14 fast vol 14

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

Desmoid Tumors

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

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

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.

CLASSIC Case #250.1

50 year male with desmoid thigh

Axial T-1 MRI

Coronal T-2 MRI

Axial T-2 MRI

Axial gad contrast MRI

Resection specimen cut in path lab

Photomic

Higher power

Case #250.2 Coronal T-1 MRI

45 year female with desmoid tumor gluteus maximus

Axial T-1 MRI

Axial T-2 MRI

Resection specimen cut in path lab

Photomic

Case #250.3 Coronal T-1 MRI

61 year male with desmoid gluteus maximus

Sagittal T-2 MRI

Case #250.31

15 year female with painful enlarging desmoid past 6 months

while on chemotherapy

Sag T-2 Sag Gad

Coronal Gad shows minimal response to chemotherapy

Axial T-2

Axial Gad

vessels

Case #250.4

61 year male

axillary desmoid

Coronal T-1 MRI

Coronal T-2 MRI

Case #251

11 year male with desmoid foot

Sagittal T-1 MRI

Axial T-1 MRI

Axial T-2 MRI

Resected specimen with wedge cut out

Case #252

11 year male

desmoid posterior

compartment thigh

Coronal T-1 MRI

Coronal T-2 MRI

Axial T-2 MRI

Case #253

29 year male

recent fracture ulna

18 months later

with desmoid tumor

at fracture site

Sagittal T-2 MRI 18 months later with desmoid tumor

Axial T-2 MRI at 18 months

Photomic

Post op resection

distal ulna with

synostosis of distal

ulnar tip to radius

Case #253.1 Sagittal T-1 MRI

14 year male with desmoid tumor hand 2 years

Sagittal T-2 MRI

Axial T-1 MRI

Axial T-2 MRI

Case #1170 Axial T-1 MRI

45 year female with desmoid tumor post capsule knee

Sagittal T-2 MRI

Coronal T-2 MRI

Coronal STIR MRI

Surgical specimen cut in path lab

Photomic

Case #1170.1

61 year female with OA knee in 3/06 prior to TKA

P.O. desmoid

Sag MRI 4/03

T-1 T-2

MRI 1 year PO TKA

Sag T-2 Gad

Axial

T-1 T-2

Gad

Surgical resection

Case #1171

9 year male with desmoid tumor posterior axilla

CT scan

tumor

Axial T-2 MRI

tumor

Photomic

Aponeuratic

Fibroma

Case #1172

6 year male with aponeuratic fibroma middle finger

Photomic showing punctate calcific chondroid tissue

Another photomic with punctate calcification

Case #1173

21 year male with aponeuratic fibroma index finger

Surgical specimen showing calcium deposits

Photomic showing chondroid calcification

Infantile

Fibromatosis

CLASSIC Case #254

2 month male with infantile fibromatosis right leg

Dysplastic bowing fibula at age 2 years

AP LAT

Progressive changes at 7 years

Sagittal T-1 MRI at 7 years

Sagittal T-2 MRI age 7

Elastofibroma

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

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.

CLASSIC Case #255 Axial T-1 MRI

50 female with subscapular elastofibroma

Axial T-2 MRI

Resected surgical specimen

Photomic showing elastic fibers

Elastic fibers seen with Verhoeff elastin stain

Case #255.1

35 yr female with

slightly tender lump

below tip of scapula

for 1 yr

Axial T-1

T-2

Gad

Cor T-1 T-2 Gad

Nodular Fascitis

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.

CLASSIC Case #256

25 year female with nodular fascitis forearm

Surgical resection

Surgical specimen

Photomic

Plantar Fibroma

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.

CLASSIC Case #257

32 year female with plantar fibroma

Photomic

Higher power

Malignant Fibrous

Histiocytoma

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

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

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.

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.

CLASSIC

Case #258

60 year male

MFH adductor

compartment

Coronal T-1 MRI

Coronal T-2 MRI

Axial T-2 MRI

Widely resected specimen

Specimen cut in path lab

Photomic

Higher power showing pleomorphic cells

Case #258.1 Cor T-1 STIR

74 year male with painful mass adductor compartment 3 mos

MFH

Axial T-1 T-2

Gad

Case #258.2 Axial MRI

T-1 T-2

Gad C 64 year female with painful

and enlarging buttock mass

for 4 months

MFH

Sag T-1 T-2

Cor T-1 PD


72 year male with MFH posterior compartment thigh

Axial T-2 MRI

Photomic

Case #259.1

30 year male with enlarging painful thigh mass for 6 months

Giant cell variant of MFH

Axial T-1 T-2

Gad

Cor T-1 T-2 Gad

Sag T-2 Gad

Wide resection

Case # 260 Axial T-1 MRI

67 year male with subcutaneous MFH thigh

Axial T-2 MRI

Sagittal T-2 MRI

Case #261

62 year female

MFH anterior thigh

AP x-ray

Sagittal proton

density MRI

Sagittal T-2 MRI

Axial proton density MRI

Axial T-2 MRI

Case #261.1

55 yr female with enlarging and painful popliteal mass for 5 mos

Sag T-1 T-2

Gad

Axial T-1 T-2 FS

Gad

Cor T-1 T-2

Gad

Surgical resection


43 year female with juxta scapular myxoid MFH

Axial T-2 MRI

Another T-2 MRI

Case #263 Coronal proton density MRI

72 year female with MFH deltoid muscle

tumor

Another proton density coronal cut

tumor

Resected specimen cut in path lab

Photomic

Case #263.1

83 year male with

painful axillary mass

3 months

MFH

CT scan

Cor T-1 T-2 Gad

Axial T-1 T-2 FS Gad

Case #264

79 year female with MFH forearm

Sagittal T-1 MRI

Axial T-1 MRI

Axial T-2 MRI

Case #264.1

78 year old female with painful mass forearm for 4 months

Sag T-1 T-2 Gad

Axial T-1 T-2

Gad


65 year old female with large hematoma in MFH thigh

blood

Axial proton density MRI

tumor

blood

Axial T-2 MRI

blood

muscle

Coronal T-1 MRI

blood

tumor

Case #265.1

45 year male with

large forearm hematoma

and small MFH at base

of hematoma

blood

MFH

Axial T-1 MRI blood

MFH

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

Axial T-1 MRI showing fluid-fluid level

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

Axial T-1 MRI

Case #1175.1

55 year male with non tender

mass in pretibial area one year

Old hematoma

MFH pseudotumor

Sag T-2 Gad

Cor PD FS Axial Gad

Case #1175.1

68 year female with tender swelling inner thigh 3 months

Cat scratch fever MFH pseudotumor

Coronal STIR Sagittal T-2

Axial T-1

Axial PD

Low Grade

Fibromyxoid

Sarcoma

Case #1178

55 year male with low grade fibromyxoid sarcoma shoulder

biopsy

CT scan

tumor

Another CT cut

tumor

Axial T-1 MRI

tumor

Axial T-2 MRI

tumor

Surgical specimen

Closer look similar to desmoid tumor

Photomic

Photomic showing myxoid area

CT chest shows pulmonary mets

Case #1178.1

35 year female with

tender lump on dorsum

of foot 6 months

Cor T-1

Gad

Low grade fibromyxoid sarc

Axial T-1 Gad

Fibrosarcoma

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,

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.

Case #266

41 year male

fibrosarcoma

forearm

Axial T-2 MRI CLASSIC

Coronal T-2 MRI

Axial T-2 MRI

Wide resection specimen cut in path lab

Photomic

Higher power

Case #267 Coronal proton density MRI

55 year male with fibrosarcoma vastus intermedius

Axial T-2 MRI

Surgical specimen cut in path lab

Photomic

Case #1179

52 year male with

fibrosarcoma arm

Axial T-1 MRI

tumor

Axial T-2 MRI

tumor

Surgical specimen

Photomic


26 year male with fibrosarcoma calf

Case # 1181 Sagittal T-1 MRI

51 year female with fibrosarcoma ankle

Axial T-1 MRI

Dermatofibrosarcoma

Protuberans

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.

Case #268

30 year male with dermatofibrosarcoma protuberans

on the bottom of the heel

CLASSIC

Macro section of resection specimen

Photomic

Higher power

Case #269

Dermatofibrosarcoma protuberans resection specimen

Specimen cut in path lab

Macro section

Photomic

Leiomyosarcoma

Case #1182 Axial PD MRI

47 year female with leiomyosarcoma inguinal area

Axial T-1 MRI

Coronal PD MRI showing tumor arising from femoral vein

Photomic

14 fast vol 14

Documents