Desmoid

tumors
 What are desmoid
tumors?

Desmoid tumors are cytologically bland fibrous
neoplasms originating from the musculoaponeurotic
structures throughout the body.

The term desmoid derived from the Greek word

desmos, which means tendonlike.

They often appear as : infiltrative, usually well-

differentiated, firm overgrowths of fibrous tissue, and
they are locally malignant .
Although desmoid tumors most commonly arise from
the rectus abdominis muscle, they may arise in any
skeletal muscle ;(shoulders, legs, or upper arms).

They also commonly grow in and around the organs
of the abdomen, such as the small or large intestines.

For this reason, doctors often talk about desmoid

tumors that are :
1. intra-abdominal desmoid tumors.
2. Extra-abdominal desmoid tumors.
 Termenology:

The tumor also may be called one of the
following:

*Aggressive fibromatosis.
*Deep fibromatosis.
*Desmoid fibromatosis .
*Familial infiltrative fibromatosis.
*Hereditary desmoid disease.
*Musculoaponeurotic fibromatosis.
 Epidemiology:
Frequency:

Overall, desmoid tumors are reported to account for 0.03% of all
neoplasms.
When present in patients with familial polyposis of the colon, the
prevalence of desmoid tumors is as high as 13%.
Sex:
Desmoid tumors most commonly occur in women after childbirth.
Desmoid tumors are twice as common in females than in males;
however, and the female-to-male ratio was 1.2:1. In children, the sex
incidence is equal.
Age:
Although desmoid tumors are more common in persons aged 10-40
years than in others, they do occur in young children and older adults.
causes of desmoid

tumors:
The cause of desmoid tumors is uncertain and may be
related to: risk factors
 Risk factors:

A family predisposition to cancer: A condition called
familial adenomatous polyposis (FAP), which is
caused by a genetic mutation on chromosome 5 , can
increase the risk of developing colorectal cancer and
predispose people to desmoid tumors. This is
sometimes called Gardener Syndrome.

Gardener Syndrome: familial adenomatous polyposis coli

+osteoma +sebaceous cyst +desmoid tumor
Hormonal factors: Desmoid tumors most commonly
appear in young women during or after pregnancy.
The tumors regress during menopause and after
tamoxifen treatment.

Desmoid tumors may regress after exposure to oral
contraceptives. The proliferative response of fibroblasts
to estrogen has been established.

Trauma: Some researchers believe that physical

trauma, particularly repeated trauma, might play a
role.
For example, some people can develop a desmoid tumor
after surgery or chronic injuries.
 Prognosis:

Despite their benign histologic appearance and
negligible metastatic potential, the tendency of
desmoid tumors to cause
1. local infiltration is significant in terms of:
(deformity, morbidity, and mortality resulting from
pressure effects )and
2. potential obstruction of vital structures and organs.

Local desmoid tumor recurrence rates are reported to

be as high as 70%.
A positive surgical margin is a significant risk factor
for recurrence.

Intra-abdominal desmoid tumors may kill patients
with familial adenomatous polyposis.

 Five-year survival rates of such patients with stage I,

II, III, and IV intra-abdominal desmoid tumors were
found to be 95%, 100%, 89%, and 76%, respectively.
 Clinical presentation
and behavior:

 The symptoms of desmoid tumors can vary greatly
and are dependent upon:
the tumor’s size and location, and how far it’s spread.

 The most common symptom of a desmoid tumor is a

relatively firm mass or lump ;The lump is often
mildly painful.
Other symptoms include:


1. rectal bleeding and severe abdominal pain when a
desmoid tumor in the abdomen and compresses the
intestine .

2. localized pain or stiffness when a desmoid tumor

compresses nerves, blood vessels, or an organ

3. limping and other mobility difficulties, particularly

when associated with a lump.
 Behavior:
It is important to know that the desmoid tumors that
present superficially on the abdominal wall behave
much differently than the ones that are deep inside the
abdomen or pelvis. 
One very interesting aspect of desmoid tumors is that
on some occasions they can shrink or become
quiescent (deep sleep) without any therapy.

 Other desmoid tumors may be stable or grow very

slowly over a period of months and years.
In conclusion, these tumors has a wide range of
behaviors and therefore their management is very
different.
 History:

Although desmoid tumors can arise in any skeletal
muscle, they most commonly develop in the anterior
abdominal wall and shoulder girdle.
Retroperitoneal neoplasms are more common in
familial polyposis coli and Gardner syndrome after
abdominal surgery than in other conditions.

A history of trauma (often surgical) to the site of the

desmoid tumor is elicited in 1 in 4 cases.
 Physical
examination:

1.Extra-abdominal desmoid tumors: (Peripheral
desmoid tumors)
are firm, smooth, and mobile. They often adhere to
surrounding structures. The overlying skin is usually
unaffected.
The presence of such a soft tissue growth should alert
the clinician to delve more deeply into the family history
for evidence of familial polyposis coli and Gardner
syndrome.
Extra-abdominal desmoid tumors are rare and may
be first evident as gradually increasing leg
swelling. Desmoid tumors may rarely appear on the
foot.

Extra-abdominal desmoid tumors may also be seen
(rarely) in the urological system, including in the
bladder and scrotum.

2. Intra-abdominal desmoid tumors:

remain asymptomatic until their growth and infiltration
cause visceral compression.
Symptoms of intestinal, vascular, ureteric, or neural
involvement may be the initial manifestations.
4. Breast desmoid tumors:
Desmoid tumors account for 0.2% of primary breast tumors,
developing from muscular fasciae and aponeuroses.
Desmoid tumors may mimic breast cancer.

Intra-abdominal desmoid tumor
 Diagnosis:
1. Ultrasound: used to show if the tumor is solid.

2. Imaging scans: (MRI), and (CT) scans.
These show whether the tumor is attached to other
tissues and whether it can be safely removed with
surgery.
3. Biopsy: The preferred diagnostic test is biopsy of the
tumor. A fine-needle aspiration biopsy specimen may be
considered.
On electron microscopic examination, the spindle cells
of desmoid tumors appear to be myofibroblasts.
The myofibroblast is the cell considered to be
responsible for the development of desmoid tumors .


4.Investigations for the presence of Gardner syndrome:
 Colonoscopy and fundal examination.

 Staging:

The stage is determined from the results of physical
exams, imaging tests and biopsies that have been done.
StageI :asymptomatic,<10 cm maximum diameter ;and
not growing .
StageII: mild symptoms; <10 cm; and not growing .
stageIII: modwrat symptoms ;or bowel/ureteric
obstruction ;or 10 to 20 cm ; or slowly growing .
StageIV: sever symptoms; or >20 cm ; or rapidly
growing.
 Differential
diagnosis:

Fibrosarcoma.
Infantile fibrosarcoma.
Reactive fibrosis .
Solitary (malignant)fibrous tumor.
Fibrous hematoma of infancy.
Lieomyoma (pelvic location).
Deep soft tissue lieomyoma in the foot.
Colonic malignancy in submucosal location.
 
Approach
Considerations:
1. Primary surgery with negative surgical margins is
the most successful primary treatment modality for
desmoid tumors. Positive margins after surgery reflect a
high risk for recurrence.

2. Radiation therapy :
 recurrent disease.
 as primary therapy to avoid disfiguring surgery .
 postoperatively, preoperatively.
 as the sole treatment.
 Radiotherapy alone for gross disease or after a
microscopically incomplete resection yields local
control rates of approximately 75-80%.
3. Pharmacologic therapy : pharmacotherapy are to
induce remission, to prevent complications, and to
reduce morbidity.
 antiestrogens.

 prostaglandin inhibitors.
 nonsteroidal anti-inflammatory drugs (NSAIDs).
 Antineoplastic agents: These agents inhibit cell
growth and proliferation as:
 Doxorubicin (Adriamycin, Rubex)
 Dacarbazine (DTIC-Dome)
 Carboplatin (Paraplatin)

 Pharmacologic agents result in objective response

rates of approximately 40-50%; the duration of
response is variable.
4. Physical therapy: Following both surgical and
nonsurgical treatment, a person may need physical
therapy to regain function in the areas affected by the
tumor; 
5.Magnetic resonance‒guided high-intensity focused
ultrasound: may prove a safe and effective option for
selected desmoid tumors.

6. Wait-and-see approach:
For tumors that are asymptomatic or nonprogressive,
some prefer
 Excision of the :
Tumor

Aggressive, wide surgical resection is the treatment of
choice.

This sometimes necessitates removal of most of an

anterior compartment of a leg.

Extensive cases may require excision plus adjuvant

treatment including: chemotherapy and repeat
surgery.
In selected patients, radical resection with
intraoperative margin evaluation by frozen sections
followed by immediate mesh reconstruction may be a

safe and effective procedure providing definitive cure
yet minimizing functional limitations.

Long-Term Monitoring:
After surgery, MRI may be useful for monitoring
desmoid tumor recurrence
 ‫‪‬‬
‫‪ ‬ايمان منير شريف علي عبد هللا ‪ ‬بسمه عصام عبد المنعم حسنين‬
‫‪ ‬بسمه محمد عبد الجواد عزام‬ ‫‪ ‬باسم شوقي ابراهيم داود‬
‫‪ ‬بسمه محمد علي سالم‬ ‫‪ ‬بسمه رزق محمد دعبس‬
‫‪ ‬تقي ايمن انور صابر‬ ‫‪ ‬بسمه رمزي كمال علي‬
‫‪ ‬بسمه عادل جوده محمد درويش ‪ ‬ثراء سيد محمد مصطفي خضر‬

‫‪ ‬منار خالد مصطفي‬