S p e c i a l A r t i c l e P i c t o r i a l E s s ay

Shinagare et al.
Imaging of Desmoid Tumors
Special Article
Pictorial Essay

A to Z of Desmoid Tumors
Atul B. Shinagare1
Nikhil H. Ramaiya1
Jyothi P. Jagannathan1
Katherine M. Krajewski1
Angela A. Giardino1
James E. Butrynski2
Chandrajit P. Raut 3
Shinagare AB, Ramaiya NH, Jagannathan JP, et al.

Keywords: complications, deep fibromatosis, desmoid

tumors, extraabdominal desmoids, treatment-related
changes
DOI:10.2214/AJR.11.6657
Received February 10, 2011; accepted after revision
May 3, 2011.
1
Department of Imaging, Dana-Farber Cancer Institute,
and Department of Radiology, Brigham and Womens
Hospital, 450 Brookline Ave, Boston, MA 02215.
Addresscorrespondence to A. B. Shinagare
(ashinagare@partners.org).
2
Department of Medical Oncology, Dana-Farber Cancer
Institute, Harvard Medical School, Boston, MA.
3
Department of Surgery, Brigham and Womens Hospital,
Harvard Medical School, Boston, MA.

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OBJECTIVE. The purpose of this article is to illustrate the common locations of desmoid
tumors (deep fibromatosis), complications of intra- and extraabdominal desmoids, and treatment-related changes in their imaging appearance.
CONCLUSION. Desmoids are locally aggressive fibrous tumors with a tendency to recur. Desmoids can be intraabdominal, in the abdominal wall, or extraabdominal. Complications, such as compression or invasion of adjacent structures, and abscess formation can
occur. Treatment options include observation, local treatment (surgery, radiotherapy), or systemic therapy (conventional chemotherapy, molecular targeted agents).

esmoid tumors, also called deep

or aggressive fibromatosis, are
uncommon mesenchymal neoplasms with a fibrotic bandlike
consistency [1]. These are locally aggressive
tumors without potential for distant metastases. Local recurrence and adjacent organ
involvement are important causes of morbidity and mortality. The incidence of desmoid
tumors in the general population is 24 cases
per million per year, with a slight female preponderance and peak incidence in the third
and fourth decades [2]. These tumors are
more aggressive in younger patients, with recurrence rates up to 87% [3].
Desmoids present as a firm mass, particularly when extraabdominal. The firm consistency can make penetration of tumor difficult
during imaging-guided biopsy [4]. Desmoids
have spiculated infiltrative margins and are
typically adherent to adjacent structures. Microscopically, they consist of poorly defined
fascicles of uniform spindle cells and fibroblasts in dense collagen stroma. Mitotic activity is low, and necrosis is rare [2, 4]. This
article will summarize the features of desmoids that radiologists should be aware of,
specifically the common locations, complications, and treatment-related changes.
Risk Factors
The association of abdominal and extraabdominal desmoids with familial adenomatous polyposis (FAP) (Fig. 1) and Gardner syndrome (Fig. 2) is well established [5].

Trauma, prior surgery, pregnancy (Fig. 3),

and oral contraceptive use are other risk factors [1, 6]. Estrogen has been implicated as a
growth factor for desmoids [1].
Location
Desmoid tumors can be intraabdominal (Figs. 13), within the abdominal wall
(Fig. 4), or extraabdominal (Figs. 510). In
various studies, 2869% of desmoids were
intraabdominal (mesenteric or pelvic) or
abdominal wall, and the remaining were extraabdominal [7]. Desmoids associated with
FAP and Gardner syndrome are typically intraabdominal, usually located in the smallbowel mesentery (Figs. 13). Pregnancy-associated desmoids tend to arise within the
rectus abdominis or internal oblique muscles
[1] (Fig. 4). Extraabdominal desmoids are intimately associated with muscle and fascia,
with the most common locations being the
shoulder and upper extremity (33%) (Figs.
5 and 6), gluteal region and lower extremity (30%) (Figs. 7 and 8), chest wall or back
(17%) (Fig. 9), and head and neck (10%) [1,
2, 4]. Isolated cases of breast desmoids have
been reported [8] (Fig. 10). Multiple desmoid tumors are seen in 15% of cases [1].
Imaging Features
Although use of radiography, bone scintigraphy, and ultrasound for imaging of desmoid
tumors has been described, in practice, the
imaging of desmoids relies mainly on CT and
MRI. On ultrasound, desmoid tumors appear

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as hypoechoic soft tissue masses with variable
vascularity [4] (Fig. 11). CT shows a softtissue mass of variable attenuation and enhancement [4] (Figs. 24 and 10). Margins
may be indistinct because of infiltration of
adjacent structures. Heterogeneous attenuation may be seen because of necrosis or degeneration (Figs. 3 and 4).
MRI, with its excellent soft-tissue contrast, is best suited for optimum evaluation of
extraabdominal desmoids because it allows
accurate depiction of their relationship with
adjacent structures [4]. Desmoids may have
heterogeneous signal and inhomogeneous
enhancement because of variable distribution of spindle cells, collagen, and myxoid
matrix (Figs. 79). On T2-weighted and proton density images, signal intensity is usually
intermediate, between skeletal muscles and
subcutaneous fat (Figs. 59), at times with
the presence of hypointense bands corresponding to collagen bundles [9]. Over time,
they become more hypointense because of
increasing collagen deposition and decreasing cellularity (Fig. 7A), whereas recurrent
or actively growing desmoids tend to have a
higher T2 signal, probably because of higher
cellularity (Fig. 7B) [10]. On 18F-FDG-PET/
CT, the median maximum standardized uptake value of desmoid tumors has been reported to be 4.1 (range, 1.08.1) [11].
The differential diagnosis of desmoid tumors includes other neoplasms, such as lymphoma, pleomorphic sarcoma, fibrosarcoma,
and giant cell tumor of the tendon sheath
[1]. Although the imaging appearance may
be suggestive, histopathologic confirmation
must be obtained in all cases before definitive treatment.
Complications
Complications of desmoid tumors result
from their locally aggressive nature, leading to compression and invasion of adjacent
structures. Intraabdominal desmoids, especially those associated with FAP or Gardner syndrome, are more infiltrative and may
cause intestinal (Fig. 12) or ureteral obstruction or encase the mesenteric vessels [2, 12]
(Fig. 2). Non-FAP-associated desmoids, in
our experience, have smooth margins and
are less likely to be infiltrative (Fig. 13). Pelvic desmoids can infiltrate the urinary bladder (Fig. 14) or may cause hydrosalpinx (Fig.
12). Extraabdominal desmoids may encase
and compress adjacent structures, including
vessels or nerves. Chest wall desmoids can
invade the pleura (Fig. 5). Desmoids rarely

undergo abscess formation (Fig. 12), which

may require surgical intervention or percutaneous drainage. Large desmoid tumors may
undergo mucoid or cystic degeneration.
Treatment Options
A multidisciplinary approach tailored to
the individual patient is required for optimum
management of desmoids. Stable asymptomatic desmoids can be observed [13] (Fig.
15). Treatment is necessary for symptomatic
desmoids, especially those with mass effect
on critical structures. Treatment choice is dictated by anatomic considerations. If feasible,
surgical resection with a wide margin is the
conventional treatment of choice for symptomatic desmoids [2]. However, recurrence is
common (1977%) (Fig. 7) and more frequent
with extraabdominal desmoids (3050%)
than intraabdominal desmoids (1530%) [1,
4]. Often, resection is not possible because
of close association with vital structures, in
which cases, systemic therapy with or without surgical excision should be considered [2]
(Fig. 2). This is more commonly seen with
FAP- or Gardner-associated mesenteric desmoids, which are often unresectable or lead to
significant morbidity, including perioperative
hemorrhage, short-bowel syndrome, intestinal ischemia, obstruction, or fistula formation
[12]. Therefore, conservative management has
been recommended in such cases whenever
possible, using analgesia and minimally invasive therapy as needed [12]. Recently, there
has been an increasing shift toward conservative management of desmoids [12, 13].
Nonsurgical treatment options include radiation and systemic therapy. Radiation has
been reported to be comparable to surgery
and is also useful as an adjuvant treatment to
reduce the risk of local recurrence [14] (Fig.
16). Systemic treatment options include cytotoxic agents, such as anthracyclines (Fig.
2); molecular targeted agents, such as imatinib; interferon; and antiestrogen hormonal
therapy, such as tamoxifen [2, 11, 7]. Among
systemic agents, a higher radiologic response
rate has been reported with anthracyclines
and antiestrogen treatment [7]. In some cases, after treatment, the size of the desmoid
tumors may remain unchanged, but there
may be a decrease in signal intensity and enhancement of the desmoids (Fig. 8).
Role of Imaging in Desmoid Tumors
Initial imaging in desmoids is usually performed to determine the extent, local mass
effects, and potential resectability. Desmoids

being observed or managed nonoperatively should undergo periodic imaging assessment, especially if intraabdominal. MRI
may have prognostic value because higher
T2 signal is reportedly associated with more
rapid growth [15]. Given the potential for recurrence, regular clinical and possible imaging follow-up have been recommended after
therapy, initially every 36 months [12, 16].
Patients undergoing chemoradiation undergo
6- to 12-week imaging follow-up. Desmoids
being observed are initially followed-up with
imaging at a 3- to 6-month interval. The imaging interval is then adjusted depending on
the rate of growth and presence of symptoms.
At our institution, MRI is usually preferred
for pre- and postoperative evaluation of patients undergoing surgery, and whenever possible, we prefer the use of gadolinium for better delineation of the extent of desmoids.
Conclusion
Desmoid tumors are a challenging clinical condition with locally aggressive behavior and a strong tendency for recurrence.
Although the imaging appearance may be
suggestive, histopathologic confirmation is
necessary before treatment. Management
options include observation, surgical resection, radiotherapy, conventional chemotherapy, hormonal agents, and newer molecular
targeted agents. A multidisciplinary approach tailored to the individual patient is
usually needed, depending on the location,
local effects, and clinical course.
References
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Fig. 131-year-old woman with familial

adenomatous polyposis syndrome. T2-weighted
MR image in coronal plane shows large mesenteric
mass (arrow) that was proven to be desmoid tumor on
histopathology.

etal Radiol 1990; 19:495499

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f_guidelines.asp. Accessed November 26, 2010

Fig. 253-year-old man with Gardner syndrome and large mesenteric desmoid.
A, Coronal contrast-enhanced CT image shows large homogeneous mesenteric desmoid tumor (thick arrow).
Note infiltrative margins of mass (curved arrow). Desmoid is intimately associated with mesenteric artery
(thin arrow). On axial images (not shown), mesenteric vessels could be seen branching within mass. Close
association with mesenteric vessels made surgical resection difficult. Hence, patient was treated with
doxorubicin.
B, Axial contrast-enhanced CT image after 3 months of treatment with doxorubicin shows marked decrease in
size of desmoid (thick arrow). Mesenteric vessels can be seen separate from mass (thin arrow). Desmoid was
then successfully resected.

Fig. 327-year-old woman with pregnancyassociated mesenteric desmoid. Mass, which

presented during second trimester of pregnancy,
was initially thought to be of ovarian origin. During
exploration at time of cesarean delivery, mass was
found to be of mesenteric origin and unresectable
because it was adherent to superior mesenteric
vessels. It was biopsied, and histopathology showed
it to be desmoid. Axial image from contrast-enhanced
CT performed postpartum shows large mesenteric
mass (arrowheads) that is intimately associated with
mesenteric vessels (arrow).

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Imaging of Desmoid Tumors

Fig. 429-year-old woman with Gardner syndrome
with abdominal wall and mesenteric desmoids. Axial
contrast-enhanced CT image shows large somewhat
low-attenuation desmoid arising within right
rectus muscle (straight arrow) and another smaller
mesenteric desmoid (arrowhead). Ill-defined soft
tissue is also noted surrounding mesenteric vessels
(curved arrow).

Fig. 528-year-old woman with large mass in left shoulder region.

A, T1-weighted MR image in coronal plane shows large homogeneous mass in left shoulder region (arrows) that is isointense to skeletal muscle. This was proven to be
desmoid on histopathology.
B, T2-weighted fat-suppressed MR image in coronal plane shows intermediate signal of desmoid (straight arrows). Note nodular protrusion from mass medially toward
pleura (curved arrow), raising concern for pleural invasion.
C, T1-weighted fat-suppressed gadolinium-enhanced MR image in axial plane shows homogeneously enhancing mass (straight arrows) and better delineates pleural
invasion (curved arrow).

Fig. 643-year-old woman with desmoid tumor of

left arm.
A, Proton density MR image in axial plane shows
mass involving left deltoid muscle (straight arrows).
Note that signal intensity of mass is between low
signal of preserved deltoid muscle (curved arrow) and
high signal of subcutaneous fat (black dot).
B, T1-weighted fat-suppressed gadolinium-enhanced
MR image in coronal plane shows moderate
homogeneous enhancement of mass (straight
arrows). Note subcutaneous stranding, and overlying
skin thickening and retraction (curved arrow), which
may represent reactive changes or extension of
desmoid tumor.

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Shinagare et al.
Fig. 746-year-old man with left gluteal desmoid
tumor.
A, T2-weighted MR image in axial plane shows mass
deep in relation to gluteus maximus muscle (thick
arrows) that is iso- to hypointense to muscle. Note
hypointense areas within mass (thin arrow). Patient
underwent resection of this desmoid.
B, MRI was performed 4 years after surgical
resection. Proton density image in axial plane shows
heterogeneous recurrent mass with predominantly
intermediate signal intensity (arrows). Note atrophy
of overlying gluteus maximus muscle (arrowhead).

Fig. 827-year-old woman with thigh desmoid.

A, T1-weighted fat-suppressed gadolinium-enhanced
MR image in coronal plane shows heterogeneous
mass in left thigh (arrowheads) with heterogeneous
enhancement in its superior portion.
B, Coronal T1-weighted fat-suppressed gadoliniumenhanced image from follow-up MRI performed after
9 months of doxorubicin treatment shows minimal
decrease in size of desmoid (arrowheads); however,
moderate decrease in degree of enhancement
is noted, indicating response to treatment. Also
note decreased signal intensity of desmoid, which
indicates treatment response as well. This patient
also had calf desmoid (not shown) that showed
similar posttreatment changes.

Fig. 937-year-old man with posterior chest wall

desmoid.
A, Axial proton density MR image shows infiltrative
heterogeneous mass in posterior chest wall that has
superficial moderate signal component (arrow) and
deeper linear low-signal component (arrowhead).
B, Axial T1-weighted fat-suppressed gadoliniumenhanced MR image shows heterogeneous
enhancement. Superficial component that had
moderate T2-signal intensity shows higher
enhancement (arrow), and deeper T2-hypointense
component shows less enhancement (arrowhead).

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B
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Imaging of Desmoid Tumors

Fig. 1045-year-old woman with resected right
breast cancer who has breast desmoid. Note bilateral
breast implants (arrowheads).
A, Contrast-enhanced CT image in coronal plane
shows low-attenuation mass in right breast (arrow),
causing mass effect on inferior surface of right breast
implant. Note tail of mass insinuating between
costal cartilages (curved arrow).
B, Coronal T1-weighted fat-suppressed gadoliniumenhanced subtracted MR image shows enhancing
mass (arrow). Breast implant is again noted
(arrowhead).

B
Fig. 1134-year-old woman with desmoid tumor of
right rectus sheath. Ultrasound image in transverse
plane shows heterogeneous hypoechoic mass
(arrowheads) showing low-grade vascularity on color
Doppler imaging (square).

Fig. 1226-year-old woman with Gardner syndrome and intraabdominal desmoid tumor, with history of
multiple episodes of acute abdominal pain and vomiting.
A, Coronal image from contrast-enhanced CT obtained during episode of acute abdominal pain and vomiting
shows mildly enhancing low-attenuation mass in left hemiabdomen (arrow), causing intestinal obstruction.
Note multiple dilated small-bowel loops (arrowheads).
B, Patient later presented with fever and abdominal pain. Axial contrast-enhanced CT image shows large pelvic
desmoid with central low-attenuation necrotic area (straight arrow). Also note tiny air specks within desmoid
(curved arrow) suggestive of abscess formation, which was later confirmed at surgery where desmoid and few
adherent bowel loops were resected. Note mildly dilated small-bowel loop (arrowhead).
C, Axial contrast-enhanced CT image through pelvis shows elongated low-attenuation structure with
incomplete septation seen in left hemipelvis (arrows), which was proven to be hydrosalpinx caused by pelvic
desmoid. Desmoid tumor is not visible on this image.

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Shinagare et al.

Fig. 1355-year-old man with nonfamilial

adenomatous polyposisassociated mesenteric
desmoid tumor. Axial contrast-enhanced CT
image shows large homogeneous low-attenuation
mesenteric desmoid (arrow). Note well-defined
margins of mass. Compare with infiltrative margin of
Gardner syndromeassociated desmoid in Figure 9.

Fig. 1553-year-old woman with small mesenteric

desmoid tumor.
A, Axial contrast-enhanced CT image shows small
mesenteric mass (straight arrow) closely associated
with mesenteric vessels (curved arrow). In view of
small asymptomatic desmoid and its proximity to
mesenteric vessels, this was observed without any
treatment.
B, Axial image from follow-up contrast-enhanced CT
performed after 2 years shows no significant change
in size of mesenteric desmoid (arrow).

Fig. 1657-year-old woman with recurrent desmoid

in right lung apex after initial surgical resection.
A, Coronal contrast-enhanced CT image shows
soft-tissue mass in right lung apex (arrow). Note
postoperative changes in right upper chest wall
(arrowheads).
B, Patient received radiation and cycles of
doxorubicin. Coronal image from follow-up contrastenhanced CT shows markedly decreased size of
desmoid (straight arrow), and postradiation changes
in right lung apex (curved arrow).

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Fig. 1444-year-old man with familial adenomatous

polyposisassociated desmoid tumor. Coronal
contrast-enhanced CT image shows infiltrative
mass in pelvis with invasion of urinary bladder wall
(arrows) and involvement of multiple small-bowel
loops (arrowheads). Patient underwent cystectomy
because of severe urinary complaints.

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