Beruflich Dokumente
Kultur Dokumente
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.
WEB
This is a Web exclusive article.
AJR 2011; 197:W1008W1014
0361803X/11/1976W1008
American Roentgen Ray Society
W1008
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).
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
1. Dinauer PA, Brixey CJ, Moncur JT, FanburgSmith JC, Murphey MD. Pathologic and MR imaging features of benign fibrous soft-tissue tumors
in adults. RadioGraphics 2007; 27:173187
2. Shields CJ, Winter DC, Kirwan WO, Redmond
HP. Desmoid tumours. Eur J Surg Oncol 2001;
27:701706
3. Romero JA, Kim EE, Kim CG, Chung WK, Isiklar
I. Different biologic features of desmoid tumors in
adult and juvenile patients: MR demonstration. J
Comput Assist Tomogr 1995; 19:782787
4. Murphey MD, Ruble CM, Tyszko SM, Zbojniewicz
AM, Potter BK, Miettinen M. From the archives of
the AFIP: musculoskeletal fibromatosesradiologic-pathologic correlation. RadioGraphics 2009;
29:21432173
5. Lindor NM, Greene MH. The concise handbook of
family cancer syndromes: Mayo Familial Cancer
Program. J. Natl. Cancer Inst. 1998; 90:10391071
6. Hyry P, Reitamo JJ, Ttterman S, Hopfner-Hallikainen D, Sivula A. The desmoid tumor. II.
Shinagare et al.
Analysis of factors possibly contributing to the
etiology and growth behavior. Am J Clin Pathol
1982; 77:674680
7. de Camargo VP, Keohan ML, DAdamo DR, et al.
Clinical outcomes of systemic therapy for patients
with deep fibromatosis (desmoid tumor). Cancer
2010; 116:22582265
8. Okamoto K, Kurihara Y, Imamura K, et al. Desmoid tumor of the breast: the role of proton magnetic resonance spectroscopy for a benign breast
lesion mimicking a malignancy. Breast J 2008;
14:376378
9. Kransdorf MJ, Jelinek JS, Moser RP, et al. Magnetic resonance appearance of fibromatosis: a report of 14 cases and review of the literature. Skel-
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.
W1010
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).
W1012
B
AJR:197, December 2011
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.
Shinagare et al.
W1014