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Fibrosarkoma adalah tumor yang berasal dari sel mesenkim yang terdiri dari malignant fibroblasts
dalam matriks kolagen. Tumor ini dapat tumbuh pada soft tissue atau sebagai tumor primer atau sekunder
pada tulang. Dahulu, fibrosarkoma lebih sering didiagnosa dari pada saat ini, karena saat ini telah dapat
dilakukan pemeriksaan histologis yang dapat membedakan tumor-tumor yang tampak mirip dengan
fibrosarkoma, misalnya tumor desmoid, malignant fibrous histiocytoma, malignant schwannoma, dan highgrade osteosarcoma.
Dua tipe utama dari fibrosarkoma pada tulang adalah primer dan sekunder. Fibrosarkoma primer
adalah keganasan fibroblastik yang memproduksi kolagen dengan jumlah bervariasi. Tipe primer ini bisa
berasal dari sentral (dari medullary canal) atau perifer (dari periosteum). Fibrosarkoma sekunder berasal dari
lesi yang sudah ada sebelumnya atau dari paparan radioterapi pada tulang atau soft tissue. Tumor ini lebih
agresif dan memiliki prognosis yang lebih buruk.

Etiologi
Fibrosarkoma, sama halnya dengan soft tissue sarkoma lainnya, tidak memiliki penyebab pasti. Penelitian
terbaru mengindikasikan bahwa kebanyakan sarkoma berasal dari mutasi genetik.

Presentasi klinis
Sarkoma pada tulang biasanya muncul dengan nyeri dan pembengkakan. Bisa tumbuh besar dan dapat
merusak integritas struktur tulang dan menyebabkan fraktur patologis sebagai tanda awal.
Secara general, lesi yang melibatkan lebih dari 50% korteks tulang, ukuran lebih dari 2 cm, atau yang
mengenai medial calcar dari femur memiliki resiko terbesar untuk patah. Riwayat bone infarct, radiasi, atau
faktor resiko lainnya harus diperhatikan oleh dokter sebagai kemungkinan untuk terjadinya fibrosarkoma
sekunder.
Soft tissue sarkoma lebih sering muncul sebagai massa yang tidak nyeri. Biasanya muncul di dalam
fascia, sehingga sebelum terdiagnosis, tumor ini telah tumbuh besar di dalam.
Kebanyakan lesi muncul di sekitar lutut, proximal femur dan panggul, atau di proximal lengan. Bisa
bervariasi dan nonspesifik (fixed, firm mass, atau localized area of tenderness). Perubahan neurologis atau
vaskuler merupakan tanda extensive disease.
DD : fibrous dysplasia, fibrous hitiocytoma, osteosarcoma, paget sarcoma, malignant fibrous
histiocytoma, malignant neurosarcoma

Imaging Studies
Plain radiography
Plain radiographs of the involved anatomic region are needed to evaluate for primary or secondary
involvement of bone. (See the image below.) Typically, an osteolytic area of destruction with a permeative
or moth-eaten appearance is present. Little periosteal reaction or reactive sclerosis is depicted.

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.

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
For sarcomas arising in bone, computed tomography (CT) 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 of
the chest may be appropriate. CT is highly sensitive for metastatic disease.
Magnetic resonance imaging
Magnetic resonance imaging (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] It is useful in providing
information about the local extent, lesion size, and involvement of the neurovascular structures.
Fibrosarcoma of bone typically has extraosseous extension.
Canale et al performed a retrospective review of MRI features in six cases of infantile fibrosarcoma (patient
age range, 0-6 months).[2] A well-circumscribed single mass was the most common finding (five 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 three patients, the internal signal was homogeneous; in the other three, it was heterogeneous.[2] An
intense enhancement was seen in the three contrast-enhanced images that were available: heterogeneous
in two and homogeneous in one. There was osseous erosion observed in the patient with distant metastasis.
The tumors in all cases disappeared with chemotherapy and limited surgery.[2]
Bone scanning
Bone scanning with technetium-99m is a very useful adjunct in the evaluation of tumor stage.
It aids in the detection of bone metastatic or polyostotic disease. For fibrosarcoma, bone scanning has
largely been supplanted by MRI. The main limitation of bone scanning is that it often is nonspecific.

Other modalities
Some authors have suggested the use of gallium and ultrasound scans for diagnosis. At present, the value of
these tests for staging of sarcomas remains limited.
Diagnostic Procedures
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. In addition, it 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 (see the image below). A slight degree of cellular
pleomorphism exists.

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.

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.

Wojcik et al assessed clinicopathologic and immunohistochemical features of primary sclerosing epithelioid


fibrosarcoma (SEF) in eight patients (median age, 52 years; range, 25-73 years).[5] Tumors mostly involved
long bones of the extremities, were predominantly lytic, and were poorly marginated. Histologically, six
tumors had pure SEF morphology; two had hybrid SEF/low-grade fibromyxoid sarcoma morphology; one
showed focal dystrophic mineralization (limited to areas of necrosis); and none showed the lacelike
mineralization pattern typical of osteosarcoma.
The majority of the tumors (6/8) strongly expressed MUC4.[5] All but one patient tested negative for SATB2;
in that case, variable weak to moderate staining occurred in approximately 50% of nuclei. The authors
concluded that the combination of morphology, MUC4 expression, and the absence of SATB2 expression
was highly useful in helping to establish the correct diagnosis.
Staging
Several staging systems are used for tumors of the musculoskeletal system. The two most common
systems are that of the Musculoskeletal Tumor Society and that 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.
Laboratory Studies
Laboratory studies generally are not helpful during the initial evaluation.

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 employed 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.
Infantile fibrosarcoma
Sulkowski et al conducted a retrospective, nonrandomized study aimed at defining the extent of surgical
resection needed in the treatment of infantile fibrosarcoma, as well as the role of chemotherapy in
management.[6] The study cohort consisted of 224 patients aged 0-2 years. Of the 64 patients (28.6%) with
positive margins, 36 (56.3%) had microscopic disease, and 12 (18.8%) had macroscopic disease; margin
status was unknown for 16 (25%). None of the patients had metastases. In all, 171 (76.4%) were treated with
surgical resection.
The disease-free survival rate was 90.6%.[6] No significant survival difference was noted with regard to
margin status, nodal involvement, tumor size, or treatment modality. The use of multimodal therapy (surgery
in conjunction with chemotherapy) increased over time. A small increase in survival was associated with
negative margins and multimodal therapy, but neither result was statistically significant. The authors
suggested that future studies investigating tumor biology and chemosensitivity may determine optimal
management of infantile fibrosarcoma.
Follow-up
With fibrosarcoma, as with all sarcomas of the musculoskeletal system, successful treatment 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%.
Russell et al reported on four patients with infantile fibrosarcoma treated with chemotherapy and surgical
resection, all of whom had excellent functional outcome.[1] The patient with fibrosarcoma of the neck
displayed rapid tumor shrinkage. Two of the lower-extremity tumors had only modest changes in dimensions,
but on 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.
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.