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 ProcedureTatalaksana fibrosarkoma adalah dengan eksisi

FrequencyFibrosarkoma 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 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 TherapyAdjunctive 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 TherapyComplicationsLocal 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 PrognosisIf 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 ControversiesContinued 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].