DOI:10.2214/AJR.07.2334
AJR 2007; 189:633-640
© American Roentgen Ray Society
MRI and Biologic Behavior of Desmoid Tumors in Children
M. Beth McCarville1,2,
Fredric A. Hoffer1,2,
C. Scott Adelman1,
Joseph D. Khoury3,
Chenghong Li4 and
Stephen X. Skapek5,6
1 Division of Diagnostic Imaging, Department of Radiological Sciences, St. Jude
Children's Research Hospital, 332 N Lauderdale St., Memphis, TN
38105-2794.
2 Department of Radiology, University of Tennessee, College of Medicine,
Memphis, TN.
3 Department of Pathology, St. Jude Children's Research Hospital, Memphis,
TN.
4 Department of Biostatistics, St. Jude Children's Research Hospital, Memphis,
TN.
5 Department of Hematology–Oncology, University of Tennessee, College of
Medicine, Memphis, TN.
6 Department of Oncology, St. Jude Children's Research Hospital, Memphis,
TN.
Received December 30, 2006;
accepted after revision March 30, 2007.
Supported in part by the American, Lebanese, and Syrian Associated
Charities (ALSAC) and Grant 5 R25 CA023944 from the National Cancer
Institute.
Address correspondence to M. B. McCarville
(beth.mccarville{at}stjude.org).
Abstract
OBJECTIVE. The outcome of desmoid tumor in children cannot be
reliably predicted on the basis of histologic findings. We sought to determine
whether the postoperative presence of residual or recurrent tumor can be
predicted on the basis of demographic variables and baseline MRI features of
the tumor. We also aimed to determine how imaging features change during
adjuvant treatment and how the imaging features relate to the histologic
features.
MATERIALS AND METHODS. Two radiologists retrospectively reviewed
images from 281 MRI examinations performed at baseline and during
postoperative therapy for desmoid tumor. The examinations had been performed
on 17 children treated between September 1991 and March 2003. Tumor volume;
distinctness of margins; involvement of bone and neurovascular bundle; and
T1-weighted, T2-weighted, and STIR signal intensity and contrast enhancement
pattern were recorded. Baseline imaging and demographic features were
correlated with the postoperative presence of residual or recurrent tumor.
Imaging changes during follow-up were compared with treatment response and
outcome. The imaging features of eight tumors were compared with percentage
cellularity and collagen deposition in biopsy samples obtained within 30 days
of imaging.
RESULTS. Baseline involvement of the neurovascular bundle approached
significance as a predictor of the presence of residual or recurrent tumor
(p = 0.08). Other baseline imaging and demographic features were not
predictive (p 
0.4). Changes in imaging features were variable
during follow-up. T2-weighted and STIR signal intensity may be correlated with
percentage cellularity and collagen deposition.
CONCLUSION. MRI has limited value in prediction of the postoperative
presence of residual or recurrent desmoid tumor in children. It is useful,
however, for detecting disease and monitoring postoperative adjuvant
therapy.
Keywords: aggressive fibromatosis • children • desmoid tumor • MRI
Introduction
Fibromatosis of childhood is a broad spectrum of fibrotic neoplasms that
include congenital fibromatosis, congenital fibrosarcoma, fibromatosis coli,
and desmoid tumor (also known as aggressive fibromatosis or desmoid-type
fibromatosis). The most common of these conditions is desmoid tumor, a
monoclonal fibroblastic proliferation arising in musculoaponeurotic tissues.
Desmoid tumor is classified by the World Health Organization as a tumor of
intermediate grade. Although desmoid tumor does not metastasize and associated
mortality is rare, loss of muscle and joint function is often associated with
tumors that develop in the extremities. The optimal treatment is
controversial; a multidisciplinary approach including surgery, chemotherapy,
and radiation therapy is often used. The goal of treatment is to provide local
control while preserving function and appearance. Surgery historically has
been the primary treatment. However, achieving the wide margin that offers the
best chance of avoiding recurrence is difficult because of the infiltrative
nature of desmoid tumors, and local recurrence after surgical excision is
common [1,
2]. Postoperative adjuvant
therapies have included radiation therapy and cytotoxic and noncytotoxic
chemotherapy (e.g., nonsteroidal antiinflammatory drugs and estrogen
antagonists), but the appropriate use of these treatments in the pediatric
population needs to be defined. Of particular concern in children are the
long-term effects of such treatments in the management of a relatively benign
disease [2,
3]. Because the disease is
rather rare, few reports have described the imaging features, treatment, and
outcome among pediatric patients
[2,
4]. Therefore, we reviewed our
experience with desmoid tumors in children to determine whether demographic
variables and MRI findings are associated with tumor recurrence, histologic
features, treatment response, and outcome.
Materials and Methods
Imaging, Medical Record, and Pathologic Review
Between September 1991 and March 2003, 17 children were referred to our
institution for evaluation of desmoid tumor, seven for recurrent or residual
disease after treatment at a local institution. After obtaining institutional
review board approval, we reviewed the MR images and medical records. Our
review was conducted in compliance with the Health Information Portability and
Accountability Act of 1996. Joint review of baseline (before intervention) and
follow-up (after intervention) images was performed by two pediatric
radiologists who were aware of the diagnosis of desmoid tumor but did not know
the patient's age, treatment history, or disease outcome. For each imaging
examination, the reviewers recorded the number and anatomic location of the
tumors and the volume of each tumor using the following formula:
(anteroposterior diameter x transverse diameter x length) x
0.52.
Baseline tumor images were assessed for local tumor infiltration by
determination of the percentage of tumor margin that was indistinct (not
sharply defined from surrounding soft tissue) on the sequence that best showed
the tumor margins. Baseline images also were assessed for involvement of the
neurovascular bundle and bone, defined as abutting if tumor touched the
structure, encasing if tumor surrounded any part of it, and direct invasion if
tumor invaded it. Baseline T1-weighted images were assessed to determine the
predominant ( 50%) tumor signal intensity, defined as isointense,
hypointense, or hyperintense relative to adjacent normal muscle.
Because previous reports [5,
6] have suggested that areas of
desmoid tumor that are hyperintense on T2-weighted and STIR images are
associated with active fibroblastic proliferation, baseline and follow-up
images were reviewed for subjective determination of the percentage of the
entire tumor volume that was hyperintense to normal muscle on these sequences
and the predominant signal intensity. If fat had higher signal intensity than
muscle on these sequences, the images were considered inadequately fat
suppressed, and they were excluded from analysis. The sequence that gave the
higher predominant signal intensity was used for analysis when there was a
discrepancy. Tumors lacking a predominant signal intensity were considered of
mixed intensity. Baseline and follow-up images also were subjectively assessed
for percentage of the entire tumor volume that was contrast enhanced and
overall enhancement intensity, which was graded as mild (equal in signal
intensity to muscle), moderate (higher signal intensity than muscle but lower
signal intensity than blood vessels), or intense (same signal intensity as or
higher signal intensity than blood vessels).
For tumors on which follow-up was conducted until the end of therapy,
imaging changes that occurred between the imaging date closest to initiation
of treatment and either immediately before a change in treatment technique
(radiation therapy vs chemotherapy vs surgery) or the most recent follow-up
evaluation were reported. Using the criteria established for the Pediatric
Oncology Group 9650 and Children's Oncology Group ARST0321 desmoid tumor
studies, we assessed tumor response on follow-up images as the product of the
largest bidirectional tumor diameters as follows: progressive disease if the
product increased more than 25% above the smallest product of bidimensional
diameters recorded; stable disease if the product showed a 25% or smaller
increase or decrease; partial response if the product decreased more than 50%;
and minor response if the product decreased between 25% and 50%. Tumors were
considered completely resolved when no tumor was identified on MR images.
From medical records we recorded patient age at diagnosis, sex, medical
history, operative and pathologic reports, clinical course, and outcome of
desmoid tumor treatment. Because desmoid tumor can be associated with familial
adenomatous polyposis, we also recorded family history of familial adenomatous
polyposis. Pathologic specimens obtained within 30 days of MRI were reviewed
by one pathologist, who graded the amount of collagen deposition and the
percentage cellularity of each specimen. Collagen deposition was graded as
follows: 1, rare bands; 2, readily notable collagen; 3, abundant collagen.
Percentage cellularity was based on the average cellularity seen in 10 random
high-power fields.
Statistical Analysis
For statistical analysis, residual and recurrent tumor after surgical
resection was considered a single outcome, and patients were categorized as
having or not having residual or recurrent tumor. We used the Wilcoxon and
Mann-Whitney tests to compare age, baseline percentage tumor enhancement,
baseline tumor enhancement intensity, baseline tumor percentage indistinct
margin, and baseline tumor volume of patients with recurrent or residual
tumors and those without. We analyzed the association between sex, baseline
T1-weighted signal intensity, baseline tumor involvement of the neurovascular
bundle or bone, and residual or recurrent tumor by using the Fisher's exact
test. For the purposes of analysis, baseline tumor involvement of the
neurovascular bundle and bone was a dichotomous variable (involvement vs no
involvement). Involvement was defined as abutting, surrounding, or invading.
Tumor enhancement intensity was considered a dichotomous variable: intense
versus moderate or mild. No statistical test was performed for baseline tumor
T2-weighted or STIR signal intensity because all tumors were predominantly
hyperintense on these sequences. The analyses were performed with SAS v9.1
(SAS Institute) and StatXact PROCs v6.2 (Cytel) software.
Results
Demographic Features and Medical History
The study group comprised seven girls and 10 boys with a mean age at
diagnosis of 7 years 10 months (range, 2 months to 19 years 10 months). We
found no correlation between age at diagnosis (p = 0.7) or sex
(p = 0.6) and disease recurrence
(Table 1). One patient had
familial adenomatous polyposis (Gardner syndrome) (Figs.
1A,
1B, and
1C).
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TABLE 1: Correlation Between Baseline Tumor Demographic and MRI Variables and
Presence of Residual or Recurrent Desmoid Tumor After Surgical
Resection
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Fig. 1A —19-year-old male with Gardner syndrome and massive desmoid
tumor of paraspinal musculature. Baseline sagittal T2-weighted MR image
(TR/TE, 5,950/116) shows hypointense bands (arrows) coursing through
hyperintense tumor.
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Fig. 1B —19-year-old male with Gardner syndrome and massive desmoid
tumor of paraspinal musculature. Sagittal unenhanced T1-weighted MR image
(618/14, B) and contrast-enhanced T1-weighted image (804/14, C)
show lack of enhancement of hypointense bands (arrows) in A.
These nonenhancing, hypointense bands are probably areas of fibrosis within
enhancing active fibroblasts. Patients with Gardner syndrome or familial
adenomatous polyposis have 20% lifetime risk of extraabdominal or, more
commonly, intraabdominal desmoid tumor.
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Fig. 1C —19-year-old male with Gardner syndrome and massive desmoid
tumor of paraspinal musculature. Sagittal unenhanced T1-weighted MR image
(618/14, B) and contrast-enhanced T1-weighted image (804/14, C)
show lack of enhancement of hypointense bands (arrows) in A.
These nonenhancing, hypointense bands are probably areas of fibrosis within
enhancing active fibroblasts. Patients with Gardner syndrome or familial
adenomatous polyposis have 20% lifetime risk of extraabdominal or, more
commonly, intraabdominal desmoid tumor.
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Management and Outcome
One patient was lost to follow-up. The follow-up times of the other 16
patients ranged from 6 months to 9 years 7 months with an average follow-up
time of 4 years 5 months. Sixteen of the 17 patients received treatment with
up-front surgical resection. Four of these patients had no residual or
recurrent tumor after surgery, six had residual tumor, and six had recurrent
tumor in or near the surgical bed. One patient had an unresectable chest wall
tumor, which was treated with up-front radiation therapy and then subtotal
surgical resection and chemotherapy. This patient had a partial response. For
the 12 patients with residual or recurrent disease, treatment varied
considerably. Five received treatment with repeated resection alone. The
tumors of two patients were grossly resected. One of these tumors recurred
multiple times (Figs. 2A and
2B), and the affected finger
was amputated to achieve final control. The other patient was lost to
followup. Tumors of the other three patients were subtotally resected; two
residual tumors remained stable, and one progressed, but the patient did not
receive additional treatment. The other seven patients with residual or
recurrent tumors received combinations of chemotherapy (n = 5),
surgery (n = 4), and radiation therapy (n = 4). One of these
seven patients had a complete response; two, a minor response; and two, a
partial response. Two patients had progressive disease. Therefore, after
surgery alone (n = 10) or combined treatment (n = 7), five
patients had a complete response; two, a minor response; three, a partial
response; two, stable disease; four, progressive disease; and one was lost to
follow-up.
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Fig. 2A —10-month-old girl with desmoid tumor of middle finger.
T2-weighted coronal image (TR/TE, 3,000/30) shows predominantly hyperintense
mass (arrows) with fairly sharp margins, except for proximal and
distal margins.
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Fig. 2B —10-month-old girl with desmoid tumor of middle finger. T1
contrast-enhanced coronal image (400/20) shows peripheral tumor enhancement
and central lack of enhancement (arrows). Although small, this tumor
necessitated amputation of digit for local control.
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Imaging Findings
The 17 patients underwent a total of 281 MRI examinations (mean, 17
examinations per patient; range, one to 22). Baseline images of the original
tumor, before any intervention, were available for 10 patients. T1-weighted
images were available for all 10 patients, T2-weighted or STIR images for five
patients, and contrast-enhanced images for eight patients. Imaging features
and outcome after surgical resection of these 10 baseline tumors are
summarized in Table 2. Three of
the five baseline tumors imaged with T2-weighted or STIR sequences had
predominantly high signal intensity with hypointense areas and bands
interspersed throughout the tumor. These areas and bands did not become
enhanced after contrast administration (Figs.
1A,
1B,
1C,
2A, and
2B). Such areas were found in
many of the recurrent tumors. The correlation between baseline tumor imaging
features and outcome is shown in Table
1. Involvement of the neurovascular bundle approached significance
as a predictor of the presence of residual or recurrent tumor (p =
0.08). In four patients, baseline tumors involved both the neurovascular
bundle and bone, and all four patients had residual (n = 3) or
recurrent tumor (n = 1) after surgical resection. None of the other
imaging parameters was a significant predictor of the presence of recurrent or
residual disease.
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TABLE 2: Baseline Tumor MRI Features and Assessment of Presence of Residual or
Recurrent Tumor After Surgical Resection (n = 10)
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Posttreatment MR images were available for 16 patients. Four patients were
treated with radiation, one for presumed residual tumor after total gross
resection. This patient did not have convincing imaging evidence of residual
tumor on follow-up MRI examinations. The changes in tumor imaging features
that occurred during radiation therapy in the other three patients are shown
in Table 3. One of these tumors
increased in size and two became smaller. The percentage of tumor volume that
was hyperintense on T2-weighted or STIR images decreased in all three tumors
(Figs. 3A and
3B). In three patients new
desmoid tumors developed within or near the radiation field. All of the
patients were clinically considered to have progressive disease, and they
underwent additional surgical resection or chemotherapy. Six patients (eight
desmoid tumors) underwent follow-up until the end of chemotherapy. The
evolution of MRI features during chemotherapy was variable from person to
person and is summarized in Table
3. Follow-up MRI showed one tumor completely resolved.
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Fig. 3A —14-year-old girl with recurrent desmoid tumor in left foot.
Sagittal STIR image (TR/TE, 2,500/18; flip angle, 140°) 2 weeks after
completion of radiation therapy shows tumor (arrows) predominantly
hyperintense to muscle. Some of hyperintense signal may be result of edema
from recent radiation.
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Fig. 3B —14-year-old girl with recurrent desmoid tumor in left foot.
Sagittal STIR image (3,500/18; flip angle, 140°) 2 years 7 months after
A shows tumor (arrows) has grown slightly but is predominantly
hypointense to muscle.
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Histology and Associated MRI Findings
Of 44 resections or biopsy procedures, eight were conducted within 30 days
of MRI. Histologic and corresponding MRI findings for these eight are shown in
Table 4. The relation between
percentage cellularity and collagen deposition was inverse. Tumors with higher
percentage cellularity had less collagen deposition than did those with lower
percentage cellularity. The tumor with the least cellularity (5%) and abundant
collagen had the least percentage volume enhancement (30%) (Figs.
4A,
4B,
4C, and
4D). The tumor with the
greatest percentage cellularity (50%) and rare bands of collagen had
enhancement of 90% of the tumor volume (Figs.
5A,
5B,
5C, and
5D).
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Fig. 4B —2-year-old girl with desmoid tumor in right lateral aspect of
chest. Unenhanced T1-weighted axial image (25/9, B) and
contrast-enhanced T1-weighted axial image (765/14, C) show no
enhancement of tumor (arrows) at this level.
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Fig. 4C —2-year-old girl with desmoid tumor in right lateral aspect of
chest. Unenhanced T1-weighted axial image (25/9, B) and
contrast-enhanced T1-weighted axial image (765/14, C) show no
enhancement of tumor (arrows) at this level.
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Fig. 4D —2-year-old girl with desmoid tumor in right lateral aspect of
chest. Photomicrograph of biopsy specimen obtained 5 days after
A–C reveals sparse cells (5%) separated by prominent collagen
bundles. Entrapped peripheral nerve (arrows) is evident.
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Fig. 5A —14-year-old girl with desmoid tumor in left ankle. Findings
contrast to MRI features in Figures
4A,
4B,
4C, and
4D. STIR coronal image (TR/TE,
3,500/18; flip angle, 90°) shows tumor has uniformly high signal intensity
(arrows) relative to muscle.
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Fig. 5B —14-year-old girl with desmoid tumor in left ankle. Findings
contrast to MRI features in Figures
4A,
4B,
4C, and
4D. Unenhanced T1-weighted
axial image (650/15, B) and contrast-enhanced T1-weighted axial image
(710/15, C) show uniform moderate tumor enhancement
(arrows).
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Fig. 5C —14-year-old girl with desmoid tumor in left ankle. Findings
contrast to MRI features in Figures
4A,
4B,
4C, and
4D. Unenhanced T1-weighted
axial image (650/15, B) and contrast-enhanced T1-weighted axial image
(710/15, C) show uniform moderate tumor enhancement
(arrows).
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Fig. 5D —14-year-old girl with desmoid tumor in left ankle. Findings
contrast to MRI features in Figures
4A,
4B,
4C, and
4D. Photomicrograph of biopsy
specimen obtained 2 weeks after A–C shows 50% cellularity with
rare collagen bands.
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Discussion
Desmoid tumor, the most common type of fibromatosis in children, is
characterized by intertwining fibroblasts and myofibroblasts arranged in broad
bundles that infiltrate surrounding structures and are usually surrounded by
abundant collagen. Although benign, desmoid tumors have variable biologic
potential, which cannot be predicted on the basis of histologic features, and
tend to recur after local excision. The nomenclature used to describe these
tumors in children is confusing. Terms such as infantile and juvenile
fibromatosis imply distinctive biologic properties in different age groups
[7–11].
Such distinctive features are not evident in the published literature, and
although our cohort was small, we found no difference in the clinical behavior
or MRI features of these tumors among patients of different ages. In our
study, nine of 10 primary tumors were isointense in relation to muscle on
T1-weighted images, whereas T2-weighted and STIR images showed predominantly
high signal intensity. Contrast enhancement was intense in most of the primary
tumors. Most of the tumors had enhancement of 80% or more of the tumor volume.
Tumor margins ranged from 100% sharply defined to 40% infiltrative. None of
these MRI characteristics was predictive of the presence of residual or
recurrent disease.
Thirteen (77%) of our patients had residual or recurrent tumor after
up-front surgical resection. We found that patients with tumors involving the
adjacent neurovascular bundle or both the neurovascular bundle and bone were
more likely than others to have residual or recurrent tumor after surgical
resection. This finding is not surprising because tumors involving these
structures are difficult to resect completely without morbidity. Our finding
is consistent with the results of a 1995 retrospective study
[12] of pediatric patients
with desmoid tumors that showed surgical resection with negative margins was
associated with a 70% recurrence-free survival rate. Therefore, we favor
surgical treatment of children with desmoid tumor if the operation is likely
to result in complete resection and if it can be performed without undue
morbidity, such as amputation. In our cohort, patients with residual or
recurrent disease who were treated with chemotherapy fared better than those
treated with radiation therapy. One of six patients treated with chemotherapy
had progressive disease, compared with three of four patients treated with
radiation. An interesting finding was that additional tumors developed within
or near the radiation field of three of the four patients treated with
radiation therapy, although a causal relation was not established because of
the small sample size.
Although our sample size was small, histologic results showed that
percentage tumor cellularity was inversely related to collagen deposition.
Tumors with greater percentage tumor cellularity contained more hyperintense
material on T2-weighted and STIR images than did the tumor that was only 5%
cellular with abundant collagen. This result agrees with those of other
investigators [5,
6] who reported a similar
correlation between T2-weighted signal intensity and tumor cellularity and a
natural evolution of desmoid tumor toward shrinkage with a decrease in
T2-weighted signal intensity. However, the small size of biopsy specimens
makes correlation with MRI findings prone to error because small sample
volumes may not reflect the predominant tumor composition. This problem may
explain our finding of moderate and intense contrast enhancement among tumors
that contained mixed amounts of cellularity in abundant collagen within biopsy
samples.
We found that the evolution of imaging features of tumors on which
follow-up was conducted until the end of adjuvant therapy was quite complex. A
surprising finding was that several tumors that grew became less intense on
T2-weighted and STIR images and enhanced less than tumors that became smaller.
This finding runs counter to the prediction that tumors containing abundant
collagen would be less metabolically active than those with less collagen and
higher percentage cellularity. Perhaps increased collagen deposition is
partially responsible for tumor enlargement in addition to growth that results
from the proliferative activity of fibroblasts. We need to confirm our
findings in a larger cohort and to more accurately assess tumor metabolic
activity using other imaging techniques.
The main limitations of our study were the retrospective nature and
relatively small cohort size. Patients in our study were treated with a
variety of chemotherapeutic agents, radiation therapy, and surgery, and
imaging time points were not prospectively determined or protocol driven. This
limitation made it difficult to assess the evolution of MRI features in
response to the therapies and precluded meaningful statistical analysis of the
correlation between them. Another study limitation was the lack of uniformity
of MRI acquisition techniques because some studies were performed at
institutions other than ours. This problem may have limited accurate
comparison of MRI features such as tumor signal intensity and contrast
enhancement both between patients and between imaging examinations for an
individual patient.
Despite the limitations, we found that in children the probability of the
presence of residual or recurrent desmoid tumor after surgical resection does
not appear to be related to sex or age at diagnosis. MRI assessment of
baseline tumor involvement of the neurovascular bundle and bone is probably
useful for prediction of the presence of residual or recurrent disease after
surgery. Baseline MRI assessment of tumor volume; distinctness of margins; and
T1-weighted, T2-weighted, and STIR signal intensity and contrast enhancement
patterns does not appear useful for prediction of the presence of residual or
recurrent tumor. Of the MRI features we investigated in tumors on which
follow-up was conducted until the end of chemotherapy and radiation therapy,
size alone seems to be the best indicator of the activity of desmoid tumors.
Even so, MRI is invaluable in detection of residual and recurrent disease and
in monitoring for tumor progression. Large prospective clinical trials with
standardized imaging time points are needed for better assessment of the
evolution of MRI features of desmoid tumors in response to therapy. In
addition, more precise pathologic–radiologic correlation, including
pathologic inspection of the entire resected tumor specimen, when achievable,
is necessary for full understanding of the significance of MRI signal
characteristics relative to the histologic features of desmoid tumor. Because
the biologic activity of this tumor cannot be predicted on the basis of
demographic, histologic, or conventional MRI features, functional imaging
techniques, such as dynamic contrast-enhanced MRI and contrast-enhanced
sonography for quantifying tumor perfusion and PET for assessing metabolic
activity, may offer valuable insight into the behavior of desmoid tumor. Such
information should allow oncologists to better tailor the clinical management
of this difficult disease.
Acknowledgments
We thank Tina Davis and Carrie Claggett for their patience and assistance
with data management.
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Abstract
Introduction
