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Desmoid Tumor of the Anterior
Abdominal Wall
ABSTRACT
Desmoid tumors (also called aggressive fibromatosis, deep
musculoaponeurotic fibromatosis, and also formerly termed
fibrosarcoma grade I of the desmoid type) are locally aggressive
tumors with no known potential for metastasis or dedifferentiation.
These benign tumors are associated with a fertile women after
pregnancy as in this case who/whereby a felt small mass was felt in
a woman’s her right iliac fossa after her last labor//following
delivery. increasing in size by time. Many patients have a history of
breast cancer or breast surgery as in this case/patient who had
fibroadenoma of breast. Many patients usually have a history of
breast cancer or breast surgery and in the case of our patient, there
was history of fibroadenoma.
Preoperative evaluation included hemoglobin%, ultrasound and CT
scan. The mass was excised surgically without abdominal
reconstruction and subsequent histology revealed desmoid tumor.
The patient discharged with uneventful recovery.
Desmoid tumors (also called aggressive fibromatosis, deep
musculoaponeurotic fibromatosis, and also formerly termed
fibrosarcoma grade I of the desmoid type) are locally
aggressive tumors with no known potential for metastasis or
dedifferentiation. These benign tumors are associated with
fertile women after pregnancy and many patients usually
have a history of breast cancer or breast surgery.
This is a case report of a woman who gave history of
fibroadenoma and who was found to have had a small mass
her right iliac fossa following delivery.
The mass was found to be within the abdominal wall after
abdominal UltraSound (US) and Computed Tomography (CT)
scan of the abdomen was done. Full blood count, Urea,
electrolytes and creatinine were done and found to be
normal.
The mass was excised surgically without abdominal
reconstruction and subsequent histology revealed desmoid
tumor. The patient discharged with uneventful recovery.
Keywords: Desmoid tumor, Anterior abdominal wall, Gardner
syndrome
Introduction
Desmoid tumors are (also called desmoids fibromatosis) are rare
soft tissue tumors accounting for 0.03% of all tumors [2]. The term
desmoids was coined by Muller in 1838 and is derived from the
Greek word desmos, which means tendon like [1]. The tumors are
slowly growing benign fibrous tumors without any metastatic
potential but a have strong tendency to invade locally and to recur.
They are frequently seen in patients with familial adenomatous
polyposis (FAP) and are also usually seen in women between
puberty and 40 years of age [3]. Desmoid Tumors occasionally
exhibit rapid growth and are seen in the abdominal wall, intraabdominally and rarely on the extremities [3,4,5]
Surgery, although followed by frequent local recurrence, is generally
considered the treatment mainstay. Other treatment modalities
include radiotherapy, chemotherapy (using cyclophosphamide,
doxorubicin, tacarbazin, methotrexate, vincristine and actinomycinD). Hormonal therapy (tamoxifen) with either steroid or a potent
nonsteroidal anti-inflammatory drug (e.g., Indomethacin, sulindac
or Colchicine) has/have been used both preoperatively and
postoperatively [6]
CASE REPORT:
A 23-year- old married housewife not pregnant presented with a 9
months history of a painless right lower abdominal painless
mass/swelling which had developed her last delivery which had
been by cesarean section. She was multiparous and was not
pregnant at the time of presentation and she gave history of the
swelling having progressively increased in size. She reported having
experienced dizziness, poor appetite and weight loss but there was
no alteration in bowel habits. She had also started felling nauseated
one month prior to coming to hospital and also complained of
excessive menstrual blood loss. A breast lump (fibroadenoma) had
been excised from one of her breasts 2 years prior during her last
pregnancy.
On examination, she was anxious, thin, looked pale, not
jaundiced, and had no palpable lymph nodes. She had a cesarean
section scar seen, no abdominal striae and there was visible an
obvious large swelling at the right lower abdominal quadrant with
no overlying skin changes. The mass was not
moving
with
respiration, was firm, fixed, non-tender, well-defined and it’s
dimensions were 15 cm by 15 cm.
with
soft
surface and locally restricted mobility, not tender
,hard mass, clear edge, not
disappeared on raising the patient
from supine to sitting
posture and no palpable intraabdominal
organs
were noticed.
INVESTIGATIONS
Full blood count, urea, electrolytes and creatinine were normal.
Abdominal US was reported as showing evidence of a well-defined,
isogonic solid mass located at the right side of lower abdominal
cavity, contiguous with the anterior abdominal wall measuring
about 11 cm. by 7.5 cm and containing dots of calcification.
The CT scan of abdomen (Fig.1) confirmed an anterior abdominal
wall mass (9.8 cm x 8 cm) with a smooth outline and with no
evidence of invasion of the surrounding structures.
Fig.1 (CT scan of the abdomen)
Operation:
Under general anesthesia and through a right lower paramedian
incision the mass was excised from the anterior abdominal wall. It
was located between rectus abdominis muscle and the posterior
rectus sheath.
Please label figure 2&3 properly
with a caption for each
Fig.3 (Fig.2,3 Lateral view of the mass attached to posterior rectus
sheath.)
Fig.4 (View of the posterior rectus sheath after complete removal of
the mass.)
.
Fig.2
PATHOLOGY:
Histopathological ( Fig.5&6 ) result was benign spindle cell lesion
C/W fibromatosis (Desmoids tumor ) with no evidence of
malignancy is seen
Fig.5
Fig.6
Fig.5,6 ( Histopathological photos confirming Desmoids tumor)
DISCUSSION:
Desmoid tumors are rare and they account for about 0.03 percent
of all neoplasms and less than 3 percent of all soft tissue tumors
[ref]. The estimated incidence in the general population is two to
four per million population per year and they are slightly more
common in women than in men [7,8]. Most desmoids arise
sporadically, although approximately 2 percent are associated with
Familial Adenomatous Polyposis (FAP). Desmoid tumors affect
between 10 to 20 percent of patients with FAP [9]. They are an
unusual cause of a breast mass, and they may be mistaken for a
primary or recurrent breast cancer. Many patients have a history of
breast cancer or breast surgery. In a series of 32 patients with a
breast desmoid, eight (25 percent) had a previous history of breast
cancer, and 14 (44 percent) had prior breast surgery [10].
CONCLUSION:
REFERENCES
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Vikas,Avinash Pratap Singh, Puspendra Baghel, Varun Pendro,
Vishal Kirti Jain,Shrikant Jagdish Jai, Sanjeev Kumar, andM. C.
Songra
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and Steffen Bülow The Danish Polyposis Register, Department of
Surgical Gastroenterology, Hvidovre University Hospital,Hvidovre,
Denmark
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