Fibrosarcoma Treatment & Management

Updated: Aug 22, 2016
  • Author: Ian D Dickey, MD, FRCSC; Chief Editor: Harris Gellman, MD  more...
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Treatment

Approach Considerations

To obtain local control, surgical resection with a cuff of normal tissue (wide margins) and reconstruction of the subsequent defect are necessary.

Surgical treatment (including biopsy) of fibrosarcoma should not proceed unless complete patient care is available. Complete care includes biopsy and interpretation of biopsy findings, access to oncologists and radiation oncologists, and definitive resection.

Fibrosarcomas should be removed by trained orthopedic oncologists who can provide a state-of-the-art treatment program; this would involve a team of well-trained specialists with advanced experience in treating these tumors.

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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.

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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.

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 (IFS), as well as the role of chemotherapy in management. [13] 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%. [13] 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 IFS.

The European Paediatric Soft Tissue Sarcoma Study Group evaluated a conservative therapeutic strategy in 50 infants (median age, 1.4 months) with localized IFS. [14] Initial surgery was suggested only if it could be done without mutilation. No further therapy was administered to those with initial complete (group I/R0; n=11) or microscopic incomplete (group II/R1; n=8) resection; vincristine-actinomycin (VA) chemotherapy was administered to those with initial inoperable tumors (group III/R2; n=31), with delayed conservative surgery planned after tumor reduction.

After delivery of VA chemotherapy to 25 children with group III/R2 and one with group II/R1 disease, there was a 68% rate of response to VA; only three children required mutilating surgery. [14] The 3-year event-free survival was 84% and the overall survival 94.0% at a median follow-up of 4.7 years (range, 1.9-9.0 years). Alkylating or anthracycline based chemotherapy was avoided in 71.0% of patients needing chemotherapy.

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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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Long-Term Monitoring

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.

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