New Recording 9

0:21

S… Speaker 1 (New Recording 9)

Thank you.

1:03

S… Speaker 1 (New Recording 9)

Oh, yeah.

1:29

S… Speaker 1 (New Recording 9)

What's my tower?

1:54

S… Speaker 1 (New Recording 9)

Thank you very much.

2:27

S… Speaker 1 (New Recording 9)

Anyway, we have a local reference to U.S.T.E.K.A.T.

2:43

S… Speaker 1 (New Recording 9)

Thank you.

2:46

S… Speaker 1 (New Recording 9)

Thank you.

2:47

S… Speaker 1 (New Recording 9)

Thank you.

2:59

S… Speaker 1 (New Recording 9)

That's why it doesn't...

3:02

S… Speaker 1 (New Recording 9)

It doesn't... It doesn't matter.

3:02

S… Speaker 1 (New Recording 9)

It doesn't matter.

3:03

S… Speaker 1 (New Recording 9)

It doesn't matter. It doesn't matter. It doesn't

3:19

S… Speaker 1 (New Recording 9)

matter.

3:37

S… Speaker 1 (New Recording 9)

This case,

3:38

S… Speaker 1 (New Recording 9)

how do you see

3:42

S… Speaker 1 (New Recording 9)

that?

4:34

S… Speaker 1 (New Recording 9)

What else is that?

4:35

S… Speaker 1 (New Recording 9)

Thank you.

5:01

S… Speaker 1 (New Recording 9)

You're the

5:20

S… Speaker 1 (New Recording 9)

1 .04 for the paper.

10:01

S… Speaker 1 (New Recording 9)

Exactly.

20:02

S… Speaker 1 (New Recording 9)

Around this,

20:03

S… Speaker 1 (New Recording 9)

we can get a little bit,

20:04

S… Speaker 1 (New Recording 9)

a little bit.

20:04

S… Speaker 1 (New Recording 9)

But it's not

20:11

S… Speaker 1 (New Recording 9)

surgery. Surgery is not here,

20:12

S… Speaker 1 (New Recording 9)

it's just medical medicine.

20:13

S… Speaker 1 (New Recording 9)

For example,

20:14

S… Speaker 1 (New Recording 9)

surgery is not gradation therapy.

20:16

S… Speaker 1 (New Recording 9)

It's not systemic therapy.

20:20

S… Speaker 1 (New Recording 9)

Do you want to go to the board?

20:21

S… Speaker 1 (New Recording 9)

Do you want to

20:29

S… Speaker 1 (New Recording 9)

go to the board?

20:50

S… Speaker 1 (New Recording 9)

This is a great place, but it's a great place.

25:09

S… Speaker 1 (New Recording 9)

Thank you.

25:56

S… Speaker 1 (New Recording 9)

How are you?

25:57

S… Speaker 1 (New Recording 9)

How

26:03

S… Speaker 1 (New Recording 9)

are you?

26:03

S… Speaker 1 (New Recording 9)

How are

26:22

S… Speaker 1 (New Recording 9)

you?

26:28

S… Speaker 1 (New Recording 9)

Thank you.

26:52

S… Speaker 1 (New Recording 9)

Thank you.

27:24

S… Speaker 1 (New Recording 9)

Thank you.

27:57

S… Speaker 1 (New Recording 9)

Keep it, keep it.

28:32

S… Speaker 1 (New Recording 9)

Thank you.

29:03

S… Speaker 1 (New Recording 9)

That's why

29:25

S… Speaker 1 (New Recording 9)

The name I know

29:47

S… Speaker 1 (New Recording 9)

Assalamu alaikum.

29:48

S… Speaker 1 (New Recording 9)

Good afternoon.

29:49

S… Speaker 1 (New Recording 9)

My name is Guthamma Amr.

29:52

S… Speaker 1 (New Recording 9)

I am the general manager of Merck and Saladin.

29:54

S… Speaker 1 (New Recording 9)

First of all,

29:55

S… Speaker 1 (New Recording 9)

I would like really to thank you for the time,

29:58

S… Speaker 1 (New Recording 9)

for spending,

29:58

S… Speaker 1 (New Recording 9)

I would say, a good part.

30:00

S… Speaker 1 (New Recording 9)

of the weekend with us in lovely Riyadh.

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S… Speaker 1 (New Recording 9)

Yes, the weather is,

30:03

S… Speaker 1 (New Recording 9)

yeah, but it's a lovely weather.

30:06

S… Speaker 1 (New Recording 9)

Actually,

30:07

S… Speaker 1 (New Recording 9)

yeah, we will be spending today a couple of hours discussing a

30:12

S… Speaker 1 (New Recording 9)

patient population that may have been a bit overlooked with little,

30:16

S… Speaker 1 (New Recording 9)

I would say, or like few therapeutic options.

30:18

S… Speaker 1 (New Recording 9)

And that meant we are really proud,

30:20

S… Speaker 1 (New Recording 9)

glad that soon we will bring,

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I would say, one of the therapeutic options for those patients.

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S… Speaker 1 (New Recording 9)

And with that,

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S… Speaker 1 (New Recording 9)

I would like really to thank and welcome Dr.

30:33

S… Speaker 1 (New Recording 9)

Ashwaq Al -Alayan.

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S… Speaker 1 (New Recording 9)

I don't think she needs her introduction,

30:37

S… Speaker 1 (New Recording 9)

but I will do it anyway.

30:38

S… Speaker 1 (New Recording 9)

So,

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S… Speaker 1 (New Recording 9)

Dr.

30:39

S… Speaker 1 (New Recording 9)

Ashwaq, she's the head of the Adult Medical Oncology

30:43

S… Speaker 1 (New Recording 9)

in the National Guard Hospital in Riyadh.

30:47

S… Speaker 1 (New Recording 9)

Yes.

30:47

S… Speaker 1 (New Recording 9)

And yeah,

30:49

S… Speaker 1 (New Recording 9)

thank you so much,

30:50

S… Speaker 1 (New Recording 9)

Dr. Ashwaq,

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S… Speaker 1 (New Recording 9)

and she will guide us and lead us through the day.

30:54

S… Speaker 1 (New Recording 9)

Thank you so much.

30:57

S… Speaker 1 (New Recording 9)

Thank you,

30:58

S… Speaker 1 (New Recording 9)

everyone, for attending,

30:59

S… Speaker 1 (New Recording 9)

and thank you,

31:00

S… Speaker 1 (New Recording 9)

Merck, for pulling this.

31:02

S… Speaker 1 (New Recording 9)

So we'll talk just a little bit about the objective of the meeting,

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S… Speaker 1 (New Recording 9)

and we know,

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S… Speaker 1 (New Recording 9)

like, there's more tumors,

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S… Speaker 1 (New Recording 9)

a rare tumor,

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S… Speaker 1 (New Recording 9)

but we know to look at the current patient

31:13

S… Speaker 1 (New Recording 9)

journey and identify the critical gaps in diagnosis and

31:17

S… Speaker 1 (New Recording 9)

referring to the patient and,

31:19

S… Speaker 1 (New Recording 9)

at the end,

31:19

S… Speaker 1 (New Recording 9)

treatment pathway.

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S… Speaker 1 (New Recording 9)

We are looking for career internal diagnosis,

31:24

S… Speaker 1 (New Recording 9)

referral efficiency and access to specialized center.

31:28

S… Speaker 1 (New Recording 9)

We need to understand because you're at the top oncologist who's treating a dysmorphic

31:32

S… Speaker 1 (New Recording 9)

tumor, what's the fact that that influence...

31:36

S… Speaker 1 (New Recording 9)

choosing the treatment option.

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S… Speaker 1 (New Recording 9)

We have various options,

31:39

S… Speaker 1 (New Recording 9)

surgery,

31:40

S… Speaker 1 (New Recording 9)

filtration, systemic therapy,

31:42

S… Speaker 1 (New Recording 9)

or watch and wait.

31:43

S… Speaker 1 (New Recording 9)

So we need to understand.

31:44

S… Speaker 1 (New Recording 9)

I hear one of these people I want to learn from you guys.

31:47

S… Speaker 1 (New Recording 9)

So we look to gather information about your experience because we

31:52

S… Speaker 1 (New Recording 9)

all have an experience with taking eye.

31:53

S… Speaker 1 (New Recording 9)

But probably of the new medication,

31:56

S… Speaker 1 (New Recording 9)

I think you have two patients or three patients.

32:00

S… Speaker 1 (New Recording 9)

Yet, I don't have any patient.

32:02

S… Speaker 1 (New Recording 9)

I ordered the medication and I'm waiting.

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S… Speaker 1 (New Recording 9)

So we're going to discuss the DeFi trial and

32:08

S… Speaker 1 (New Recording 9)

we're going to look at the real -world experience about the new medication and Dr.

32:12

S… Speaker 1 (New Recording 9)

Abdullah,

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S… Speaker 1 (New Recording 9)

he will talk about a case.

32:16

S… Speaker 1 (New Recording 9)

So without any further delay,

32:19

S… Speaker 1 (New Recording 9)

we'll start with Dr.

32:20

S… Speaker 1 (New Recording 9)

Canaan.

32:20

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Dr.

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S… Speaker 1 (New Recording 9)

Canaan is a non -medical oncologist.

32:24

S… Speaker 1 (New Recording 9)

He's the director of the MENA NCCN.

32:28

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And here we'll talk about the overview,

32:30

S… Speaker 1 (New Recording 9)

impact,

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S… Speaker 1 (New Recording 9)

and disease problems.

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S… Speaker 1 (New Recording 9)

So it's an overview,

32:34

S… Speaker 1 (New Recording 9)

then we're going to hear again from him.

32:36

S… Speaker 1 (New Recording 9)

I'd

32:51

S… Speaker 1 (New Recording 9)

like to thank Mark for giving me the opportunity to talk to distinguished

32:56

S… Speaker 1 (New Recording 9)

experts in Sankoma.

32:57

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So today's topic is going to be,

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or at least my talk is going to be,

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a brief overview on this one's tumors.

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I'll speak for about 15 minutes,

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S… Speaker 1 (New Recording 9)

then I think Dr.

33:07

S… Speaker 1 (New Recording 9)

Ashwar will take the lead with regards to discussion.

33:10

S… Speaker 1 (New Recording 9)

So what are these tumors?

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S… Speaker 1 (New Recording 9)

They're locally aggressive fibroblastic tumors

33:17

S… Speaker 1 (New Recording 9)

that have or that are supposed to not metastasize.

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S… Speaker 1 (New Recording 9)

They are locally aggressive,

33:23

S… Speaker 1 (New Recording 9)

locally infiltrative.

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S… Speaker 1 (New Recording 9)

Their disease course is unpredictable.

33:29

S… Speaker 1 (New Recording 9)

Some of them will regress.

33:32

S… Speaker 1 (New Recording 9)

spontaneously and of course surgery used

33:36

S… Speaker 1 (New Recording 9)

to be the mainstay of therapy and there are high rates of records after

33:40

S… Speaker 1 (New Recording 9)

surgery.

33:47

S… Speaker 1 (New Recording 9)

Quite rare.

33:47

S… Speaker 1 (New Recording 9)

I think because we are sarcoma oncologists,

33:50

S… Speaker 1 (New Recording 9)

I think most of us should have this point to us.

33:53

S… Speaker 1 (New Recording 9)

However, if we're talking about general population or general cancer population,

33:56

S… Speaker 1 (New Recording 9)

it does represent 0 .03 % of all cancers.

34:00

S… Speaker 1 (New Recording 9)

So very rare.

34:01

S… Speaker 1 (New Recording 9)

Less than 3 % of all soft tissue sarcomas.

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S… Speaker 1 (New Recording 9)

And peak age at diagnosis is quite valuable.

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S… Speaker 1 (New Recording 9)

So you have probably your FAP patients,

34:10

S… Speaker 1 (New Recording 9)

which are diagnosed at an earlier age,

34:13

S… Speaker 1 (New Recording 9)

and you have your...

34:14

S… Speaker 1 (New Recording 9)

other patients who are diagnosed at a later age.

34:19

S… Speaker 1 (New Recording 9)

There is a slight female predominance.

34:22

S… Speaker 1 (New Recording 9)

There is no significant racial or ethnic predilection.

34:25

S… Speaker 1 (New Recording 9)

And about 5 to 50 % of patients are

34:29

S… Speaker 1 (New Recording 9)

actually FAB related.

34:31

S… Speaker 1 (New Recording 9)

I'll talk about the risk factors in the next few slides.

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S… Speaker 1 (New Recording 9)

So we do divide checkpoint tumors

34:38

S… Speaker 1 (New Recording 9)

into...

34:40

S… Speaker 1 (New Recording 9)

are too major sometimes.

34:41

S… Speaker 1 (New Recording 9)

You have your sporadic ones,

34:43

S… Speaker 1 (New Recording 9)

which are the majority.

34:44

S… Speaker 1 (New Recording 9)

They represent about 85 % of these cases.

34:47

S… Speaker 1 (New Recording 9)

Some of them have antecedent major trauma.

34:51

S… Speaker 1 (New Recording 9)

Some of them don't.

34:53

S… Speaker 1 (New Recording 9)

Molecularly,

34:55

S… Speaker 1 (New Recording 9)

the majority of them would have CTNNB1 mutations.

35:00

S… Speaker 1 (New Recording 9)

activating or an active beta -catenin pathway.

35:03

S… Speaker 1 (New Recording 9)

And there are your FAP -related desmoids.

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These are less common.

35:09

S… Speaker 1 (New Recording 9)

They represent between 5 % to 15 % of all

35:14

S… Speaker 1 (New Recording 9)

desmoid cases.

35:15

S… Speaker 1 (New Recording 9)

These cases are known to have an APC gene mutation,

35:20

S… Speaker 1 (New Recording 9)

a germline one.

35:22

S… Speaker 1 (New Recording 9)

So they do require germline testing and sort of genetic counseling.

35:28

S… Speaker 1 (New Recording 9)

In terms of when a patient has basically

35:33

S… Speaker 1 (New Recording 9)

FAP,

35:34

S… Speaker 1 (New Recording 9)

the likelihood that they're going to develop a dysmoid is about 20%.

35:38

S… Speaker 1 (New Recording 9)

So,

35:40

S… Speaker 1 (New Recording 9)

as I mentioned,

35:43

S… Speaker 1 (New Recording 9)

FAP carries a risk of development

35:47

S… Speaker 1 (New Recording 9)

of dysmoids at about 20%.

35:49

S… Speaker 1 (New Recording 9)

It is an autosomal dominant syndrome caused by a germline

35:53

S… Speaker 1 (New Recording 9)

APC mutation.

35:57

S… Speaker 1 (New Recording 9)

FAP associated desmoids anatomically typically go

36:01

S… Speaker 1 (New Recording 9)

intra -abdominally or abdominal wall and

36:05

S… Speaker 1 (New Recording 9)

there is a syndrome called Garner syndrome and this is when you

36:09

S… Speaker 1 (New Recording 9)

have FAP,

36:10

S… Speaker 1 (New Recording 9)

desmoid,

36:12

S… Speaker 1 (New Recording 9)

osteomas,

36:13

S… Speaker 1 (New Recording 9)

epidermal cysts and supernumerary teeth.

36:16

S… Speaker 1 (New Recording 9)

Now, patients with FAP have a higher likelihood

36:20

S… Speaker 1 (New Recording 9)

of developing colon cancer,

36:22

S… Speaker 1 (New Recording 9)

and I think their lifetime risk of developing colon cancer is about 70%.

36:27

S… Speaker 1 (New Recording 9)

So these patients,

36:28

S… Speaker 1 (New Recording 9)

when identified,

36:29

S… Speaker 1 (New Recording 9)

should receive genetic counseling,

36:31

S… Speaker 1 (New Recording 9)

should see a cancer geneticist,

36:33

S… Speaker 1 (New Recording 9)

and the majority of them should actually have an indication for

36:38

S… Speaker 1 (New Recording 9)

doing prophylactic connectivity.

36:42

S… Speaker 1 (New Recording 9)

So, just like I mentioned,

36:43

S… Speaker 1 (New Recording 9)

the risk factors,

36:44

S… Speaker 1 (New Recording 9)

the biggest one is antecedent

36:48

S… Speaker 1 (New Recording 9)

trauma,

36:49

S… Speaker 1 (New Recording 9)

about 30 % of sporadic desmoid tumors.

36:52

S… Speaker 1 (New Recording 9)

Then you have your FAP,

36:54

S… Speaker 1 (New Recording 9)

probably 5 to 15%.

36:56

S… Speaker 1 (New Recording 9)

And,

36:57

S… Speaker 1 (New Recording 9)

of course, hormonal or pregnancy.

36:59

S… Speaker 1 (New Recording 9)

With pregnancy,

37:01

S… Speaker 1 (New Recording 9)

you get your high estrogen surge.

37:03

S… Speaker 1 (New Recording 9)

And if you actually have a desmoid tumor,

37:07

S… Speaker 1 (New Recording 9)

the likelihood that it's going to grow is actually quite significant.

37:11

S… Speaker 1 (New Recording 9)

Some of these patients postpartum due to a

37:15

S… Speaker 1 (New Recording 9)

rapid decline in hormones,

37:17

S… Speaker 1 (New Recording 9)

they do get postpartum regression.

37:23

S… Speaker 1 (New Recording 9)

Molecularly,

37:24

S… Speaker 1 (New Recording 9)

we have two major pathways.

37:25

S… Speaker 1 (New Recording 9)

You have the beta -catenin pathway,

37:27

S… Speaker 1 (New Recording 9)

where dysregulation of this wind beta -catenin pathway is

37:32

S… Speaker 1 (New Recording 9)

implicated in the pathogenesis of this one tumors.

37:34

S… Speaker 1 (New Recording 9)

So you get,

37:36

S… Speaker 1 (New Recording 9)

basically,

37:37

S… Speaker 1 (New Recording 9)

activation of this pathway.

37:40

S… Speaker 1 (New Recording 9)

You get your beta -catenin,

37:42

S… Speaker 1 (New Recording 9)

and it goes intranuclei,

37:44

S… Speaker 1 (New Recording 9)

where you get proliferation and propagation of...

37:48

S… Speaker 1 (New Recording 9)

types of tumors when you have an activating mutation in this gene.

37:52

S… Speaker 1 (New Recording 9)

There is your NOTCH pathway which is also active

37:56

S… Speaker 1 (New Recording 9)

in dysmoid tumors.

37:58

S… Speaker 1 (New Recording 9)

When it is dysregulated,

37:59

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NOTCH does activate downstream pathways.

38:03

S… Speaker 1 (New Recording 9)

You get your gamma secretanes which cleaves part

38:08

S… Speaker 1 (New Recording 9)

of this.

38:08

S… Speaker 1 (New Recording 9)

Then you get your NOTCH intercellular domain which goes inside

38:12

S… Speaker 1 (New Recording 9)

the nucleus,

38:14

S… Speaker 1 (New Recording 9)

causes propagation and progression.

38:17

S… Speaker 1 (New Recording 9)

And this is where,

38:19

S… Speaker 1 (New Recording 9)

basically,

38:20

S… Speaker 1 (New Recording 9)

Neurogesis tend to work as it is a gamma -syncrasy inhibitor.

38:24

S… Speaker 1 (New Recording 9)

Now,

38:25

S… Speaker 1 (New Recording 9)

the intercalation or the crosstalk between your beta -catenin

38:30

S… Speaker 1 (New Recording 9)

pathway and the notch pathway causes,

38:32

S… Speaker 1 (New Recording 9)

may further contribute to this point,

38:34

S… Speaker 1 (New Recording 9)

progression or hyphogenesis.

38:39

S… Speaker 1 (New Recording 9)

How do these patients present?

38:41

S… Speaker 1 (New Recording 9)

I think the majority of them do present with a

38:46

S… Speaker 1 (New Recording 9)

measurable mass,

38:48

S… Speaker 1 (New Recording 9)

whether it's painful or painless.

38:50

S… Speaker 1 (New Recording 9)

They can develop virtually at any body site.

38:54

S… Speaker 1 (New Recording 9)

However, we do like to divide them into extremity,

38:57

S… Speaker 1 (New Recording 9)

abdominal wall,

38:59

S… Speaker 1 (New Recording 9)

and intra -abdominally.

39:01

S… Speaker 1 (New Recording 9)

The intra -abdominal ones are the ones that are harder to treat due to

39:05

S… Speaker 1 (New Recording 9)

their surgical difficulty and resection.

39:07

S… Speaker 1 (New Recording 9)

And the majority of FAB patients actually have intra -abdominal

39:11

S… Speaker 1 (New Recording 9)

test points.

39:12

S… Speaker 1 (New Recording 9)

They can be multifocal at N1 sites.

39:16

S… Speaker 1 (New Recording 9)

I do have a couple of patients with extremity samples

39:20

S… Speaker 1 (New Recording 9)

that have multifocal type of tumors.

39:24

S… Speaker 1 (New Recording 9)

These are very difficult to insect.

39:25

S… Speaker 1 (New Recording 9)

And symptoms depend on vocation and size.

39:33

S… Speaker 1 (New Recording 9)

So, how common is each anatomical location?

39:37

S… Speaker 1 (New Recording 9)

So, we're going to talk about extra -abdominal,

39:40

S… Speaker 1 (New Recording 9)

well,

39:40

S… Speaker 1 (New Recording 9)

actually, let's start with abdominal wall,

39:42

S… Speaker 1 (New Recording 9)

because that's probably the easiest.

39:43

S… Speaker 1 (New Recording 9)

It represents about 20 % of these dyspoints.

39:47

S… Speaker 1 (New Recording 9)

Your intra -abdominal,

39:49

S… Speaker 1 (New Recording 9)

which are the difficult ones to treat,

39:51

S… Speaker 1 (New Recording 9)

are about 20%.

39:52

S… Speaker 1 (New Recording 9)

Your joints and extremities are 30%,

39:55

S… Speaker 1 (New Recording 9)

and everything else is going to be 34%.

40:00

S… Speaker 1 (New Recording 9)

extra abdominal,

40:01

S… Speaker 1 (New Recording 9)

other than joint extremity,

40:03

S… Speaker 1 (New Recording 9)

so your head and neck,

40:04

S… Speaker 1 (New Recording 9)

neck or chest wall.

40:06

S… Speaker 1 (New Recording 9)

What symptoms

40:10

S… Speaker 1 (New Recording 9)

do these patients complain of?

40:13

S… Speaker 1 (New Recording 9)

So there is the physical part,

40:15

S… Speaker 1 (New Recording 9)

and of course there is the psychological impact.

40:19

S… Speaker 1 (New Recording 9)

If we're going to talk about the physical part,

40:21

S… Speaker 1 (New Recording 9)

the spigament,

40:22

S… Speaker 1 (New Recording 9)

these patients typically have enlarging masses

40:26

S… Speaker 1 (New Recording 9)

that are difficult to control.

40:28

S… Speaker 1 (New Recording 9)

The majority of them would actually present,

40:30

S… Speaker 1 (New Recording 9)

unfortunately,

40:31

S… Speaker 1 (New Recording 9)

late.

40:32

S… Speaker 1 (New Recording 9)

So these patients,

40:33

S… Speaker 1 (New Recording 9)

because of the location,

40:34

S… Speaker 1 (New Recording 9)

do get neuropathic type of pain,

40:37

S… Speaker 1 (New Recording 9)

decreased range of motion,

40:39

S… Speaker 1 (New Recording 9)

especially the extremity ones,

40:40

S… Speaker 1 (New Recording 9)

pain and fatigue.

40:43

S… Speaker 1 (New Recording 9)

And of course,

40:44

S… Speaker 1 (New Recording 9)

psychologically,

40:44

S… Speaker 1 (New Recording 9)

it has a major impact as well on these patients.

40:47

S… Speaker 1 (New Recording 9)

The majority of them have some psychological

40:52

S… Speaker 1 (New Recording 9)

issues,

40:53

S… Speaker 1 (New Recording 9)

such as fear,

40:55

S… Speaker 1 (New Recording 9)

insomnia,

40:56

S… Speaker 1 (New Recording 9)

and concern about lack of knowledge about healthcare providers.

41:02

S… Speaker 1 (New Recording 9)

We lack a lot of knowledge.

41:05

S… Speaker 1 (New Recording 9)

In

41:09

S… Speaker 1 (New Recording 9)

terms of symptom burden,

41:11

S… Speaker 1 (New Recording 9)

the majority or the highest burden of symptom would actually

41:15

S… Speaker 1 (New Recording 9)

be pain.

41:16

S… Speaker 1 (New Recording 9)

And I suspect also issues with

41:20

S… Speaker 1 (New Recording 9)

movements specifically when tumors are close to joints or

41:25

S… Speaker 1 (New Recording 9)

are in extremities.

41:27

S… Speaker 1 (New Recording 9)

And of course there is an impact on quality of life,

41:29

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difficulty in doing certain activities,

41:32

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whether they're moderate or vigorous,

41:35

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hopelessness,

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frustration,

41:38

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insomnia,

41:40

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and fear of the unknown.

41:42

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And this is basically data

41:46

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from a natural history study on testosterone tumors.

41:49

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How do we biopsy these tumors?

41:53

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Cold biopsies is mandated.

41:57

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We always argue against excisional biopsies

42:01

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because there is a risk when you do an excisional biopsy that you leave tumor

42:05

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behind and the risk of progression would actually be higher.

42:09

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And of course there is also a theoretical risk of seeding.

42:13

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With coronatal biopsy,

42:17

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I think pathologists are...

42:20

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known to actually diagnose these studies.

42:22

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Immunohistochemically,

42:24

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these tumors have positivity environmentin,

42:28

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smooth muscle actin,

42:30

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and nuclear beta -catenin.

42:32

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Molecular testing is encouraged,

42:35

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so doing a large panel NGS,

42:38

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working for CT and MP1 mutations.

42:41

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These are highly sensitive for core biopsies,

42:43

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and these are diagnostics of these good tumors.

42:49

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What about FAPs?

42:51

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I'm not sure what my other colleagues are doing,

42:54

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but any patient who's young who comes to us with this one should

42:58

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be referred to our cancer genetics clinic to get H &Y testing

43:03

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looking specifically for FAP.

43:04

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So getting an accurate family history is

43:08

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essential, specifically for colorectal cancer,

43:11

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and 70 % of FAP patients should develop colorectal cancer

43:15

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at any one point.

43:17

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patients should be offered the colonoscopy and germline genetic testing.

43:20

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If they have more than or equal

43:25

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to one of these two,

43:25

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their age at presentation is less than 40,

43:29

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they have a retroabdominal or retroperitoneal dyspoin tumor,

43:33

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or they have multifocal disease,

43:35

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or there is a family history of colorectal cancer.

43:39

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I mentioned,

43:39

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I think, previously in a prior talk,

43:42

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one of my first patients was a 28 -year -old guy with rectal cancer

43:46

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who developed colorectal cancer and had

43:50

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two of his family members die from colorectal cancer.

43:53

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We did her blood testing and he actually had fat.

43:56

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I have a question for the

44:02

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previous slide

44:07

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regarding do you really have evidence about the seeding?

44:27

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I'm not sure there is a specific evidence,

44:30

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but I have seen a case where this has actually seen

44:34

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it. At this point it is fibromatosis,

44:36

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it is car tissue.

44:38

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So if you puncture it and pull it,

44:40

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well, if you do an excisional biopsy,

44:42

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non -oncological biopsy where you actually cut through the

44:46

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tumor, I think the risk of progression and perhaps ceiling is actually there.

44:50

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The overall chance

44:54

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of finding FAB without prior history is 45%,

44:57

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so it is relatively low if you have any FAB.

45:00

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Family history,

45:00

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they should be managed as per the cancer genetics sort of guidelines.

45:05

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And when you have an APC mutation found,

45:07

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then this is a clear diagnosis and patients should

45:11

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be offered a prophylactic colectomy after discussion with

45:15

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a cancer geneticist.

45:16

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And of course,

45:17

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they should get on -road at this point surveillance.

45:21

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What's the best imaging modality?

45:23

S… Speaker 1 (New Recording 9)

It is actually MRI.

45:24

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There are certain MRI characteristics for disjoint

45:29

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tumors and basically in the T2 signaling

45:33

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you get hypo -intense bands in about 90 % of

45:37

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your abdominal wall at disjoints and this is very characteristic.

45:41

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When you give contrast with gadolinium it does

45:45

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enhance and over time with

45:49

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treatment.

45:51

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This T2 signal should decrease and this may actually indicate

45:55

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treatment response.

45:57

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And of course,

45:57

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this should be done by our expert radiologists.

46:01

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This is the last part of the first

46:05

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talk.

46:05

S… Speaker 1 (New Recording 9)

Thank

46:11

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you, Kenan.

46:11

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We'll now move to the discussion.

46:14

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I want to hear from all of you.

46:16

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Please use the mic because most of the questions are

46:20

S… Speaker 1 (New Recording 9)

driven by the surveying.

46:25

S… Speaker 1 (New Recording 9)

Okay, this is one of the surveys.

46:27

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If you look,

46:29

S… Speaker 1 (New Recording 9)

a colonist is radiology,

46:31

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100 % for biopsy,

46:34

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and we have 43 % talking about genetic tests.

46:39

S… Speaker 1 (New Recording 9)

So,

46:40

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the question here,

46:42

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do you have a pathologist specialized in sarcoma in your center?

46:46

S… Speaker 1 (New Recording 9)

Maybe we'll start with Dr.

46:49

S… Speaker 1 (New Recording 9)

Abdullah, then Dr.

46:50

S… Speaker 1 (New Recording 9)

Aisha.

46:51

S… Speaker 1 (New Recording 9)

Yes,

46:54

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we are.

47:22

S… Speaker 1 (New Recording 9)

Yes,

47:23

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we do have Yes,

47:27

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we do have

47:33

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Like all suspected dismalate cases reviewed by

47:37

S… Speaker 1 (New Recording 9)

them or only complex certain cases?

47:40

S… Speaker 1 (New Recording 9)

I think,

47:42

S… Speaker 1 (New Recording 9)

sorry.

47:43

S… Speaker 1 (New Recording 9)

They want to hear you.

47:45

S… Speaker 1 (New Recording 9)

Yeah,

47:47

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they are reviewed by soft tissue and sarcoma.

47:50

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Everything, not only the complex cases,

47:52

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Dr.

47:52

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Ayesha.

47:53

S… Speaker 1 (New Recording 9)

Yes, usually most of the dysmoid cases,

47:56

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we try as much as possible to present them in our sarcoma

48:00

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MDT, where it's usually attended by the

48:05

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pathologist where they go through the slide and also confirm the diagnosis.

48:09

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So even dysmoid cases,

48:11

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intra -abdominal,

48:13

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discussed in the sarcoma MDT?

48:13

S… Speaker 1 (New Recording 9)

Yes.

48:14

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Most of the cases that are presented to us are discussed

48:18

S… Speaker 1 (New Recording 9)

in the sarcoma MDT meeting.

48:21

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For us,

48:22

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we'll send for a second expert opinion for the difficult cases.

48:26

S… Speaker 1 (New Recording 9)

So where are they in Jeddah?

48:27

S… Speaker 1 (New Recording 9)

Mostly they go to a campus in the area.

48:29

S… Speaker 1 (New Recording 9)

Oh, okay.

48:30

S… Speaker 1 (New Recording 9)

Great.

48:31

S… Speaker 1 (New Recording 9)

So what was you?

48:33

S… Speaker 1 (New Recording 9)

Same?

48:34

S… Speaker 1 (New Recording 9)

Actually,

48:35

S… Speaker 1 (New Recording 9)

the one who will decide to sit or not is the pathologist.

48:38

S… Speaker 1 (New Recording 9)

Okay.

48:39

S… Speaker 1 (New Recording 9)

But many cases sit as Dr.

48:41

S… Speaker 1 (New Recording 9)

Rahman said outside.

48:42

S… Speaker 1 (New Recording 9)

Okay.

48:42

S… Speaker 1 (New Recording 9)

So what's up?

48:44

S… Speaker 1 (New Recording 9)

Actually,

48:46

S… Speaker 1 (New Recording 9)

you, but in our...

48:47

S… Speaker 1 (New Recording 9)

We have a sarcoma pathologist,

48:49

S… Speaker 1 (New Recording 9)

so most of the case is diagnostic.

48:56

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Most of the cases diagnosed in our center,

48:58

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however,

48:59

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they still have a reference with King Faisal,

49:03

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and also we do have a clinic as well.

49:05

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In the meantime,

49:06

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we raised an important element.

49:08

S… Speaker 1 (New Recording 9)

For the intra -abdominal,

49:10

S… Speaker 1 (New Recording 9)

it is discussed in the GI tumor board.

49:13

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But for the moid of the extremities,

49:16

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we are discussing in the sarcoma tumor board.

49:19

S… Speaker 1 (New Recording 9)

Exactly, because the GI or the surgical oncology is doing the intra -abdominal.

49:24

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Yes, so similar to Dr.

49:26

S… Speaker 1 (New Recording 9)

Meribeth,

49:27

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so most of the abdominal and intra -abdominal are discussed initially

49:31

S… Speaker 1 (New Recording 9)

in the GI tumor board,

49:32

S… Speaker 1 (New Recording 9)

but eventually then they will be discussed again in the GI tumor

49:37

S… Speaker 1 (New Recording 9)

board.

49:37

S… Speaker 1 (New Recording 9)

Okay.

49:39

S… Speaker 1 (New Recording 9)

What are the main gaps in pathology diagnosis for this morning?

49:44

S… Speaker 1 (New Recording 9)

Do you think there is gaps in diagnosis in terms of pathology?

49:48

S… Speaker 1 (New Recording 9)

If we have a specialized pathologist,

49:52

S… Speaker 1 (New Recording 9)

do you think there is?

49:54

S… Speaker 1 (New Recording 9)

I think maybe the main gap is the availability

49:59

S… Speaker 1 (New Recording 9)

of the tissue.

50:00

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Sometimes it's difficult to get enough tissue for the

50:04

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diagnosis.

50:04

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Sometimes you need to repeat the biopsia again to reach the

50:08

S… Speaker 1 (New Recording 9)

final diagnosis.

50:09

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But overall,

50:11

S… Speaker 1 (New Recording 9)

if there is enough tissue,

50:12

S… Speaker 1 (New Recording 9)

the diagnosis by an expert pathologist is not

50:17

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difficult.

50:19

S… Speaker 1 (New Recording 9)

Yeah, it is the same like Dr.

50:21

S… Speaker 1 (New Recording 9)

Ali pointed.

50:22

S… Speaker 1 (New Recording 9)

So I believe the initial part is the time

50:26

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of the biopsy.

50:27

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And because we do have MSK,

50:30

S… Speaker 1 (New Recording 9)

which is the musculoskeletal team for the IR,

50:34

S… Speaker 1 (New Recording 9)

for the extremities,

New Recording 9

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