Calcifying aponeurotic fibroma

Rob Smith said:

My 12-year old son has a calcifying aponeurotic fibroma
(CAF) (finger). It has been excised twice, but has not
been totally removed. Thought to be only benign tumors,
recent evidence suggests CAFs may be able to turn
malignant.

Has anybody had, or know of someone with this condition?
If so, have there been any other health problems
associated with it? Are there any internet sites or
research papers that you could recommend on this subject?

I understand this is a rare condition, but any help would
be very much appreciated.

This is what I found
ard.bmjjournals.com78Open ↗ Unusual
and memorable

(may be confused with something "rheumatological or
metabolic "😉

Under "Benign tumors" here
emedicine.comtopic377.htmOpen ↗

tinyurl.com2aa5kOpen ↗ (PubMed) Calcifying aponeurotic
fibroma: a clinicopathologic study of 22 cases arising in
uncommon sites. Department of Soft Tissue Pathology, Armed
Forces Institute of Pathology, Washington, DC 20306-6000,
USA. Calcifying aponeurotic fibroma is a rare soft tissue
tumor that primarily occurs in children and adolescents and
has a strong predilection for the distal portion of the
extremities, especially the hands and feet. <skipping a
bunch which you'll read> The tumors were managed by local
excision (n=20), incomplete local excision (n=1) and biopsy
only (n=1). Follow-up information was available for 10
patients with a median follow-up interval of 94 months.
Five patients (50%) developed one or more recurrences.
Familiarity with this entity should help to avoid confusion
with other processes, including infantile and
extraabdominal fibromatoses, a chondroma of soft parts, and
a fibrous hamartoma of infancy. PMID: 9865839 [PubMed -
indexed for MEDLINE]

pathologyoutlines.comsofttissuepf.htmlOpen ↗ Calcifying
aponeurotic fibroma Soft tissue mass, usually in hands/feet
of children or young adults Tend to recur locally especially
in children; do not metastasize May be aneuploid although
benign, Cancer 1994;73:1200 May be cartilaginous analog of
fibromatosis Gross: nodule or infiltrating mass in
subcutaneous tissue or tendon, may be calcified Micro:
diffuse growth of plump or epithelioid fibroblasts with
cartilage and spotty calcification; may infiltrate fat,
striated muscle at periphery; rare mitotic figures, no
atypia, frequent osteoclast-like giant cells, chondrocyte
like cells surround calcified foci Positive stains:
vimentin, CD68, CD99, S100, muscle specific actin (50%),
smooth muscle actin (50%)
DD: rheumatoid nodule, schwannoma, fibromatosis, fibrous
hamartoma of infancy Reference: Hum Path 1998;29:1504"

So it looks like they're dealt with surgically (unless Steph
or others have a comment) and benign. J-not an expert

In misc.kids.health Rob Smith <[email hidden]> wrote:
: My 12-year old son has a calcifying aponeurotic fibroma
: (CAF) (finger). It has been excised twice, but has not
: been totally removed. Thought to be only benign tumors,
: recent evidence suggests CAFs may be able to turn
: malignant.
:
: Has anybody had, or know of someone with this condition?
: If so, have there been any other health problems
: associated with it? Are there any internet sites or
: research papers that you could recommend on this subject?
:
: I understand this is a rare condition, but any help would
: be very much appreciated.

tinyurl.com22sonOpen ↗

Best of luck to you and your son, Rob.

Richard Micaela's dad

"J" <[email hidden]> wrote in message
"]news:[email hidden]...

Quoted message said:

This is what I found
ard.bmjjournals.com78Open ↗
Unusual and memorable

(may be confused with something "rheumatological or
metabolic "😉

Under "Benign tumors" here
emedicine.comtopic377.htmOpen ↗

snip

Thanks very much J for going to this trouble, it is much
appreciated. After reviewing the articles you found, it
seems that calcifying aponeurotic fibroma (CAF) is usually
written up as being benign. However, one concern is that we
have seen a reference to an article titled: "Rare Malignant
Transformation of a Calcifying Aponeurotic Fibroma". Has
anyone seen this article by Rajkumar Amaravati, Christopher
J Zielinski, and Ralph S. De Simone? The article was
published in the Journal of Bone & Joint Surgery, the
reference is: JBone Joint Surg Am 2002 84: 1889 October 1
2002, 84 [10].

If anyone can give me a brief summary of the conclusions
reached in this article, this would be much appreciated.

Regards, Rob Smith [email hidden] (genuine e-
mail address)

(apologies if this shows up twice--slight bobble with the
news reader)

Rob Smith said:

However, one concern is that we have seen a reference to
an article titled: "Rare Malignant Transformation of a
Calcifying Aponeurotic Fibroma". Has anyone seen this
article by Rajkumar Amaravati, Christopher J Zielinski,
and Ralph S. De Simone? The article was published in the
Journal of Bone & Joint Surgery, the reference is: JBone
Joint Surg Am 2002 84: 1889 October 1 2002, 84 [10].

I'm not sure from what you wrote if you already visited
PubMed (<URL:http://www.pubmed.gov>), but here's the limited
bit they've got: <URL:http://www.ncbi.nlm.nih.gov/entrez/qu-
ery.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=123-
77925>. There's no abstract available, but there's a link to
the full article; unfortunately it'll cost you $20 to read
the thing.

"Kathy Cole" <[email hidden]> wrote in message
"]news:[email hidden]...

Quoted message said:

(apologies if this shows up twice--slight bobble with the
news reader)

Rob Smith said:

However, one concern is that we have seen a reference to
an article titled: "Rare Malignant Transformation of a
Calcifying Aponeurotic Fibroma". Has anyone seen this
article by Rajkumar Amaravati, Christopher J Zielinski,
and Ralph S. De Simone? The article was published in the
Journal of Bone & Joint Surgery, the reference is: JBone
Joint Surg Am 2002 84: 1889 October 1 2002, 84 [10].

I'm not sure from what you wrote if you already visited
PubMed (<URL:http://www.pubmed.gov>), but here's the
limited bit they've got:

<URL:http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retr-
ieve&db=pubmed&do pt=Abstract&list_uids=12377925>.

Quoted message said:

There's no abstract available, but there's a link to the
full article; unfortunately it'll cost you $20 to read
the thing.

I think it's important to remember that doctors and
scientists in general tend to only write papers when they
have something interesting or "different" to present.
Therefore when searching the web you are more likely to find
papers that describe unusual outcomes than ones describing
normal or common outcomes. A paper that says "we gave the
usual treatment and the patient got better" is unlikely to
be of interest to publishers or other doctors. A quick
Google suggests that there is a high rate of recurrence
(50%) but that they "rarely turn malignant".

You could also try contacting one of the authors if you want
to get a copy of his paper. I don't recommend you ask for
medical advice just a copy of their paper. You might find
that it describes just the one case.

Google appears to have found two of them quite easily..

culpeperhospital.comPhysMstr.aspOpen ↗

vaorthopaediccenter.commeetus.htmOpen ↗

"CWatters" <[email hidden]> wrote in message
"]news:[email hidden]...

Quoted message said:

I think it's important to remember that doctors and
scientists in general tend to only write papers when they
have something interesting or "different" to present.
Therefore when searching the web you are more likely to
find papers that describe unusual outcomes than ones
describing normal or common outcomes. A paper that says
"we gave the usual treatment and the patient got better"
is unlikely to be of interest to publishers or other
doctors. A quick Google suggests that there is a high rate
of recurrence (50%) but that they "rarely turn malignant".

You could also try contacting one of the authors if you
want to get a copy of his paper. I don't recommend you ask
for medical advice just a copy of their paper. You might
find that it describes just the one case.

Google appears to have found two of them quite easily..

culpeperhospital.comPhysMstr.aspOpen ↗

vaorthopaediccenter.commeetus.htmOpen ↗

Thanks "J", Colin, Kathy, and Richard for your helpful
postings to my enquiry, they are much appreciated. I agree
that it would be worthwhile to read the full article by
Amaravati, Zielinski, and De Simone "Rare Malignant
Transformation of a Calcifying Aponeurotic Fibroma".
However, it would have been helpful if the authors could
have included one or two sentences about the key findings of
their research in an abstract.

So in the absence of the finding in the above study, we at
least know that a malignant transformation of a calcifying
aponeurotic fibroma (CAF) is likely to be rare and that CAFs
are generally benign.

If we were to assume that there is only a 1% chance that the
CAF could become malignant and travel to the lungs, then
this still poses a difficult problem for surgeons. Should
they excise the tumor locally, or should they operate with
wide margins because of the 1% possibility that the tumor
may travel? Parents may perhaps be more conservative in
their outlook on this question than surgeons and opt for
wide margins, perhaps even removal of the finger or hand.

I guess large numbers of people have had to come to grips
with such a decision, but because CAF is such a rare
condition, there is even less to go on than would often
be the case.

Given that the literature had established that there is a 1
or 2% chance that a CAF could travel, what would be your
view on the extent of surgery that should be carried out?

Thanks for your help.

Rob Smith [email hidden]

Just one final comment. I note that it says "a" in the
title.

Quoted message said:

"Rare Malignant Transformation of _a_ Calcifying
Aponeurotic Fibroma".

Perhaps it does just cover one case?