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Fibrosarcoma
Background
Fibrosarcoma is a tumor of mesenchymal cell origin that is composed of malignant
fibroblasts in a collagen background. It can occur as a soft-tissue mass or as a primary or
secondary bone tumor. Fibrosarcoma was diagnosed much more frequently in the past; it
is now more reliably distinguished histologically from similar lesions, such as desmoid
tumors, malignant fibrous histiocytoma, malignant schwannoma, and high-grade
osteosarcoma.
Images of fibrosarcoma are provided below:

Although fibrosarcoma of bone can arise anywhere, it is found most commonly about the
knee and femur. The radiograph here shows a typical
appearance of a lesion in bone.

Most pathologists describe the histologic picture of fibrosarcoma as a herringbone

pattern. It is an interlacing pattern of sheets of spindle-shaped fibroblasts in a collagen
background. This pattern is very distinctive and usually confirms the diagnosis of
fibrosarcoma.
The 2 main types of fibrosarcoma of bone are primary and secondary. Primary
fibrosarcoma is a fibroblastic malignancy that produces variable amounts of collagen. It
is either central (arising within the medullary canal) or peripheral (arising from the
periosteum). Secondary fibrosarcoma of bone arises from a preexisting lesion or after
radiotherapy to an area of bone or soft tissue. This is a more aggressive tumor and has a
poorer prognosis.
Recent studies
Russell et al reported on 4 patients with infantile fibrosarcoma (IF) treated with
chemotherapy and surgical resection, all of whom had excellent functional outcome. The
patients were diagnosed from birth up to 7 months of age, with 3 of the patients having
lower-extremity tumors and 1 having a neck tumor. The patients received combinationchemotherapy regimens consisting of vincristine, cyclophosphamide, and actinomycin;
one patient also received ifosfamide and etoposide because of tumor progression. The
patient with fibrosarcoma of the neck displayed rapid tumor shrinkage. Two of the lowerextremity tumors had only modest changes in dimensions, but upon resection, the tumor
bed contained fibrous tissue with exaggerated small caliber vessels. In the fourth case,
metastatic lesions developed in the central nervous system, orbits, lungs, and kidney after
complete removal of the primary tumor.[1]
Canale et al performed a retrospective review of MRI features in 6 cases of infantile
fibrosarcoma, with patients ranging in age from 0 to 6 months. A well-circumscribed
single mass was the most common finding (5 patients), and all the tumors were on limbs.
The initial tumor signal was isointense to muscle on T1-weighted images and

hyperintense on T2-weighted images, with all tumors being well circumscribed and half
of them containing internal fibrous septa. In 3 patients, the internal signal was
homogeneous; they were heterogeneous in the other 3. An intense enhancement was seen
in the 3 contrast-enhanced images that were available: heterogeneous in 2; homogeneous
in 1. There was osseous erosion observed in the patient with distant metastasis. The
tumors in all cases disappeared with chemotherapy and limited surgery.[2]

History of the Procedure

As with all soft-tissue and bone sarcomas, the mainstay of treatment for fibrosarcoma has
been complete excision with an adequate margin; this procedure became prevalent
following the publication and wide acceptance of Enneking's surgical principles of
musculoskeletal oncology, in the early 1980s. This surgery normally consists of resecting
a cuff of normal tissue along with the tumor.

Problem
As with all sarcomas, the long-term survival and ultimate functional outcome of cancer
treatment depend on many interrelated factors. Among these are the size and location of
the tumor, its histologic grade, and the presence of metastatic disease (eg, pulmonary
metastases). These factors are taken into account with careful evaluation (staging) of the
tumor and determine the success of treatment in obtaining good local control and
preventing subsequent disease spread.

Epidemiology
Frequency
Fibrosarcoma represents only about 10% of musculoskeletal sarcomas and less than 5%
of all primary tumors of bone. No known racial predilection exists.
Fibrosarcoma of bone occurs slightly more commonly in men than in women.
Fibrosarcoma of bone can be diagnosed in patients of any age, but it is diagnosed more
commonly in patients in the fourth decade of life. It is usually located in the lower
extremities, especially the femur and tibia.
Fibrosarcoma of the soft tissues usually affects a wider age spectrum of patients than
fibrosarcoma of the bone does, with an age range of 35-55 years. It often arises in the soft
tissues of the thigh and the posterior knee. It is generally a large, painless mass deep to
fascia and has an ill-defined margin.
An infantile form (in children < 10 y) of fibrosarcoma exists. Unlike fibrosarcoma in
adults, it has an excellent prognosiseven in the face of metastatic disease at
presentationwhen treated with a combination of neoadjuvant and adjuvant
chemotherapy and resection.[1, 2, 3]

Etiology
Fibrosarcoma, like other soft-tissue sarcomas, has no definite cause. Current research
indicates that many sarcomas are associated with genetic mutations. The more common
genetic defects include allele loss, point mutations, and chromosome translocations. See
Pathophysiology for a discussion of associated conditions.

Pathophysiology
No definite cause of fibrosarcoma is known, although genetic mutations may play a role.
Several inherited syndromes are associated with sarcomas. For example, patients with
multiple neurofibromas may have a 10% lifetime risk of developing a neurosarcoma or a
fibrosarcoma.
The occurrence of fibrosarcoma in conjunction with metallic implants used for fracture
fixation or joint reconstruction has been reported, albeit very rarely. The cause of this
transformation is unknown.
Fibrosarcoma has also been noted to arise from preexisting lesions, such as bone infarcts
and lesions associated with fibrous dysplasia, chronic osteomyelitis, and Paget disease, as
well as in previously irradiated areas of bone. This form of fibrosarcoma is very
aggressive and is associated with a much poorer outcome than is the primary
fibrosarcoma of bone.

Presentation
Sarcomas involving bone often present with pain and swelling after a long duration of
symptoms. They may even grow large enough to threaten the structural integrity of the
bone and cause pathologic fracture as the initial presentation. Generally, lesions that
involve more than 50% of the bone cortex, that are larger than 2 cm, or that involve the
medial calcar of the femur are associated with the greatest risk of fracture. A prior history
of bone infarct, irradiation, or other such risk factors should alert the physician to the
possibility of a secondary fibrosarcoma.
Soft-tissue sarcomas most often present as painless masses. The time to presentation,
however, is often shorter than with lesions involving bone. Because these lesions
frequently arise deep to the muscular fascia, they may become extremely large tumors
prior to diagnosis.
Most lesions occur around the knee, in the proximal femur and hip region, or in the
proximal arm. Findings are nonspecific and can vary from a fixed, firm mass to a
localized area of tenderness. Neurologic or vascular changes are late findings and
indicate extensive disease involvement.
Differential diagnoses include the following:

Fibrous dysplasia

Fibrous histiocytoma

Osteosarcoma

Paget sarcoma

Malignant fibrous histiocytoma

Malignant neurosarcoma

Indications
To obtain local control, surgical resection with a cuff of normal tissue (wide margins) and
reconstruction of the subsequent defect are necessary.

Relevant Anatomy
See Introduction, Clinical.

Contraindications
Surgical treatment (including biopsy) of fibrosarcoma should not proceed unless
complete patient care is available. Complete care includes biopsy and interpretation of
biopsy findings, access to oncologists and radiation oncologists, and definitive resection.
Fibrosarcomas should be removed by trained orthopedic oncologists who can provide a
state-of-the-art treatment program; this would involve a team of well-trained specialists
with advanced experience in treating these tumors.

Laboratory Studies

Laboratory studies generally are not helpful during the initial evaluation.

Imaging Studies

Plain radiographs
o

Plain radiographs of the involved anatomic region are needed to evaluate

for primary or secondary involvement of bone. Typically, an osteolytic
area of destruction with a permeative or moth-eaten appearance is present.
Little periosteal reaction or reactive sclerosis is depicted.

For bony lesions, plain radiographs often greatly assist in diagnosis and
the determination of location, size, and local extent of involvement.

For soft-tissue masses, size often can be estimated, any bone involvement
can be seen, and intralesional content (matrix) can sometimes be
determined.

Computed tomography (CT) scans

For sarcomas arising in bone, CT scanning is used to delineate bone

involvement, bone destruction, or bone reaction. The density of
fibrosarcomas is similar to that of surrounding normal muscle.

Signs of fracture or impending fracture may be seen, and the tumor can be
more accurately localized.

CT scanning of the chest may be appropriate. CT scanning is very

sensitive for metastatic disease.

Magnetic resonance imaging (MRI) scans

o

MRI may be the best modality overall for examining soft-tissue masses
and for detecting the intraosseous and extraosseous extent of many bony
sarcomas.[4]

MRI is useful in providing information about the local extent, lesion size,
and involvement of the neurovascular structures. Fibrosarcoma of bone
typically has extraosseous extension.

Bone scans
o

Bone scanning using technetium-99m is a very useful adjunct in the

evaluation of tumor stage.

Bone scanning aids in the detection of bone metastatic or polyostotic

disease.

For fibrosarcoma, bone scanning has been mostly supplanted by MRI. The
limitation with bone scanning is that it often is nonspecific.

Other
o

Some authors have suggested the use of gallium and ultrasound scans for
diagnosis. To date, the value of these tests for staging of sarcomas remains
limited.

Diagnostic Procedures

Biopsy
o

Ultimately, the diagnosis of fibrosarcoma is made with tissue obtained

from a biopsy. Biopsy should be thought of as the first step toward
treatment, rather than the last step in diagnosis. Biopsy should always
follow a full radiographic workup.

Biopsy is best performed by the treating surgeon because that physician

will be responsible for any final tumor resection and reconstruction.

Biopsy is best performed at a center where a team approach is used in

treating these rare tumors. At such centers, groups of oncologists,
pathologists, radiologists, and surgeons, all with a specific interest in these
problems, often are present. This broad pool of experience contributes
greatly to the interpretation of tests and to the ultimate treatment outcome.

Any biopsy performed must include an adequate volume of tissue. In

centers with expert interpretation, core-needle biopsy or fine-needle
aspiration may be acceptable.

The biopsy must be performed in a way that avoids compromising any

planned surgical excision or reconstruction. It must not contaminate
significant neurovascular structures.

Histologic Findings
Fibrosarcomas are tumors of malignant fibroblasts and collagen. They vary in histologic
grade.
Well-differentiated forms have multiple plump fibroblasts with deeply staining nuclei in a
rich collagen background. Intermediate-grade tumors have the typical herringbone
pattern, showing the diagnostic parallel sheets of cells arranged in intertwining whorls. A
slight degree of cellular pleomorphism exists.
High-grade lesions are very cellular, with marked cellular atypia and mitotic activity. The
matrix is sparse. No malignant osteoid formation should be present. Higher grades are
extremely anaplastic and pleomorphic, with bizarre nuclei that bring to mind the
histologic features of malignant fibrous histiocytoma. In fact, some pathologists believe
that the division between malignant fibrous histiocytoma, high-grade osteosarcoma, and
fibrosarcoma may be artificial.

Staging
Several staging systems are used for tumors of the musculoskeletal system. The 2 most
common systems are those of the Musculoskeletal Tumor Society and of the American
Joint Committee on Cancer. Both systems include histologic grade, tumor site, and
presence or absence of metastasis. Other factors that may be important in staging are the
size and depth of the tumor.

Medical Therapy
Adjunctive therapy, such as radiation treatment and chemotherapy, can improve local
control and may make the appearance of clinically evident metastatic disease less likely.
The use of chemotherapy is controversial, but chemotherapy is generally used in bone
lesions. Radiation therapy is used in conjunction with surgery for soft-tissue
fibrosarcomas, with or without chemotherapy.

Surgical Therapy
In general terms, treatment of fibrosarcoma involves a combination of adequate local
tumor control and avoidance or treatment of distant disease. Many factors are involved
and contribute to the ultimate prognosis. To obtain local control, surgical resection with a
cuff of normal tissue (wide margins) and reconstruction of the subsequent defect are
necessary.

Follow-up
As with all sarcomas of the musculoskeletal system, successful treatment of fibrosarcoma
must be accompanied by an organized plan for clinical follow-up. This often involves a
schedule of repeat examinations and diagnostic studies. Patients often are monitored for a
minimum of 5 years.
At preset intervals, the patient is reexamined, and plain radiographs of the involved site
are obtained. Repeat staging studies of the local area and of the chest also are performed.

Complications
Local recurrence may occur in up to 60% of cases and is the reason that postoperative
radiation, preoperative radiation, or both are often recommended. Local recurrence is
reduced to about 25% when postoperative irradiation is used.

Outcome and Prognosis

If all grades are included, primary fibrosarcoma of the bone has a worse prognosis than
osteosarcoma, with a 5-year survival rate of 65%. In high-grade primary fibrosarcoma,
the 10-year survival rate is less than 30%. Secondary fibrosarcoma is associated with a
very poor outcome, the survival rate at 10 years being less than 10%.
For congenital fibrosarcoma of bone in children, the prognosis (which is related to age
and to time to diagnosis) is much better, with the disease having long-term survival rates
of higher than 50%.
Soft-tissue fibrosarcoma is associated with a 40-60% survival rate at 5 years. The
infantile form has an even better 5-year survival rate, in excess of 80%.

Future and Controversies

Continued advances in the molecular biology of sarcomas may further elucidate the very
distinct clinical behavior of the various types of fibrosarcoma and ultimately provide
better solutions to their respective treatment.