AJR 2005; 184:1749-1753
© American Roentgen Ray Society
Myxoinflammatory Fibroblastic Sarcoma: MR Appearance and Pathologic Correlation
Ukihide Tateishi1,
Tadashi Hasegawa2,
Hiroaki Onaya1,
Mitsuo Satake1,
Yasuaki Arai1 and
Noriyuki Moriyama1
1 Division of Diagnostic Radiology, National Cancer Center Hospital and
Institute, Tsukiji, Chuo-Ku, 104-0045, Tokyo, Japan.
2 Pathology Division, National Cancer Center Hospital and Institute, Tsukiji,
Tokyo, Japan.
Received June 2, 2004;
accepted after revision July 28, 2004.
Address correspondence to U. Tateishi.
Supported in part by grant for Scientific Research Expenses for Health and
Welfare Programs, The Foundation for the Promotion of Cancer Research, and
second-term Comprehensive 10-year Strategy for Cancer Control.
Abstract
OBJECTIVE. The purpose of our study was to define the MR appearance
of myxoinflammatory fibroblastic sarcoma of the soft tissues and to make
correlations with the histopathologic features.
CONCLUSION. Myxoinflammatory fibroblastic sarcoma is an uncommon
malignancy that typically affects adult subjects, who present with painless
swelling. This lesion manifests on MR images as a poorly circumscribed mass
involving the underlying tendon sheath in the distal extremities.
Introduction
Myxoinflammatory fibroblastic sarcoma of the soft tissues is a rare
low-grade tumor of uncertain origin that usually arises in the hands and feet.
Myxoinflammatory fibroblastic sarcoma was first described in 1998 by
Meis-Kindblom and Kindblom [1].
Montgomery et al. [2] named the
tumor "inflammatory myxohyaline tumor" of the distal extremities
with virocyte or Reed-Sternberg-like cells. Histologic characteristics are the
spindle to epithelioid neoplastic cells as the manifestation of malignancy
admixed with the myxoid and hyalinized matrix, the inflammatory infiltrate,
and bizarre virocyte or Reed-Sternberg-like cells with enlarged vesicular
nuclei
[1-3].
More than 100 cases of myxoinflammatory fibroblastic sarcoma have been
reported, with a large series identified in two articles
[1-6].
However, MRI findings of myxoinflammatory fibroblastic sarcoma have rarely
been documented. The purpose of this study was to characterize the MR
appearance of myxoinflammatory fibroblastic sarcoma and to correlate that
appearance with the histopathologic features.
Materials and Methods
MR images of all patients with pathologically proven myxoinflammatory
fibroblastic sarcoma at our institution were retrospectively reviewed. Our
institutional review board gave its approval for a review of patient records
and images. The patients were identified by review of our institution's
pathology database for a 2-year period. The affected patients included three
males and one female who ranged in age from 15 to 62 years old (mean age, 35
years). All histopathologic specimens were reviewed by an experienced
pathologist to confirm the diagnosis. Histopathologic examination in all
patients showed spindle and epithelioid tumor cells with mild nuclear atypia.
Ganglionlike cells and Reed-Sternberg-like cells were also prominent in all
cases. Inflammatory cells, including neutrophils, lymphocytes, and
eosinophils, were densely present in all cases. Immunohistochemistry was
performed in all patients, and all tumors displayed immunoreactivity to
vimentin, smooth-muscle actin, and CD34. These histopathologic characteristics
were compatible with the diagnosis of myxoinflammatory fibroblastic sarcoma
[7]. Medical records were
reviewed by one of the authors for presenting complaints, disease progression,
and outcome. Radiographs, available for all patients, were also evaluated by
two radiologists for the presence of soft-tissue masses or nodules,
mineralization, and bone destruction. The findings were recorded by
consensus.
T1- and T2-weighted MR images were obtained in the sagittal and coronal
planes using a surface coil. T1-weighted conventional spin-echo MR images were
obtained using a 20-cm field of view, 3.5- to 5-mm section thickness, TR
range/TE of 450-520/15, 160 x 256 matrix, and 2 signals acquired.
T2-weighted fast spin-echo acquisitions with (n = 3) or without
(n = 1) fat suppression were performed using a 20-cm field of view,
3.5- to 5-mm section thickness, 3,600-4,000/120, 160 x 256 matrix, and 2
signals acquired. After the IV administration of 0.1 mmol of gadopentetate
dimeglumine (Magnevist, Schering) per kilogram of body weight, transverse
T1-weighted images with (n = 3) or without (n = 1) fat
suppression were obtained in the sagittal and coronal planes.
MR images were reviewed by two radiologists and findings were recorded by
consensus. Images were evaluated for lesion location and size, depth
(superficial or deep), shape of margin (well or ill defined), and the presence
or absence of extracompartmental extension. To define depth, superficial
lesions did not involve the superficial fascia, and deep lesions were deep in
relation to or invaded the superficial fascia. The relationship between tumor
and the underlying tendon sheath was also evaluated. MR images were evaluated
for predominant signal intensity characteristics (low, intermediate, high),
signal homogeneity or heterogeneity, and enhancement characteristics. On
T1-weighted images, low signal intensity was defined as signal intensity less
than that of muscle; intermediate signal intensity, similar to that of muscle;
and high signal intensity, similar to that of fat. On T2-weighted images, low
signal intensity was defined as signal intensity similar to that of muscle;
intermediate signal intensity, greater than that of muscle but less than that
of fat; and high signal intensity, equal to or greater than that of fat. Tumor
enhancement was visually graded as greater than, less than, or equal to that
of surrounding muscle and vessels.
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Fig. 1A. —Myxoinflammatory fibroblastic sarcoma in 62-year-old man with
painless mass in finger. Sagittal T1-weighted MR image (TR/TE, 450/15) shows
poorly circumscribed mass beneath tendon sheath of flexor hallucis longus
(arrows).
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Fig. 1C. —Myxoinflammatory fibroblastic sarcoma in 62-year-old man with
painless mass in finger. Photograph of histopathologic specimen shows solid
nests of spindle and epithelioid tumor cells with foci of inflammatory cell
infiltrate (arrows).
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Results
Clinical Features
All patients were symptomatic at presentation. Presenting complaints were
painless swelling of the distal extremities. The mean symptom duration was 4.8
months. Tumors arose from the feet (n = 2), hands (n = 1),
and fingers (n = 1). All patients received excisional biopsy for
definitive diagnosis and primary therapy. Surgical margins were adequate in
three patients and inadequate in one patient. The one patient with an
inadequate surgical margin underwent subsequent wide resection. Chemotherapy
and radiation therapy were not included in the treatment regimen in any
patient. Local recurrence occurred 26.5 months after the initial surgery in
two patients. These patients received wide resection. At the latest follow-up
(27-82 months; mean, 45 months), no patients had developed further recurrence
or metastasis.
MRI Findings and Pathologic Correlations
The gross characteristics of the resected specimens featured multinodular
architecture corresponding to MRI features. The mean tumor diameter was 2.4 cm
(range, 1.2-3.0 cm). Tumors were located along the tendon sheath in all
patients. Findings of extensive involvement surrounding the tendon sheath by
the tumor were seen. In two patients, the tumor existed beneath the tendon
sheath (Figs. 1A,
1B, and
1C), and in two it involved the
surrounding tendon sheath diffusely and focally infiltrated the dermis (Figs.
2A,
2B, and
2C). One patient had an
ill-defined, irregularly marginated mass involving the ulnar nerve and the
tendon sheath of the flexor carpi ulnaris (Figs.
2A,
2B, and
2C).
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Fig. 2A. —Myxoinflammatory fibroblastic sarcoma in 31-year-old man with
painless mass in subcutaneous soft tissue of wrist. Coronal contrast-enhanced
T1-weighted MR image (TR/TE, 520/15) shows poorly circumscribed mass with
ill-defined border. Tumor involves surrounding tendon sheath diffusely and
focally infiltrates dermis (arrow).
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Fig. 2B. —Myxoinflammatory fibroblastic sarcoma in 31-year-old man with
painless mass in subcutaneous soft tissue of wrist. Axial contrast-enhanced
T1-weighted MR image (520/15) shows mass involving ulnar nerve
(arrow) and tendon sheath of flexor carpi ulnaris
(arrowhead).
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Fig. 2C. —Myxoinflammatory fibroblastic sarcoma in 31-year-old man with
painless mass in subcutaneous soft tissue of wrist. Photograph of
histopathologic specimen reveals that numerous small nodules consisting of
tumor cells infiltrate along ulnar nerve (arrows).
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Cortical invasion was not identified in any patient on radiographs. All
tumors showed predominantly low signal intensity relative to muscle on
T1-weighted MR images (Figs.
3A,
3B, and
3C). Two lesions showed
moderate and homogeneous enhancement after the IV administration of contrast
material (Figs. 1A,
1B,
1C,
3A,
3B, and
3C). The cut surface of
resected specimens showed solid nests of neoplastic cells that featured
spindle and epithelioid cells with higher cellularity, which corresponded to
homogeneous enhancement on contrast-enhanced MR images. Two lesions showed
heterogeneous enhancement of the tumor that correlated with geographic areas
of the myxoid stromal matrix on microscopic observations (Figs.
4A,
4B, and
4C). On T2-weighted MR images,
all lesions had intermediate signal intensity greater than that of muscle but
less than that of fat (Figs.
2A,
2B, and
2C). In all cases, the cut
surface of specimens revealed solid nests of cellular areas with foci of
hyalinized collagen fibers and hypocellular areas with a myxoid stromal
matrix, which corresponded to the imaging appearance of intermediate signal
intensity on T2-weighted MR images.
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Fig. 3A. —Myxoinflammatory fibroblastic sarcoma in foot of 32-year-old
woman with local recurrence. Sagittal T2-weighted MR image (TR/TE, 3,600/120)
shows mass of sheetlike appearance beneath dorsal portion of tendon sheath.
Tumor shows intermediate signal intensity, greater than that of muscle
(arrow).
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Fig. 3B. —Myxoinflammatory fibroblastic sarcoma in foot of 32-year-old
woman with local recurrence. Sagittal contrast-enhanced fat-saturated
T1-weighted MR image (520/15) shows homogeneous enhancement of tumor
(arrows).
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Fig. 3C. —Myxoinflammatory fibroblastic sarcoma in foot of 32-year-old
woman with local recurrence. Photograph of histopathologic specimen shows
sheetlike proliferation of spindle-shaped tumor cells (arrows) with
ganglionlike cells, Reed-Sternberg-like cells, and lymphoid cells surrounding
tendon sheaths.
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Fig. 4A. —Myxoinflammatory fibroblastic sarcoma in foot of 24-year-old
man with local recurrence. Coronal fat-saturated T2-weighted MR image (TR/TE,
3,500/105) shows mass of branching pattern that occurred along extensor
digitorum longus tendon sheaths of second and fourth toes (arrows).
Tumor shows intermediate signal intensity, greater than that of muscle.
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Fig. 4B. —Myxoinflammatory fibroblastic sarcoma in foot of 24-year-old
man with local recurrence. Coronal contrast-enhanced T1-weighted MR image
(520/15) shows heterogeneous enhancement of tumor (arrows).
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Fig. 4C. —Myxoinflammatory fibroblastic sarcoma in foot of 24-year-old
man with local recurrence. Photograph of histopathologic specimens shows
proliferation of spindle-shaped tumor cells (arrows) with prominent
nucleoli in abundant myxoid stromal matrix.
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Two patients developed recurrent tumors and underwent follow-up MRI after
treatment. One patient developed a mass of sheetlike appearance beneath the
dorsal portion of the underlying tendon sheath (Figs.
3A,
3B, and
3C). Signal characteristics and
homogeneous enhancement patterns were similar to those of the primary tumors.
Histopathologic examination of this patient showed an infiltrate of lymphoid
cells and a marked proliferation of spindle-shaped tumor cells surrounding the
tendon sheaths.
In the second patient, a mass of branching pattern occurred along the
extensor digitorum longus tendon sheaths of the second and fourth toes without
distortion of the architecture of the tendon sheaths (Figs.
4A,
4B, and
4C). This patient had also MRI
findings suggesting capsular involvement in the metatarsophalangeal joint of
the second toe. Histopathologic examination revealed that the tumor arose from
the extensor digitorum longus tendon sheaths and also involved the extensor
digitorum brevis tendon sheath, cutaneous nerve, and dermis.
Discussion
Myxoinflammatory fibroblastic sarcoma is a rare tumor of the subcutaneous
soft tissue that can arise on the trunk but most commonly occurs in the
distant extremities [1,
2]. According to the literature
and our experience, myxoinflammatory fibroblastic sarcoma is a tumor that most
commonly affects adults who are symptomatic at presentation
[1,
2]. All patients in our series
were symptomatic, with common complaints of a painless mass.
Myxoinflammatory fibroblastic sarcoma has a relatively good prognosis with
a long life expectancy despite frequent local recurrence
[1-3].
Two of our patients developed local recurrence, with an average duration of
26.5 months. According to the literature, the local recurrence rate in
patients with myxoinflammatory fibroblastic sarcoma ranges from 22% to 67%
[1,
2]. The metastasis rate in
patients with myxoinflammatory fibroblastic sarcoma is uncertain. Metastases
have been reported to develop in only a few cases
[1].
In all of our patients, excisional biopsy for definitive diagnosis and
primary therapy was performed. However, tumor margins in one of our patients
were inadequate and the patient underwent subsequent wide resection. Tumors
are often removed piecemeal by surgical procedures, with curative wide
resection considered to be the adequate treatment of choice
[1].
Grossly, myxoinflammatory fibroblastic sarcoma forms a poorly circumscribed
mass surrounding the tendon sheath that may extend into the dermis and
skeletal muscle. Microscopically, the tumor is characterized by solid nests of
atypical spindle and epithelioid cells in a myxoid stroma and dense
inflammatory infiltrates. The tumor cells often have large vesicular nuclei
similar to those of virocytes or Reed-Sternberg cells. The immunophenotype is
positive for vimentin, with variable immunoreactivity for CD34, CD68,
cytokeratin, and smooth-muscle actin
[1-6].
On MR images, myxoinflammatory fibroblastic sarcoma typically manifests as
a poorly circumscribed mass with a multinodular appearance. Extensive
involvement surrounding the tendon sheath is also a common feature.
The appearance of the extension along the tendon sheath in this tumor is
similar to that seen in tenosynovitis. Differentiating tenosynovitis from
myxoinflammatory fibroblastic sarcoma solely on MRI findings is difficult.
Tenosynovitis also can lead to an ill-defined soft-tissue mass or enlargement
of its sheath. However, this condition typically manifests as the accumulation
of fluid with increased signal intensity of the affected tendon on T2-weighted
MR images [8]. Clinical
characteristics can allow the differentiation of tenosynovitis from
myxoinflammatory fibroblastic sarcoma because tenosynovitis often decreases in
size during the course of disease, whereas myxoinflammatory fibroblastic
sarcoma usually grows with infiltration
[1].
MRI findings of myxoinflammatory fibroblastic sarcoma also closely resemble
those of giant cell tumors of the tendon sheath, proliferative fasciitis,
acral fibromyxoma, myxoid liposarcoma, and myxofibrosarcoma
[9-13].
These conditions could not be distinguished radiologically from
myxoinflammatory fibroblastic sarcoma on the basis of our study results.
Signal characteristics and enhancement patterns were nonspecific. However,
heterogeneous enhancement on contrast-enhanced MR images corresponded to
geographic areas of the myxoid stromal matrix in the pathologic specimens. In
two of our patients, MRI findings of recurrent tumors were ill defined and the
tumors had sheetlike appearances involving the tendon sheath. A significant
association may exist between recurrent tumors and the tendon sheath.
In summary, myxoinflammatory fibroblastic sarcoma typically affects adult
subjects as a painless mass of the distal extremities at presentation.
Myxoinflammatory fibroblastic sarcoma usually manifests on MR images as a
multinodular and poorly circumscribed mass involving the surrounding tendon
sheath. Although it is unlikely that such a rare condition could reasonably be
diagnosed on the basis of MRI findings alone, the condition should be
considered in the differential diagnosis of a soft-tissue mass in the distal
extremities of adult patients.
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Abstract
Introduction
