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Fibrosarkoma merupakan tumor yang

berasal dari sel mesenkim yang terdiri atas


fibroblast malignan pada kolagen. Kondisi
ini dapat terjadi sebagai masa pada jaringan
lunak atau sebagai tumor tulang primer dan
sekunder. Dua jenis utama dari fibrosarkoma
tulah adalah tipe primer maupun sekunder.
Fibrosarkoma dapat berbentuk sentral
(timbul pada kanalis medulla) atau perifer
(muncul pertama kali dari periosteum).
Fibrosarkoma sekunder pada tulang berasalk
dari lesi yang pernah terjadi sebelumnya
atau setelah dilakukannya radioterapi
terhadap jaringan lunak dan tulang. Bentuk
ini lebih agresif dan memiliki prognosis
yang lebih buruk.

History of the Procedure
Tatalaksana fibrosarkoma adalah dengan
eksisi

Frequency
Fibrosarkoma lebih sering terjadi pda laki-
laki dibandingkan perempuan. Fibrosarkoma
pada tulang dapat terjadi pada seluruh usia,
tetapi biasanya terjadi pada usia dekade
keempat. Biasanya terjadi pada ektrimitas
bawah, khususnya femur dan tibia.
Firbrosarkoma pada adanak (usia <10 tahun)
memiliki prognosis lebih baik, bahkan jika
terjadi metastasis, jika diterapi dengan
kombinasi neoadjuvant dan adjuvant
kemoterapi dan reseksi.
[1, 2, 3]


Etiology
Etiologi tidak jelas. Dikaitkan dengan
adanya mutasi genetic. Adanya
kemungkinan kehilangan alel pada gen,
mutasi, translokasi kromosom.

Pathophysiology
Tidak ada penyebab definitive fibrosarkoma
yang diketahui.



Presentation
Sarkoma menyebabkan nyeri dan bengkak
setelah terjadi durasi yang lama. Dapat
mengganggu integritas dari tulang dan
menyebabkan fraktur. Umumnya, lesi
melibatkan korteks pada tulang,
berukuran>2cm, atau melibatkan medial
calcar dari femur.
Riwayat infark tulang, irradiasi, atau risiko
untuk terjadinya fibrosarkoma sekunder.
Sarcoma pada jaringan lunak tidak
menimbulkan rasa nyeri. Lesi terjadi dari
fascia muskulus, sehingga tumor lebih besar.
Kebanyakan lesi terjadi sekitar dengkul,dan
femur proksimal, dan daerah hip, atau pada
proksimal dari lengan. Adanya perubahan
neurologis atau vascular merupakan temuan
akhir dari penyakit dan menandakan adanya
proses perkembangan penyakit.

Differential diagnoses
Fibrous dysplasia
Fibrous histiocytoma
Osteosarcoma
Paget sarcoma
Malignant fibrous histiocytoma
Malignant neurosarcoma

Imaging Studies
Plain radiographs
Computed tomography (CT) scans
Magnetic resonance imaging (MRI) scans
Bone scans

Diagnostic Procedures
Biopsy

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

Surgical Therapy
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.

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