Mesenteric fibromatosis in a young girl without familial adenomatous polyposis
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Transcript of Mesenteric fibromatosis in a young girl without familial adenomatous polyposis
www.elsevier.com/locate/jpedsurg
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: [email protected] (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.
References
[1] Shields CJ, Winter DC, Kirwan WO, et al. Desmoid tumours. Eur J
Surg Oncol 2001;27:701-6.
[2] David SS, Khanduri P. A case of primary mesenteric fibromatosis.
ANZ J Surg 1992;62:813-4.
[3] Remmele W, Muller-Lobeck H, Paulus W. Primary mesenteritis,
mesenteric fibrosis and mesenteric fibromatosis. Report of four cases,
pathology, and classification. Pathol Res Pract 1988;184:77-85.
[4] Tsamandas AC, Tzanakakis GN, Karatzas T, et al. Mesenteric
fibromatosis. Br J Clin Pract 1994;48:79 -81.
[5] Yamaguchi K, Hirakata R, Maeda S, et al. Spontaneous isolated intra-
abdominal mesenteric fibromatosis. Case report. Eur J Surg 1991;157:
293 -6.
[6] McAdam WA, Goligher JC. The occurrence of desmoids in patients
with familial polyposis coli. Br J Surg 1970;57:618 -31.
[7] Reitamo JJ, Scheinin TM, Hayry P. The desmoid syndrome. New
aspects in the cause, pathogenesis and treatment of the desmoid tumor.
Am J Surg 1986;151:230 -7.
[8] Janinis J, Patriki M, Vini L, et al. The pharmacological treatment of
aggressive fibromatosis: a systematic review. Ann Oncol 2003;14:
181 -90.
[9] Burke AP, Sobin LH, Shekitka KM, et al. Intra-abdominal fibroma-
tosis. a pathologic analysis of 130 tumors with comparison of clinical
subgroups. Am J Surg Pathol 1990;14:335-41.
[10] Kyle SM, Keenan RA. Mesenteric fibromatosis preventing restorative
proctectomy. ANZ J Surg 1992;62:240 -1.
[11] Suehiro S, Inai K. Mesenteric fibromatosis in familial polyposis. A
case report and a review of Japanese cases. Acta Pathol Jpn 1987;37:
1837-43.
[12] Gardner EJ. A genetic and clinical study of intestinal polyposis, a
predisposing factor for carcinoma of the colon and rectum. Am J Hum
Genet 1951;3:167-76.
[13] Gardner EJ. Follow-up study of a family group exhibiting dominant
inheritance for a syndrome including intestinal polyps, osteomas,
fibromas and epidermal cysts. Am J Hum Genet 1962;14:376-90.
[14] Al Salem AH, Al Hayek R, Qureshi SS. Solitary intestinal
fibromatosis: a rare cause of intestinal perforation in neonates. Pediatr
Surg Int 1997;12:437-40.
[15] Turken A, Senocak ME, Kotiloglu E, et al. Solitary intestinal
fibromatosis mimicking malabsorption syndromes. J Pediatr Surg
1995;30:1387-9.
[16] Al Jadaan SA, Al Rabeeah A. Mesenteric fibromatosis: case report
and literature review. J Pediatr Surg 1999;34:1130-2.
S. Huerta et al.E36
[17] Mecrow IK, Miller V, Lendon M, et al. Mesenteric fibromatosis
presenting with ascites in childhood. J Pediatr Gastroenterol Nutr
1990;11:118 -22.
[18] Yannopoulos K, Stout AP. Primary solid tumors of the mesentery.
Cancer 1963;16:914 -27.
[19] Cianchi F, Perigli G, Pucciani F, et al. Giant mesenteric fibromatosis: a
case report. Ann Ital Chir 1995;66:531 -5.
[20] Sarihan H, Abes M, Yildiz K, et al. Mesenteric fibromatosis. Eur J
Pediatr Surg 1998;8:107-10.
[21] Shivaram A, Dalzell AM, Kokai GK. Clinical quiz: unusual ileal
pathology. Mesenteric fibromatosis. J Pediatr Gastroenterol Nutr
2003;37:251,286.