Hi again Concerned, As usual, this is not a substitute for medical
advice or direct medical evaluation and treatment.
Let me go through your question step-by-step:
========
Definition
========
Also known as desmoid tumors or desmoid fibromatosis, these are, as
you say, relatively rare tumors. Although generally considered
benign, there is an aggressive form called aggressive fibromatosis
that can grow rapidly.
http://whippetrun_bc.tripod.com/the_fibromatosis_page/
More information can be found in this eMedicine article:
"Desmoid tumors are histologically benign fibrous neoplasms
originating from the musculoaponeurotic structures throughout the
body. The term desmoid, coined by Muller in 1838, is derived from the
Greek word desmos, which means tendonlike.
Desmoid tumors often appear as infiltrative, usually
well-differentiated, firm overgrowths of fibrous tissue, and they are
locally aggressive. The synonym aggressive fibromatosis describes the
marked cellularity and aggressive local behavior. This course and the
tendency for recurrence make the treatment of these relatively rare
fibrous tumors challenging.
Pathophysiology: Although desmoid tumors most commonly arise from the
rectus abdominis muscle in postpartum women and in scars due to
abdominal surgery, they may arise in any skeletal muscle. The tumors
tend to infiltrate adjacent muscle bundles, frequently entrapping them
and causing their degeneration. Although fixation to
musculoaponeurotic structures is apparent, the overlying skin is
normal. The myofibroblast is the cell considered to be responsible for
the development of desmoid tumors (see Procedures)."
The above article also discusses frequency and possible causes,
including a possible link to hormones.
As you state, these are rare tumors:
"Though described over a century ago, there is a relative paucity of
data concerning desmoids. In the relative paucity of data concerning
desmoids. In the general population, they are rare, accounting for
0.03% of all neoplasms,[7,8] and have an estimated incidence of 2?4
per million per year,[9] though this figure may reflect a lack of
reporting of these tumours."
There has also been an association with both Gardner's Syndrome and
Familial Adenomatous Polyposis (FAP):
"An association with familial adenomatous polyposis of the colon (FAP)
has been well documented,[10,11] a has a more pronounced association
with the subgroup, Gardner?s syndrome.[12] Both abdominal and
extra-abdominal desmoids occur more commonly in FAP patients,
exhibiting an incidence of 3.5?32%.[12?16] In the original Gardner
kindred the incidence was 29%.[12]"
Shields CJ. Winter DC. Kirwan WO. Redmond HP. Desmoid tumours.
[Review] [71 refs] [Journal Article. Review. Review, Tutorial]
European Journal of Surgical Oncology. 27(8):701-6, 2001 Dec.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11735163&query_hl=1
This article is not freely available online, but you can request a
free reprint from Dr. Redmond in Ireland:
profredmond@eircom.net
This is an excellent review article and gives more details than the
eMedicine article in terms of possible etiologies by which these
tumors arise and treatment considerations.
========
Treatment
========
The rarity of desmoid tumours, coupled with the variability in their
clinical course, renders these lesions a vexing entity, and makes
demonstration of the efficacy of any specific intervention difficult.
Surgical excision remains the principle therapeutic manoeuvre, however
the scope of the resection and the influence of involved margins on
local recurrence remain controversial."
Shields CJ. Winter DC. Kirwan WO. Redmond HP. Desmoid tumours.
[Review] [71 refs] [Journal Article. Review. Review, Tutorial]
European Journal of Surgical Oncology. 27(8):701-6, 2001 Dec.
This article also discusses chemotherapy and radiation therapy, which
are not as effective as wide surgical resection.
Here is a summary of treatment options from the eMedicine article above:
"Medical Care:
Radiation therapy may be used as a treatment of recurrent disease or
as primary therapy to avoid mutilating surgical resection.
Pharmacologic therapy with antiestrogens and prostaglandin inhibitors
may also be used.
In cases of recurrent extra-abdominal desmoid tumors in which surgery
is contraindicated or in cases of recurrence, a chemotherapeutic
regimen of doxorubicin, dacarbazine, and carboplatin may be effective.
Surgical Care:
Aggressive, wide surgical resection is the treatment of choice.
Complete surgical excision of desmoid tumors is the only effective method of cure."
The following should also be noted:
"With an incidence of 0.03%,[7,8,14] many surgeons will encounter a
negligible number during a career. Failure to recognize the potential
for malignant behaviour in this tumour renders desmoids susceptible to
inadequate treatment."
==================
Regrowth / Recurrence
==================
Again from eMedicine:
"Local recurrence rates are reported to be as high as 70%."
The following article has more to say about recurrent abdominal fibromatosis.
"Recommendations for drug therapy may differ slightly for patients
with recurrent, unresectable intraabdominal desmoids. These tumors
commonly arise after colon resection for familial adenomatous
polyposis [5]. They are notoriously difficult to adequately excise,
with very frequent local recurrence. It is unclear to what extent this
is due to failure to obtain adequate surgical margins and to what
extent the desmoids arise as a direct result of surgical trauma within
the abdomen, setting up a self-sustaining cycle with each attempt at
resection [42]. For the same reasons, intraabdominal desmoid tumors
are frequently multiple.
If the desmoid(s) are small, asymptomatic and unlikely to cause bowel
obstruction, an initial trial of NSAID therapy is warranted. Sulindac
therapy, with appropriate precautions against gastric bleeding, or a
cyclooxygenase 2 inhibitor could be tried. Even if the tumor does not
shrink immediately, in the absence of clear progression of tumor the
therapeutic trial should continue until it is clear that no benefit is
likely, since many months of treatment may be necessary.
If NSAID therapy fails, a trial of antiestrogen therapy is indicated,
again with enough time to clearly evaluate an effect. If antiestrogens
fail, or in any situation where the desmoids are large or threaten
bowel obstruction or fistula formation, chemotherapy should be
instituted. Hopefully, one or more of these measures will result in
stabilization and relief of pain."
Samuels BL. Management of recurrent desmoid tumor after surgery and
radiation: role of cytotoxic and non-cytotoxic therapies. [Review] [42
refs] [Case Reports. Journal Article. Review. Review, Tutorial]
Surgical Oncology. 8(4):191-6, 1999 Dec.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11128832&query_hl=8
This article is not freely available online, but you can request a
reprint from Dr. Samuels:
brian.samuels@advocatemedical.com
======
Experts
======
Surgery:
At UCLA, the following surgeons have published articles on desmoid
tumors / aggressive fibromatosis:
Dr. Frederick Eilber
UCLA Department of Surgical Oncology
(310) 825-7086
http://www.surgery.medsch.ucla.edu/oncology/doctors_Eilber.shtml
http://www.amjclinicaloncology.com/pt/re/ajco/abstract.00000421-199812000-00011.htm;jsessionid=DkJAou5uRzAYevi2996p6ec4F5j71tn548NxkQ1dfymAelUlvNsA!-1213525936!-949856144!9001!-1
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9856660&query_hl=12
Dr. Fritz Eilber is also on staff in surgical oncology at UCLA:
http://www.surgery.medsch.ucla.edu/oncology/doctors_EilberFritz.shtml
The surgical oncology group at UCLA is probably one of the best places
to start in looking for treatment.
_____
Radiation treatment:
A group from the Jonsson Comprehensive Cancer Center at UCLA recently
published one of the major papers on the role of radiation in the
treatment of recurrent fibromatosis. Here is the reference and links
to their department:
Goy BW, Lee SP, Eilber F, Dorey F, Eckardt J, Fu YS, Juillard GJ,
Selch MT. The role of adjuvant radiotherapy in the treatment of
resectable desmoid tumors. Int J Radiat Oncol Biol Phys. 1997 Oct
1;39(3):659-65.
http://www.radonc.ucla.edu/
Dr. Michael T. Selch
http://www.radonc.ucla.edu/faculty/selch/index.htm
Dr. Guy Juillard
http://www.radonc.ucla.edu/faculty/juillard/index.htm
Dr. Steve P. Lee
http://www.radonc.ucla.edu/faculty/lee/index.htm
=============================
Other references and resources:
Nuyttens JJ, Rust PF, Thomas CR Jr, Turrisi AT 3rd. Surgery versus
radiation therapy for patients with aggressive fibromatosis or desmoid
tumors: A comparative review of 22 articles. Cancer. 2000 Apr
1;88(7):1517-23. Review.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=10738207&query_hl=3
Related articles from PubMed:
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Display&dopt=pubmed_pubmed&from_uid=10738207
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Display&dopt=pubmed_pubmed&from_uid=11128832
The Association des Desmoide (France):
http://orphanet.infobiogen.fr/associations/ASD/anglaispro.html
St. Jude Hospital site with information on juvenile fibromatosis:
http://www.stjude.org/disease-summaries/0,2557,449_2167_10435,00.html
Vitamin D3 has been thought to be a potential treatment for
fibromatosis. You can read more in this article:
Ferah Y, Ayse K, Mustafa C, Ugur S, Murat G, Lale AI. Possible
therapeutic role of vitamin D3 in aggressive fibromatosis. Jpn J Clin
Oncol. 2004 Aug;34(8):472-5.
The article is freely available here:
http://jjco.oxfordjournals.org/cgi/content/full/34/8/472
The American Society of Clinical Oncology had a relevant poster session in 2004:
Therapy of aggressive fibromatosis is still an open question: A series
of patients treated at a single institution. Journal of Clinical
Oncology, 2004 ASCO Annual Meeting Proceedings (Post-Meeting Edition).
Vol 22, No 14S (July 15 Supplement), 2004: 9060.
http://www.asco.org/ac/1,1003,_12-002636-00_18-0026-00_19-002775,00.asp
=============================
Search terms:
Google Scholar:
( desmoid OR fibromatosis) abdomen (ucla OR usc) surgery
limited to 1995-2006
http://scholar.google.com/scholar?hl=en&lr=&safe=off&q=%28+desmoid+OR+fibromatosis%29+abdomen+%28ucla+OR+usc%29+surgery&as_ylo=1995&as_yhi=2006&btnG=Search
I also did searches using "los angeles" rather than specific
institutions. I also searched the medical literature directly on Ovid
Medline specifying institutions in the Los Angeles area.
"aggressive fibromatosis" site:emedicine.com
desmoid tumor
Multiple similar searches on Ovid MedLine and PubMed.
=============================
I hope this information is useful. Please feel free to request
clarification prior to rating.
Best,
-welte-ga |
