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Volume 14
Fibrous Soft Tissue Tumors
Desmoid tumors---------------------Case 250-253 & 1170-1
Aponeuratic fibroma----------------Case 1172-1173
Infantile fibromatosis---------------Case 254
Elastofibroma------------------------Case 255
Nodular fascitis----------------------Case 256
Plantar fibroma-----------------------Case 257
Malignant fibrous histiocytoma----Case 258-265 &1174-75
Low grade fibromyxoid sarcoma---Case 1178
Fibrosarcoma--------------------------Case 266-7 & 1179-1181
Dermatofibrosarcoma protuberans-Case 268-269
Leiomyosarcoma----------------------Case 1182
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Desmoid Tumors
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Desmoid Tumor
One of the most common benign soft tissue tumors is the extra-
abdominal desmoid tumor, sometimes referred to as an aggressive
fibromatosis or simple desmoid tumor. As compared to the multitude
of different clinical types of infantile fibromatosis, the desmoid
tumor occurs in older children or young adults up through the age
of 40 years. The abdominal desmoid tumor is usually seen in young
adult females following pregnancy, whereas the extra-abdominal
desmoid tumor is found more commonly in men, usually in the
proximal parts of the body such as the shoulder, buttock and posterior
thigh area. Other areas include the popliteal space, the arm and
forearm. They are usually solitary lesions, however multi-centric
involvement can occur. In the case of “Gardner’s syndrome” the
desmoid tumor can be associated with polyposis of the large bowel
and cranial-facial osteomas. Desmoid tumors take their origin from
muscle fascial planes and tend to be extensively infiltrative into the
surrounding tissue with poorly defined margins that make surgical
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resection difficult. They can also occur in tendon sheaths, joint
capsules, and even inside bone. Clinically, the desmoid tumor has
the appearance of a low grade fibrosarcoma but tends to be more
invasive in character and can frequently engulf adjacent neuro-
vascular structures. Even though the desmoid tumor has a histo-
logical appearance similar to a low grade fibrosarcoma, it will
never metastasize to distant parts but can be multifocal in its
presentation in the extremity. Clinically, the desmoid tumor is
very firm to palpation. It frequently causes symptoms of pain and
is tender on deep palpation. Microscopically the tumor is heavily
collagenized but with a very low mitotic index, with an appearance
similar to plantar fibromatosis.
The desmoid tumor does not calcify but is radiodense on ordinary
radiographs and can be easily identified on a soft tissue window CT
scan. The MRI is the best imaging study for this tumor and will be
low signal on the T-1 weighted image but only intermediate high
signal on the T-2 because of the low water content in this tumor
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which helps separate it from malignant MFH and fibrosarcoma.
the desmoid tumor is related to physical activity compared to
higher grade spindle cell tumors that are more spontaneous in origin.
Treatment consists of a wide surgical resection, if possible, but
because of the high recurrence rate (about 50%), most clinicians
will advise post operative radiation therapy to the extent of 5000
centigray to the surgical site starting two weeks after surgery. This
reduces the chance of local recurrence to 10-15%. In rare situations
where the patient has multiple recurrences locally following RT,
amputation may be indicated. Even with amputation, recurrences
will be seen at the amputation site. In patients over 40 years of age
it is not unusual to see a spontaneous involution of desmoid tumors
requiring no treatment whatsoever. Estrogen may play a role in the
development of desmoid tumors and this accounts for the use of
tamoxifen with some reported clinical benefit. Other adjuvant
therapies include nonsteroidal anti-inflammatory agents and even
the use of mild chemotherapeutic agents.
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CLASSIC Case #250.1
50 year male with desmoid thigh
Axial T-1 MRI
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Coronal T-2 MRI
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Axial T-2 MRI
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Axial gad contrast MRI
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Resection specimen cut in path lab
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Photomic
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Higher power
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Case #250.2 Coronal T-1 MRI
45 year female with desmoid tumor gluteus maximus
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Axial T-1 MRI
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Axial T-2 MRI
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Resection specimen cut in path lab
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Photomic
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Case #250.3 Coronal T-1 MRI
61 year male with desmoid gluteus maximus
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Sagittal T-2 MRI
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Case #250.31
15 year female with painful enlarging desmoid past 6 months
while on chemotherapy
Sag T-2 Sag Gad
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Coronal Gad shows minimal response to chemotherapy
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Axial T-2
Axial Gad
vessels
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Case #250.4
61 year male
axillary desmoid
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Coronal T-1 MRI
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Coronal T-2 MRI
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Case #251
11 year male with desmoid foot
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Sagittal T-1 MRI
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Axial T-1 MRI
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Axial T-2 MRI
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Resected specimen with wedge cut out
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Case #252
11 year male
desmoid posterior
compartment thigh
Coronal T-1 MRI
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Coronal T-2 MRI
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Axial T-2 MRI
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Case #253
29 year male
recent fracture ulna
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18 months later
with desmoid tumor
at fracture site
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Sagittal T-2 MRI 18 months later with desmoid tumor
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Axial T-2 MRI at 18 months
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Photomic
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Post op resection
distal ulna with
synostosis of distal
ulnar tip to radius
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Case #253.1 Sagittal T-1 MRI
14 year male with desmoid tumor hand 2 years
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Sagittal T-2 MRI
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Axial T-1 MRI
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Axial T-2 MRI
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Case #1170 Axial T-1 MRI
45 year female with desmoid tumor post capsule knee
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Sagittal T-2 MRI
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Coronal T-2 MRI
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Coronal STIR MRI
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Surgical specimen cut in path lab
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Photomic
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Case #1170.1
61 year female with OA knee in 3/06 prior to TKA
P.O. desmoid
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Sag MRI 4/03
T-1 T-2
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MRI 1 year PO TKA
Sag T-2 Gad
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Axial
T-1 T-2
Gad
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Surgical resection
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Case #1171
9 year male with desmoid tumor posterior axilla
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CT scan
tumor
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Axial T-2 MRI
tumor
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Photomic
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Aponeuratic
Fibroma
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Case #1172
6 year male with aponeuratic fibroma middle finger
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Photomic showing punctate calcific chondroid tissue
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Another photomic with punctate calcification
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Case #1173
21 year male with aponeuratic fibroma index finger
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Surgical specimen showing calcium deposits
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Photomic showing chondroid calcification
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Infantile
Fibromatosis
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CLASSIC Case #254
2 month male with infantile fibromatosis right leg
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Dysplastic bowing fibula at age 2 years
AP LAT
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Progressive changes at 7 years
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Sagittal T-1 MRI at 7 years
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Sagittal T-2 MRI age 7
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Elastofibroma
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Elastofibroma
The elastofibroma is seen almost exclusively in people past the
age of 55 years. It is more common in women than men, and the
majority of cases are seen in those involved in heavy manual labor
or unusual sports requiring extensive use of the upper extremities.
It almost always occurs between the scapula and the chest wall
inferiorly and is bilateral in about 10% of cases. It is usually located
in the lower subscapular area, deep to the rhomboid and latissimus
dorsi muscles where it is firmly attached to the chest wall in the area
of the seventh and eighth ribs posteriorly. Grossly, the tumor has an
appearance similar to a desmoid tumor and microscopically there is
an intertwining of swollen eosinophillic collagen fibers and elastic
fibers in an equal proportion. The elastic fibers have a degenerated,
beaded appearance or are fragmented into small globules or droplets
that are easily seen with the Verhoeff stain that is specific for elastin
fiber. The cross section shows a characteristic serrated edge. The
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elastofibroma is considered a reactive pseudotumor with an excellent
prognosis. It is treated by a marginal surgical resection with an
extremely low recurrence rate and in some cases can be treated by
radiation therapy alone without surgery.
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CLASSIC Case #255 Axial T-1 MRI
50 female with subscapular elastofibroma
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Axial T-2 MRI
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Resected surgical specimen
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Photomic showing elastic fibers
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Elastic fibers seen with Verhoeff elastin stain
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Case #255.1
35 yr female with
slightly tender lump
below tip of scapula
for 1 yr
Axial T-1
T-2
Gad
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Cor T-1 T-2 Gad
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Nodular Fascitis
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Nodular Fascitis
Nodular fascitis is a very common, self-limiting, reactive process
rather than a true neoplasm occurring in young adults. Typically it
arises spontaneously and rapidly in the subcutaneous portion of the
lower aspect of the forearm and is associated with inflammatory
pain, swelling and local tenderness. Histologically, nodular fascitis
can take on the appearance of a spindle cell sarcoma. The lesion
grows quite rapidly over a period of several weeks, is usually
solitary, and usually occurs in the upper extremity. It is usually less
than 3 cm in diameter, has a grayish-white appearance and can have
myxoid changes. Histologically, the lesion is a fibroblastic pseudo-
tumor similar to granulation tissue with a fair number of mitotic
figures but no atypical mitoses will be seen. The fibroblasts are
infiltrated by lymphocytes. Treatment consists of local surgical
excision with a rare recurrence rate of less than 2%. No adjuvant
therapy is indicated.
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CLASSIC Case #256
25 year female with nodular fascitis forearm
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Surgical resection
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Surgical specimen
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Photomic
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Plantar Fibroma
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Plantar Fibroma The plantar fibroma is a fairly common clinical entity similar to
to the palmar fibroma. It arises from the plantar fascia and is usually
located on the medial portion of the arch of the foot. As opposed to
palmar fibromatosis that is typically seen in older patients, the plantar
fibroma is more common in young adults or even in children. In
some cases, there is a familial background (this is particularly true
in Scandinavians), with a greater incidence in males. The plantar
fibroma is frequently asymptomatic in which case surgical treatment
is not indicated. However, if pain results, especially if pressure on an
adjacent central nerve causes symptoms, a simple surgical resection
can be performed. Both the palmar and plantar fibromas have a
characteristic fibrotic histological appearance that is quite cellular
in areas and can be misdiagnosed as a malignant sarcoma. Some
patients will have fibromas in their hands and feet simultaneously
and can also have penile fibromas. A contracture of the finger is
typical in palmar fibromas whereas toe contractures are not seen in
plantar fibromatosis.
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CLASSIC Case #257
32 year female with plantar fibroma
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Photomic
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Higher power
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Malignant Fibrous
Histiocytoma
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Malignant Fibrous Histiocytoma
The malignant fibrous histiocytoma (MFH) is the most common
high grade soft tissue tumor. It occurs typically in older patients past
the age of 50 years and is more common in men. The most common
type is the so-called storiform or pleomorphic malignant fibrous
histiocytoma, followed next by the myxoid, giant cell, and inflamma-
tory subtypes. The pleomorphic form is seen in patients between the
the age of 50 and 70 years, and is usually found in a deep location in
a large muscle about the thigh, hip or retroperitoneal area. These
tumors usually present with symptoms of pain along with a tumor
mass that has been present for at least 3 or 4 months, but sometimes
these lesions can be asymptomatic. The gross appearance of the
tumor is a multinodular lesion that can have separate satellite lesions
located within the same muscle belly, usually at the upper or lower
pole of the lesion. On MRI, the lesion may demonstrate central
necrosis. The color is a dirty gray to reddish tan. Because this tumor
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is usually high grade, it demonstrates a high index of mitotic activity
and the cell types usually found are malignant fibroblasts mixed
with anaplastic pleomorphic histiocytes. This tumor has a high
potential to metastasize to the lung in about 40-45% of cases. It can
go to regional lymph nodes in 10% of cases. With appropriate
surgical and adjuvant therapy, the local recurrence rate is in the
neighborhood of 10-15% after a wide surgical resection. When these
tumors are located in a superficial, subcutaneous location and
present with a size less than 5 cm, the overall prognosis is much
better, with only a 20% chance of metastasis to the lung. However, in
the more common, deeply located lesions that measure over 5 cm in
diameter, the overall survival rate is 55%.
The usual treatment for the high grade MFH is a wide surgical
resection when possible. If the tumor is too large and cannot be
resected safely because of major neurovascular invasion or multiple
compartmental contamination, an aggressive high level amputation is
sometimes indicated. Following surgical resection, most patients
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with the more aggressive lesions will be treated with adjuvant
postoperative radiation therapy, in the neighborhood of 6500 centi
gray to the tumor site, with a wider field of 5500 centigray outside
the surgical site. At some institutions, patients will be treated with
preoperative radiation therapy, up to about 5500 centigray, with
an additional 1000 centigray being delivered two weeks after
surgery. The use of adjuvant chemotherapy for high grade soft tissue
sarcomas is quite controversial because there is minimal data to
suggest that the prognosis is significantly improved with its use.
Medical oncologists tend to discourage the use of this adjuvant,
especially in older patients who cannot tolerate the high toxicity
levels of current protocols.
The myxoid variant of the MFH is the second most common
type of MFH. Grossly it has a myxoid appearance and, because of
this, the chance of local contamination at the margins (with local
recurrence) is much higher. However, the metastatic rate to the lung
is only 25% giving the patient a better prognosis for survival.
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The giant cell variant, occurring in large muscle groups in older
patients, has a tendency to be a hemorrhagic tumor with a high
pulmonary metastatic rate of 50%. The inflammatory MFH is also
seen in older patients and is more common in the retroperitoneal
area, with a 50% metastatic rate to the lung.
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CLASSIC
Case #258
60 year male
MFH adductor
compartment
Coronal T-1 MRI
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Coronal T-2 MRI
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Axial T-2 MRI
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Widely resected specimen
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Specimen cut in path lab
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Photomic
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Higher power showing pleomorphic cells
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Case #258.1 Cor T-1 STIR
74 year male with painful mass adductor compartment 3 mos
MFH
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Axial T-1 T-2
Gad
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Case #258.2 Axial MRI
T-1 T-2
Gad C 64 year female with painful
and enlarging buttock mass
for 4 months
MFH
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Sag T-1 T-2
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Cor T-1 PD
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Case #259 Axial T-1 MRI
72 year male with MFH posterior compartment thigh
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Axial T-2 MRI
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Photomic
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Case #259.1
30 year male with enlarging painful thigh mass for 6 months
Giant cell variant of MFH
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Axial T-1 T-2
Gad
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Cor T-1 T-2 Gad
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Sag T-2 Gad
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Wide resection
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Case # 260 Axial T-1 MRI
67 year male with subcutaneous MFH thigh
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Axial T-2 MRI
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Sagittal T-2 MRI
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Case #261
62 year female
MFH anterior thigh
AP x-ray
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Sagittal proton
density MRI
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Sagittal T-2 MRI
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Axial proton density MRI
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Axial T-2 MRI
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Case #261.1
55 yr female with enlarging and painful popliteal mass for 5 mos
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Sag T-1 T-2
Gad
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Axial T-1 T-2 FS
Gad
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Cor T-1 T-2
Gad
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Surgical resection
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Case #262 Axial T-1 MRI
43 year female with juxta scapular myxoid MFH
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Axial T-2 MRI
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Another T-2 MRI
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Case #263 Coronal proton density MRI
72 year female with MFH deltoid muscle
tumor
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Another proton density coronal cut
tumor
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Resected specimen cut in path lab
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Photomic
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Case #263.1
83 year male with
painful axillary mass
3 months
MFH
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CT scan
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Cor T-1 T-2 Gad
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Axial T-1 T-2 FS Gad
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Case #264
79 year female with MFH forearm
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Sagittal T-1 MRI
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Axial T-1 MRI
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Axial T-2 MRI
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Case #264.1
78 year old female with painful mass forearm for 4 months
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Sag T-1 T-2 Gad
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Axial T-1 T-2
Gad
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Case #265 Axial T-2 MRI
65 year old female with large hematoma in MFH thigh
blood
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Axial proton density MRI
tumor
blood
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Axial T-2 MRI
blood
muscle
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Coronal T-1 MRI
blood
tumor
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Case #265.1
45 year male with
large forearm hematoma
and small MFH at base
of hematoma
blood
MFH
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Axial T-1 MRI blood
MFH
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Case #1174
78 year female with
large hematoma in
buttock area looking
like a hemorrhage
into an MFH
T-2 MRI showing
fluid-fluid level
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Axial T-1 MRI showing fluid-fluid level
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Case #1175
37 year female with recurrent hematoma from injury
16 years ago looking like hemorrhage into an MFH
Sagittal T-2 MRI showing fluid-fluid level
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Axial T-1 MRI
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Case #1175.1
55 year male with non tender
mass in pretibial area one year
Old hematoma
MFH pseudotumor
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Sag T-2 Gad
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Cor PD FS Axial Gad
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Case #1175.1
68 year female with tender swelling inner thigh 3 months
Cat scratch fever MFH pseudotumor
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Coronal STIR Sagittal T-2
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Axial T-1
Axial PD
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Low Grade
Fibromyxoid
Sarcoma
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Case #1178
55 year male with low grade fibromyxoid sarcoma shoulder
biopsy
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CT scan
tumor
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Another CT cut
tumor
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Axial T-1 MRI
tumor
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Axial T-2 MRI
tumor
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Surgical specimen
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Closer look similar to desmoid tumor
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Photomic
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Photomic showing myxoid area
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CT chest shows pulmonary mets
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Case #1178.1
35 year female with
tender lump on dorsum
of foot 6 months
Cor T-1
Gad
Low grade fibromyxoid sarc
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Axial T-1 Gad
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Fibrosarcoma
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Fibrosarcoma
The soft tissue fibrosarcoma is clinically very similar to the MFH,
except for the absence of malignant-appearing histiocytes. The
fibrosarcoma is less common compared to the MFH and occurs in a
slightly younger age group between the ages of 30 and 55 years,
with equal involvement in males and females. This tumor can be
very low grade, similar to a desmoid tumor, in which case it grows
slowly and is relatively painless. The high grade lesions are faster
growing, more painful and carry a worse prognosis. These tumors
tend to affect deep fascial structures, around muscles in the area of
the knee and thigh, followed next by the forearm and leg. Grossly,
the fibrosarcoma has a firm lobulated pattern with a yellowish-white
to tan coloration. Occasionally, there will be calcific ossifying
deposits seen on x-ray examination. Histologically, the fibrosarcoma
is composed of spindle cells that form collagen fiber with varying
degrees of mitotic activity from grade I through grade III or IV,
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but there are no malignant histiocytes seen the fibrosarcoma.
The treatment and prognosis depends on the grade of the tumor, with the grade I lesions behaving much like a desmoid tumor with a
very slight potential for pulmomary metastases, whereas the grade
IV have a high potential for metastasis to the lung in about 50-60%
of cases. It is rare to see lymph node involvement. Treatment
consists of wide surgical resection. With the larger deep lesions,
resection is usually followed by local radiation therapy to the extent
of 6500 centigray, however the use of adjuvant chemotherapy is
considered controversial. As in the treatment of MFH, chemotherapy
is not usually advocated by medical oncologists.
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Case #266
41 year male
fibrosarcoma
forearm
Axial T-2 MRI CLASSIC
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Coronal T-2 MRI
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Axial T-2 MRI
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Wide resection specimen cut in path lab
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Photomic
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Higher power
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Case #267 Coronal proton density MRI
55 year male with fibrosarcoma vastus intermedius
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Axial T-2 MRI
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Surgical specimen cut in path lab
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Photomic
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Case #1179
52 year male with
fibrosarcoma arm
Axial T-1 MRI
tumor
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Axial T-2 MRI
tumor
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Surgical specimen
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Photomic
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Case #1180 Axial T-2 MRI
26 year male with fibrosarcoma calf
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Case # 1181 Sagittal T-1 MRI
51 year female with fibrosarcoma ankle
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Axial T-1 MRI
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Dermatofibrosarcoma
Protuberans
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Dermatofibrosarcoma Protuberans
The dermatofibrosarcoma protuberans is considered a low to
intermediate grade fibrohistiocytic tumor characteristic in its nodular
cutaneous location and occurring far more often in males then in
females, in a young to middle-aged adult population. It is most
commonly found in the trunk or proximal extremities and can be
associated with antecedent trauma in about 15% of cases. It usually
comes on gradually as a subcutaneous nodule that is relatively
painless and therefore patients may not present to a physician for
treatment for many years. Histologically, the tumor consists of low
grade spindle cells without evidence of mitotic activity and low
grade histiocytes. The spindle cell pattern is typically a storiform
one, similar to the fibrosarcoma and MFH. The tumor tends to invade
into the adjacent subcutaneous fat and skin and requires a very wide
surgical resection to avoid a high local recurrence rate. The chance
of pulmonary metastasis, however, is very low (1%). With a success-
ful wide resection there is no need for adjuvant therapies.
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Case #268
30 year male with dermatofibrosarcoma protuberans
on the bottom of the heel
CLASSIC
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Macro section of resection specimen
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Photomic
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Higher power
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Case #269
Dermatofibrosarcoma protuberans resection specimen
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Specimen cut in path lab
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Macro section
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Photomic
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Leiomyosarcoma
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Case #1182 Axial PD MRI
47 year female with leiomyosarcoma inguinal area
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Axial T-1 MRI
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Coronal PD MRI showing tumor arising from femoral vein
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Photomic