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Mesenteric fibromatosis in a young girl without familialadenomatous polyposis

Sergio Huerta*, David R. Heubner, Daniel R. Marcus

Department of Surgery, University of California, Irvine, Orange CA 92868, USA

0022-3468/$ – see front matter D 2005

doi:10.1016/j.jpedsurg.2005.02.017

T Corresponding author.

E-mail address: shuerta@pol.net (S.

Index words:Mesenteric

fibromatosis;

Familial

adenomatous

polyposis;

Gardner’s syndrome

Abstract Mesenteric fibromatosis (MF) is a rare, benign tumor commonly associated with Gardner’s

syndrome. The signs and symptoms in patients with MF are insidious. Patients may present with

abdominal pain or discomfort when the tumors reach large sizes, which is typical at the time of

diagnosis. Differentiating MF from other neoplasms such as gastrointestinal stromal tumors may present

a diagnostic dilemma, especially in patients without any history of familial adenomatous polyposis. In

the present report, we discuss a young girl who presented with MF. A pertinent review of the literature is

also presented. This case is peculiar in that MF presented at a young age in a patient without history of

familial adenomatous polyposis and occurred in the omentum with local invasion to the stomach.

D 2005 Elsevier Inc. All rights reserved.

Mesenteric fibromatosis (MF) is a rare neoplasm with an

incidence of 2 to 4 new cases per million people per year.

This accounts for nearly 0.03% of all neoplasms [1].

Mesenteric fibromatosis is also known as deep fibroma-

toses, aggressive fibromatoses, and desmoid tumors. Prima-

ry MF is rare, but a few cases have been reported in the

literature [2-5]. Estrogen exposure appears to be a predis-

posing factor for mesenteric fibromatosis. For instance, the

incidence of MF is greater in women (80%) [6] than in men.

Secondly, MF appears to occur more frequently during

pregnancy [7]. There is also an increased risk of MF in

patients with exogenous estrogen intake [8]. Furthermore,

the incidence of MF is higher in premenopausal women

compared with postmenopausal women [7].

Trauma and previous abdominal surgery have also been

reported as predisposing factors for mesenteric fibromatosis

[9-11]. However, the strongest association is seen in patients

Elsevier Inc. All rights reserved.

Huerta).

with familial adenomatous polyposis (FAP) as a component

of Gardner’s syndrome [12,13]. In the present report, we

discuss a young girl with a large desmoid tumor who was

asymptomatic at the time of diagnosis and had no history

of FAP.

1. Case Report

A 13-year-old girl was referred to the surgical service

for management of a large, palpable intraabdominal mass

noticed only a few days before presentation by the

patient’s mother. Both the patient and the patient’s mother

denied noticing the mass before presenting to the

hospital. The patient denied any history of constitutional

symptoms including weight loss, fever, diarrhea, or

vomiting. Her past medical history was significant only

for attention deficit disorder (ADHD). She had no history

of abdominal surgeries. Her only medication was am-

phetamine/dextroamphetamine for 4 years for the treat-

ment of ADHD.

Journal of Pediatric Surgery (2005) 40, E33–E36

Fig. 1 Computed tomography scan of the abdomen demonstrated a low attenuating mass extending from the posteriolateral wall of the

stomach inferiorly.

S. Huerta et al.E34

Examination of her abdomen revealed no abdominal

distention and a well-circumscribed, mobile mass in the left

upper quadrant. She had no abdominal tenderness. Labora-

tory tests were all within normal limits. A computed

tomography scan of the abdomen revealed a well-encapsu-

lated, solid, low attenuating lesion over the left side of the

Fig. 2 Fresh tumor (A) demonstrates a mass measuring 15 � 13.5

homogeneous. Microscopic examination (C and D) demonstrates spindl

abdomen extending from the posterolateral wall of the

stomach inferiorly for approximately 14 cm. The liver,

spleen, pancreas, and adrenals were all radiographically

normal (Fig. 1).

At laparotomy, she had a large, well-defined mass in the

left upper quadrant arising from the ometum attached to the

� 7.3 cm and weighing 887 g. The cut surface (B) is pink and

e cells with low mitotic activity.

Mesenteric fibromatosis E35

greater curvature of the stomach. All the attachments of the

mesentery were divided, preserving the vascular supply to

the bowel and integrity of the capsule of the mass. The mass

was then detached from the stomach with a gastrointestinal

anastomosis device.

Pathological examination revealed a large mass mea-

suring 15 � 13.5 � 7.3 cm and weighing 887 g (Fig. 2A).

The cut surface demonstrated a pink-tan and homo-

geneous mass (Fig. 2B). Microscopic examination showed

desmin minimally positive, vimentin positive, s100 mini-

mally focally positive, cd34 negative, SMA focally

positive and MSA largely negative, and Cd117 positive

(Fig. 2B and C). These tumor markers were consistent

with mesenteric fibromatosis.

Her intraoperative and postoperative course was un-

eventful. The nasogastric tube was removed on postopera-

tive day 2 and oral intake started the following day. She was

discharged home on postoperative day 5. She was doing

well at 4 weeks of follow-up.

2. Discussion

Mesenteric fibromatosis occurs most commonly in the

mesentery of the small bowel and less frequently in the

mesocolon and omentum [9]. The age predilection for

this tumor is in the fourth decade of life. However, unusual

cases have been reported in neonates where MF arises

from the intestinal wall rather than the mesentery

[14,15]. Although uncommon, MF has also been reported

in children [16-18].

The signs and symptoms of mesenteric fibromatosis are

insidious and usually manifest when there is a large palpable

tumor resulting in abdominal discomfort or pain. Weight

loss is common in large tumors, but other constitutional

symptoms are rare. The differential diagnosis often includes

gastrointestinal stromal tumors and less commonly fibro-

sarcomas or inflammatory fibroid polyps.

Mesenteric fibromatosis is characterized by low mitotic

activity but a high propensity of invading adjacent organs,

yet with no metastatic potential [8]. Surgical resection is the

mainstay of treatment. However, because the local recur-

rence is high, especially in patients with Gardner’s

syndrome, the use of hormonal therapy (ie, tamoxifen),

nonsteroidal anti-inflammatory drugs, and interferon as well

as systemic chemotherapy may play an important role in the

treatment of MF. Modalities other than surgery are

especially advocated in patients with recurrent disease and

for patients at high risk of surgical intervention (either

because of patient’s comorbid conditions or tumor unresect-

ability) [8]. Radiotherapy plays a minor role for the

treatment of MF.

The first logical step for a tumor that is unresectable is to

start with a nonsteroidal anti-inflammatory drug such as

indomethacin or sulindac. If growth of the tumor continues,

tamoxifen should be added. Systemic chemotherapy with

methotrexate and vinblastine should be implemented in

patients refractory to these initial modalities.

The present case is peculiar in that MF presented in a

young girl without history of FAP. She had no symptoms

despite the tumor’s large size. Previously reported cases of

primary MF had presented also with atypical scenarios. In

one instance, a 52-year-old man without FAP had a 12-kg,

50-cm MF [19]. In another case, a child presented with

severe hematemesis and melena, and had MF with similar

invasion to the stomach as in the present report [20].

Shivaram et al [21] reported a case of a 13-year-old boy

with MF consisting of a small tumor arising from the

mesentery of the small bowel. In the current report, MF

originated from the omentum invading the stomach. Primary

fibromatosis, the age of onset, and the location of the tumor

constitute a unique presentation for this case.

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