.

3:56

So let's grade this left-sided, uh,

3:59

hydronephrosis based on the UTD classification system.

4:02

Number one, we have ectasis Number two,

4:05

we have not only central,

4:07

but we also have peripheral ectasis.

4:09

And then I would say that this renal parenchyma is

4:12

diffusely, but, but, uh, smoothly thin.

4:16

So this is renal parenchymal thinning.

4:18

So this is left urinary tract dilatation P three

4:22

because of that renal parenchymal thinning.

4:25

So this patient will go on, uh, uh,

4:27

to be followed not only from a neuroblastoma standpoint,

4:30

but also from a congenital hydro necrosis

4:33

follow-up standpoint.

4:35

But the next thing we're gonna do

4:36

for this infant is follow up MIBG.

4:40

So these are images from the follow-up MIBG

4:42

that this patient underwent.

4:44

And I'm going to just show you these whole body, um,

4:47

planar images to show you all the, all the areas of uptake.

4:51

So I'm gonna magnify this, um, this, uh,

4:54

coronal view here for you.

4:56

So MIBG will have physiologic uptake to salivary glands.

5:00

We'll see normal uptake in several areas,

5:03

but this is abnormal MIBG uptake associated with

5:07

that right adrenal primary neuroblastoma.

5:10

Other places that you wanna play close attention to to look

5:13

for other, uh, uh, abnormal MIBG uptake areas, uh,

5:18

is the bone marrow.

5:19

So, uh, this is a localized right adrenal, uh,

5:24

primary only neuroblastoma.

5:27

When we assign a curie score to this infant, um,

5:30

there will only be one point for a soft tissue component,

5:35

but there is no abnormal bone marrow uptake.

5:37

So curie score of one.

5:40

So this patient will be followed, um,

5:42

because this is a low risk neuroblastoma, obviously.

5:46

Um, they also have to correlate

5:47

with the en ic applica amplification status,

5:50

but we just have a localized primary tumor.

5:53

Oftentimes they will watch these tumors. They won't even

5:56

Surgically resect them, and they will involute on their

5:59

own if it doesn't change in size or if it grows over time.

6:03

As they follow these patients per the COG uh, treatment,

6:06

children's oncology group treatment protocols, um, they, uh,

6:11

may end, eventually end up resecting the

6:13

primary adrenal tumor.

6:15

Um, but if it's, if it's well-behaved,

6:18

they often spontaneously resolve when patients present

6:21

with congenital neuroblastoma.

6:23

So this is one of those tumors that it's not the end

6:26

of the world to be born with.

6:27

Oftentimes patients

6:29

with congenital neuroblastoma have excellent prognosis, um,

6:33

like this patient.