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.

1. Russell H, Hicks MJ, Bertuch AA,
Chintagumpala M. Infantile
fibrosarcoma: clinical and histologic
responses to cytotoxic
chemotherapy. Pediatr Blood
Cancer. Jul 2009;53(1):23-
7. [Medline].
2. Canale S, Vanel D, Couanet D, Patte
C, Caramella C, Dromain C.
Infantile fibrosarcoma: magnetic
resonance imaging findings in six
cases. Eur J Radiol. Oct
2009;72(1):30-7. [Medline].
3. DeComas AM, Heinrich SD, Craver
R. Infantile fibrosarcoma
successfully treated with
chemotherapy, with occurrence of
calcifying aponeurotic fibroma and
pleomorphic/spindled celled lipoma
at the site 12 years later. J Pediatr
Hematol Oncol. Jun 2009;31(6):448-
52. [Medline].
4. Stein-Wexler R. MR imaging of soft
tissue masses in children. Magn
Reson Imaging Clin N Am. Aug
2009;17(3):489-507, vi. [Medline].
5. Abbott JJ, Erickson-Johnson M,
Wang X. Gains of COL1A1-PDGFB
genomic copies occur in
fibrosarcomatous transformation of
dermatofibrosarcoma
protuberans. Mod Pathol. Nov
2006;19(11):1512-8.[Medline].
6. Antonescu CR, Erlandson RA,
Huvos AG. Primary fibrosarcoma
and malignant fibrous histiocytoma
of bonea comparative
ultrastructural study: evidence of a
spectrum of fibroblastic
differentiation. Ultrastruct Pathol.
Mar-Apr 2000;24(2):83-
91. [Medline].
7. Hadjipavlou A, Zucker J. Sarcoma in
Paget's disease of bone. In: Current
Concepts of Diagnosis and
Treatment of Bone and Soft Tissue
Tumors. Berlin:. Springer-
Verlag;1984: 383-94.
8. Hinarejos P, Escuder MC, Monllau
JC. Fibrosarcoma at the site of a
metallic fixation of the tibiaa case
report and literature review. Acta
Orthop Scand. Jun 2000;71(3):329-
32. [Medline].
9. Inwards CY, Unni KK.
Classification and grading of bone
sarcomas. Hematol Oncol Clin North
Am. Jun 1995;9(3):545-
69. [Medline].
10. Lilleng PK, Monge OR, Walloe A, et
al. Fibrosarcoma in childrena rare
tumour with long-term survival even
with advanced diseasea report of 3
cases. Acta Oncol. 1997;36(4):438-
40. [Medline].
11. Lin CN, Chou SC, Li CF. Prognostic
factors of myxofibrosarcomas:
implications of margin status, tumor
necrosis, and mitotic rate on
survival. J Surg Oncol. Mar 15
2006;93(4):294-303. [Medline].
12. Loss L, Zeitouni NC. Management
of scalp dermatofibrosarcoma
protuberans. Dermatol Surg. Nov
2005;31(11 Pt 1):1428-
33. [Medline].
13. Menon AG, Anderson KM, Riccardi
VM, et al. Chromosome 17p
deletions and p53 gene mutations
associated with the formation of
malignant neurofibrosarcomas in von
Recklinghausen
neurofibromatosis.Proc Natl Acad
Sci U S A. Jul 1990;87(14):5435-
9. [Medline]. [Full Text].
14. Nakanishi H, Tomita Y, Ohsawa M,
et al. Tumor size as a prognostic
indicator of histologic grade of soft
tissue sarcoma. J Surg Oncol. Jul
1997;65(3):183-7. [Medline].
15. Papagelopoulos PJ, Galanis E,
Frassica FJ, et al. Primary
fibrosarcoma of bone. Outcome after
primary surgical treatment. Clin
Orthop Relat Res. Apr 2000;88-
103. [Medline].
16. Peabody TD, Gibbs CP, Simon MA.
Evaluation and staging of
musculoskeletal neoplasms. J Bone
Joint Surg Am. Aug
1998;80(8):1204-18. [Medline].
17. Sangeza OP, Requena L.
Neoplasms with neural
differentiation: a review. Part II:
malignant neoplasms.Am J
Dermatopathol. Feb 1998;20(1):89-
102. [Medline].
18. Toro JR, Travis LB, Wu HJ.
Incidence patterns of soft tissue
sarcomas, regardless of primary site,
in the surveillance, epidemiology
and end results program, 1978-2001:
an analysis of 26,758 cases. Int J
Cancer. Dec 15 2006;119(12):2922-
30. [Medline].
19. Weatherby RP, Dahlin DC, Ivins JC.
Postradiation sarcoma of bone:
review of 78 Mayo Clinic
cases. Mayo Clin Proc. May
1981;56(5):294-306. [Medline].
20. Wee A, Pho RW, Ong LB. Infantile
fibrosarcoma: report of cases. Arch
Pathol Lab Med. May
1979;103(5):236-8. [Medline].
21. Willems SM, Debiec-Rychter M,
Szuhai K. Local recurrence of
myxofibrosarcoma is associated with
increase in tumour grade and
cytogenetic aberrations, suggesting a
multistep tumour progression
model.Mod Pathol. Mar
2006;19(3):407-16. [Medline].