Fibrosarcoma Treatment & Management

  • Author: Ian D Dickey, MD, FRCSC; Chief Editor: Harris Gellman, MD   more...
 
Updated: Jan 20, 2010
 

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.

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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.

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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.

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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.

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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%.

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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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Contributor Information and Disclosures
Author

Ian D Dickey, MD, FRCSC  Adjunct Professor, Department of Chemical and Biological Engineering, University of Maine; Consulting Staff, Adult Reconstruction, Orthopedic Oncology, Department of Orthopedics, Eastern Maine Medical Center

Ian D Dickey, MD, FRCSC is a member of the following medical societies: American Academy of Orthopaedic Surgeons, British Columbia Medical Association, Canadian Medical Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Stryker Orthopaedics Consulting fee Consulting; Cadence Honoraria Speaking and teaching

Coauthor(s)

James Floyd, MD  Consulting Staff, Section of Orthopedic Surgery, Manatee Memorial Hospital

James Floyd, MD is a member of the following medical societies: Florida Medical Association, Florida Orthopaedic Society, and National Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Howard A Chansky, MD  Associate Professor, Department of Orthopedics and Sports Medicine, University of Washington Medical Center

Howard A Chansky, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Sean P Scully, MD, PhD  Professor, Department of Orthopedics, University of Miami

Sean P Scully, MD, PhD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, International Society on Thrombosis and Haemostasis, and Society of Surgical Oncology

Disclosure: Nothing to disclose.

Dinesh Patel, MD, FACS  Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital

Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD  Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami, Leonard M Miller School of Medicine

Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, and Arkansas Medical Society

Disclosure: Nothing to disclose.

References

  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 bone—a 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 tibia—a 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 children—a rare tumour with long-term survival even with advanced disease—a 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. Sangüeza 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].

 
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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.
 
 
 
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