Here, our topic is fibro-.
We had a discussion of lipo-.
This is a discussion of fibro-
under soft tissue diseases.
Under fibro-, in other words
fibroblasts or fibrosis,
is what you’re looking for.
Under this, we’ll divide this into
superficial and deep.
At first, we’ll take a look at superficial.
Under superficial, poorly defined fascicles
of mature-appearing fibroblasts
surrounded by abundant
amounts of collagen, of course,
then giving rise to your fibrosis.
Now the 2 major places that
you’re focusing upon
with superficial fibromatoses
would be the hand as you see in the picture
and then the penis in a male.
Whenever there is fibrosis, in general,
would you tell me as to what then happens
to that particular structure?
That would be contraction and
often times there will be
shrinking taking place, correct,
of that organ.
Big examples there would include the liver
undergoing cirrhosis and maybe perhaps
the lung undergoing fibrosis.
With that said,
if there’s fibrosis taking place here
superficially of the hand,
you’ll notice that the hand has
now become flexed
or the hand and then fingers
become flexed like a claw,
you then will call this Dupuytren palmer.
If the same kind of thing would
then occur to the penis,
then you would call this Peyronie’s disease.
Both of these are different locations
of the same complication,
known as superficial fibromatosis.
It will never, almost never metastasize,
but it is locally aggressive,
and often times, will recur
even after excision.
You’ll notice here that,
well this was supposed to be a bunch
of fascicles of your muscle,
but instead you see a sea,
a sea of fibroblasts laying down collagen.
This is fibromatosis.
Whereas superficial fibromatosis
was to the hand and to the penis,
Dupuytren and Peyronie respectively,
when you have fibromatosis taking
place in the deep tissue,
we now call this a Desmoid tumor.
D—desmoid, d—deep fibromatosis.
In terms of histology, it would
be that of the same
that you’ll find superficial,
so lots of fibrosis.
The categories extra-abdominal,
in other words, shoulder, chest
wall, back, and thigh.
If it’s intra-abdominal,
occur in the mesenteric or the pelvic walls.
And, if you’re thinking about familial
where the mutation taking place with APC
and chromosome-5 and WNT,
and all of the transcription factors,
with your deep fibromatosis,
now we call this a variant of FAP,
called Gardner syndrome.
Don’t forget that.
Once again, deep fibromatosis
association with familial adenomatosous
is then called Gardner syndrome.
Continue discussion of fibroblast issues
and soft tissue pathology.
Here we have fibrosarcoma.
So this is a malignancy,
of course, once again composed
of as the name applies,
fibroblasts and collagen,
middle age and elderly, deep tissue,
and this particular type of pattern that
you’ll find with fibrosarcoma
is referred to as, by the textile industry—
well they’re not the ones who actually
did the histologic picture here—
but I’m just saying, the name herringbone
was adopted from the textile industry,
and that’s the pattern you’d expect
to find in fibrosarcoma.
Here we have rhabdomyosarcoma.
What does rhabdomyo- mean?
Now we’re talking about skeletal muscle.
It’s a malignant tumor of a skeletal
Here, you’re thinking about sarcoma
taking place in childhood,
and if it is—
and I’ll talk about 2 different variants
here in one second
—but if it’s less than 20 years, then rhabdo-
myosarcoma is what you have.
Usually, it will then occur
at the head and neck
or the GU tract,
and look for the classic rhabdomyoblasts.
So once again, blasts then referring
to premature cells.
If you then did have rhabdomyosarcoma
in a young girl, let’s say for example,
less than 5 years of age,
and then way back in, well let’s
say, female reproductive—
let’s say that you’re looking
at this girl’s genitals
at 5 years of age, and you find
with the naked eye,
coming out of the vagina.
What do we call that?
That’s called embryonal rhabdomyosarcoma,
or in other words, you’ve heard
You have your rhabdomyosarcoma
or in other words called embryonal
in a girl perhaps less than 5 years of age—
look for that grapelike cluster.
But in general, if rhabdomyosarcoma,
less than 20 years,
has nothing to do with embryonal
Here we will take a look at
soft tissue pathology.
Unfortunately, quite common
in population—young ladies,
perhaps African American but
could as well be Caucasian,
maybe about 27 years of age,
and then she complains that
during her menses,
she feels an awful lot of pain.
So in other words, we’re talking
about fibroids or leiomyoma.
So during her menses,
not only is she losing quite a bit
of blood, but oh my goodness,
she’s feeling dysmenorrhea.
Right? Painful menses.
But what is leiomyoma?
Leiomyoma is a smooth muscle benign tumor
that is then occupying the space
within or could be
within the uterine cavity.
So you’ll call that intracavitary.
Or you could have a leiomyoma
that’s located underneath the mucosa.
You then call that submucosal leiomyoma.
Or you could have one that’s located
more in the periphery of the uterus
by the serosa, and then you would
then call that subserosal.
I bring those 3 locations
of the uterine fibroma,
or in other words, fibroids
because each one of them gives
you something different.
For example, if you find an intracavitary
type of leiomyoma—
if you actually take a look at the
picture on the right here,
see these polyp-like structures
on the uterus?
This is not a polyp.
These are well encapsulated smooth
muscle whorled pattern
which I will show you in description
histologically of the leiomyoma.
Should have fewer than
10 mitoses per 10 hpf.
What you’re looking at here a biopsy of leio-
myoma would be that of whorled pattern.
So it’s one of those
fibroids that we have taken
the biopsy of it, and you find
a whorled pattern
of the smooth muscle cells.
This is leiomyosarcoma.
This is a malignant smooth muscle tumor,
develops in skin and deep soft tissues
of extremities and retroperitoneum.
Once again, here’s an example where
a benign tumor such as a leiomyoma will not
commonly give rise to
a malignant smooth muscle tumor
Pathology here would be painless, firm mass
with malignant spindle cells
with cigar-shaped nuclei
arranged in interweaving fascicles.
A major issue within the synovium—
it’s called synovial cell sarcoma.
So for example, in the picture here of x-ray
of the knee and the joint capsule,
and what you’re seeing there is increased
opacity therefore represents
the synovium undergoing
in which it has now become a
sarcoma. A malignant tumor
of your hinge joint.
For example, let it be the elbow, and
in this case, the knee what have you.
Initially might have been thought of
raised from your synovium.
Majority; however, will develop to large
joints such as your knee and ankle,
and you should think about adults.
Let’s take a look at the detailed pathology
of synovial cell sarcoma.
Biphasic morphology of atypical spindle
cells and the glands.
Well if you take a look
at the picture here,
a beautiful picture of your spindle cells,
well if you see the vacuolated region there,
and so is lined vacuolated region,
are the glandular cells, alright?
Now think of joint capsule.
Think of the synovial cells responsible
for then secreting
synovial fluid into your joint
So now let’s say you have a
synovial cell sarcoma.
t only makes sense that you’d
have a combination of
spindle cells then surrounding
your glandular cells
that look like they’re about to secrete
fluid into that structure.
Classic translocation that you must
have committed to memory.
and a 5-year survival rate of 30% to 60%.
It’s quite variable.
This then now completes
our soft tissue pathology section
in which we then walk through
the different organization,
in other words, we looked at tumors
of, let’s say, the smooth muscle,
skeletal muscle, rhabdo-, and leio-.
We looked at issues of
the lipid such as lipo-
we finished it off by looking
at issues in the joint
such as synovial cell sarcoma.