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Fibrosarcoma |
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Types
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Treatment
Plain radiographs Plain radiographs of the involved anatomic region are needed to evaluate for primary or secondary involvement of bone. Typically an osteolytic area of destruction with a permeative or moth-eaten appearance is present. Little periosteal reaction or reactive sclerosis is depicted. For bony lesions, plain radiographs often greatly assist with determining diagnosis, location, size, and local extent of involvement. For soft tissue masses, size often can be estimated, any bone involvement can be seen, and intralesional content (matrix) can sometimes be determined. CT scan For sarcomas arising in bone, the use of CT scan is very helpful. It delineates bone involvement, bone destruction, or bone reaction. Density of fibrosarcoma is similar to that of surrounding normal muscle. Signs of fracture or impending fracture may be seen, and the tumor can be more accurately localized. CT scanning of the chest may be appropriate. CT scan is very sensitive for metastatic disease. MRI MRI may be the best overall study for soft tissue masses and for detecting the intraosseous and extraosseous extent of many bony sarcomas. MRI is useful in providing information about the local extent, lesion size, and involvement of the neurovascular structures. Fibrosarcoma of bone typically has extraosseous extension. Bone scanning Bone scanning using technetium Tc 99m is a very useful adjunct in the evaluation of tumor stage. It helps to detect bone metastatic disease or polyostotic disease. For fibrosarcoma, it has been mostly supplanted by MRI. The limitation with bone scanning is that it often is nonspecific. Some authors have suggested the use of gallium scans and ultrasound for diagnosis. To date, the value of these tests for staging of sarcomas remains limited. Diagnostic Procedures: Biopsy Ultimately, the diagnosis of fibrosarcoma is made with tissue obtained from a biopsy. Biopsy should be thought of as the first step toward treatment, rather than the last step in diagnosis. Biopsy should always follow a full radiographic workup. Biopsy is best performed by the treating surgeon because that physician will be responsible for any final tumor resection and reconstruction. Biopsy is best performed at a center where a team approach is used in treating these rare tumors. At these centers, groups of oncologists, pathologists, radiologists, and surgeons, all with a specific interest in these problems, are often present. This broad pool of experience contributes greatly to the interpretation of tests and to the ultimate treatment outcome. Any biopsy performed must include an adequate volume of tissue. In centers with expert interpretation, core needle biopsy or fine-needle aspiration may be acceptable. The biopsy must be performed in such a way as to avoid compromising any planned surgical excision or reconstruction. It must not contaminate significant neurovascular structures. Histologic Findings: Fibrosarcomas are tumors of malignant fibroblasts and collagen. They vary in histologic grade. Well-differentiated forms have multiple plump fibroblasts with deeply staining nuclei in a rich collagen background. Intermediate grade tumors have the typical herringbone pattern showing the diagnostic parallel sheets of cells arranged in intertwining whorls. A slight degree of cellular pleomorphism exists. High-grade lesions are very cellular with marked cellular atypia and mitotic activity. The matrix is sparse. No malignant osteoid formation should be present. Higher grades are extremely anaplastic and pleomorphic with bizarre nuclei that bring to mind the histologic features of malignant fibrous histiocytoma. In fact, some pathologists believe that the division between malignant fibrous histiocytoma, high-grade osteosarcoma, and fibrosarcoma may be artificial. Staging: Several staging systems are in use for tumors of the musculoskeletal system. The two most common systems are those of the Musculoskeletal Tumor Society and of the American Joint Committee on Cancer. Both systems include histologic grade, tumor site, and presence or absence of metastasis. Other factors that may be important in staging are the size and depth of the tumor. TREATMENT Section 6 of 11 Author Information Introduction Indications Relevant Anatomy And Contraindications Workup Treatment Complications Outcome And Prognosis Future And Controversies Pictures Bibliography
Medical therapy: Adjunctive therapy, such as radiation 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 additional chemotherapy. Surgical therapy: In general terms, treatment of fibrosarcoma involves the 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. Follow-up care: 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 are also performed. COMPLICATIONS Section 7 of 11 Author Information Introduction Indications Relevant Anatomy And Contraindications Workup Treatment Complications Outcome And Prognosis Future And Controversies Pictures Bibliography
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 Section 8 of 11 Author Information Introduction Indications Relevant Anatomy And Contraindications Workup Treatment Complications Outcome And Prognosis Future And Controversies Pictures Bibliography
If all grades are included, primary fibrosarcoma of the bone has a worse prognosis than osteosarcoma, with a 5-year survival rate of 65%. Specifically, in high-grade primary fibrosarcoma, the 10-year survival rate is less than 30%. Secondary fibrosarcoma is associated with a very poor outcome, with a less than 10% survival rate at 10 years. Congenital fibrosarcoma of bone in children has a much better prognosis, related to age and time to diagnosis, with long-term survival rates higher than 50%. Soft tissue fibrosarcoma is associated with a 40-60% survival rate at 5 years. Again, the infantile form has an even better 5-year survival rate, in excess of 80%. FUTURE AND CONTROVERSIES Section 9 of 11 Author Information Introduction Indications Relevant Anatomy And Contraindications Workup Treatment Complications Outcome And Prognosis Future And Controversies Pictures Bibliography
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. PICTURES Section 10 of 11 Author Information Introduction Indications Relevant Anatomy And Contraindications Workup Treatment Complications Outcome And Prognosis Future And Controversies Pictures Bibliography
Caption: Picture 1. Though fibrosarcoma of bone can arise anywhere, it is found most commonly about the knee and femur. The radiograph depicted here shows a typical appearance of a lesion in bone. View Full Size Image eMedicine Zoom View (Interactive!) Picture Type: X-RAY Caption: Picture 2. 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. View Full Size Image eMedicine Zoom View (Interactive!) Picture Type: Photo BIBLIOGRAPHY Section 11 of 11 Author Information Introduction Indications Relevant Anatomy And Contraindications Workup Treatment Complications Outcome And Prognosis Future And Controversies Pictures Bibliography
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 2000 Mar-Apr; 24(2): 83-91[Medline]. 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. Hinarejos P, Escuder MC, Monllau JC, et al: Fibrosarcoma at the site of a metallic fixation of the tibia--a case report and literature review. Acta Orthop Scand 2000 Jun; 71(3): 329-32[Medline]. Inwards CY, Unni KK: Classification and grading of bone sarcomas. Hematol Oncol Clin North Am 1995 Jun; 9(3): 545-69[Medline]. 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]. 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 1990 Jul; 87(14): 5435-9[Medline]. Nakanishi H, Tomita Y, Ohsawa M, et al: Tumor size as a prognostic indicator of histologic grade of soft tissue sarcoma. J Surg Oncol 1997 Jul; 65(3): 183-7[Medline]. Papagelopoulos PJ, Galanis E, Frassica FJ, et al: Primary fibrosarcoma of bone. Outcome after primary surgical treatment. Clin Orthop 2000 Apr; (373): 88-103[Medline]. Peabody TD, Gibbs CP Jr, Simon MA: Evaluation and staging of musculoskeletal neoplasms. J Bone Joint Surg Am 1998 Aug; 80(8): 1204-18[Medline]. Sangueza OP, Requena L: Neoplasms with neural differentiation: a review. Part II: Malignant neoplasms. Am J Dermatopathol 1998 Feb; 20(1): 89-102[Medline]. Weatherby RP, Dahlin DC, Ivins JC: Postradiation sarcoma of bone: review of 78 Mayo Clinic cases. Mayo Clin Proc 1981 May; 56(5): 294-306[Medline]. Wee A, Pho RW, Ong LB: Infantile fibrosarcoma: report of cases. Arch Pathol Lab Med 1979 May; 103(5): 236-8[Medline]. NOTE: Medicine is a constantly changing science and not all therapies are clearly established. New research changes drug and treatment therapies daily. The authors, editors, and publisher of this journal have used their best efforts to provide information that is up-to-date and accurate and is generally accepted within medical standards at the time of publication. However, as medical science is constantly changing and human error is always possible, the authors, editors, and publisher or any other party involved with the publication of this article do not warrant the information in this article is accurate or complete, nor are they responsible for omissions or errors in the article or for the results of using this information. The reader should confirm the information in this article from other sources prior to use. In particular, all drug doses, indications, and contraindications should be confirmed in the package insert. FULL DISCLAIMER
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