Fibrous dysplasia is a common benign skeletal lesion that may involve one bone (monostotic) or multiple bones (polyostotic) and occurs throughout the skeleton with a predilection for the long bones, ...ribs, and craniofacial bones. The etiology of fibrous dysplasia has been linked to an activating mutation in the gene that encodes the alpha subunit of stimulatory G protein (G(s)alpha) located at 20q13.2-13.3. Most lesions are monostotic, asymptomatic, and identified incidentally and can be treated with clinical observation and patient education. Bisphosphonate therapy may help to improve function, decrease pain, and lower fracture risk in appropriately selected patients with fibrous dysplasia. Surgery is indicated for confirmatory biopsy, correction of deformity, prevention of pathologic fracture, and/or eradication of symptomatic lesions. The use of cortical grafts is preferred over cancellous grafts or bone-graft substitutes because of the superior physical qualities of remodeled cortical bone.
We studied seventy-three massive preserved human allografts, retrieved from two to 156 months after implantation, to provide insight into the mechanisms of their repair.
The specimens were studied ...with radiographic and histological techniques that permitted time-related quantitative analysis of the reparative mechanisms of union, cortical repair, soft-tissue attachment, fracture, and characteristics of the allograft-cement interface and the articular cartilage.
Union at cortical-cortical junctions occurred slowly (approximately twelve months) by host-derived external callus that bridged the junction and filled the gap between abutting cortices. The bone in the gap did not undergo stress-oriented remodeling even after many years, and, when the union was intentionally disrupted, failure occurred at the cement line that marked the allograft-host junction. Repair of the necrotic graft matrix was both external and internal. External repair consisted of the apposition of a thin seam of host bone on the outer surface of the graft, coating about 40% of the surface at one year and 80% at two years. Internal repair was confined to the ends and the periphery of the cortices and penetrated so slowly that only 15% to 20% of the graft was repaired by five years, after which deeper repair seldom occurred. Graft fractures in specimens retrieved soon after fracture showed only necrotic bone adjacent to the fracture site, whereas those retrieved after fracture-healing showed a marked increase in internal repair of the bone about the fracture site. When bone cement had been used to fix a prosthesis, there was no evidence of bone resorption or loosening of the device. The osteoarticular specimens showed no survival of chondrocytes in the articular cartilage. However, the architecture of the acellular cartilage was well preserved after two to three years and occasionally after as many as five years. Late degenerative changes in the articular cartilage coincided with subchondral revascularization and fragmentation, and the articulating surfaces became covered by a pannus of fibrovascular reparative tissue. Degenerative changes in articular cartilage occurred earlier and were more advanced in specimens retrieved from patients with an unstable joint than in those retrieved from patients with a stable joint.
Repair of massive human allografts is an indolent process that follows a fairly predictable course during the first few years and is influenced by other biological activities, such as fracture repair, supplementary autografting, and tumor recurrence.
Allograft bone is the primary source of graft material for structural oncological limb salvage procedures. Failure rates after massive allograft reconstructions have been reported as high as 60% at ...10 years, which are associated with a multitude of biologic processes influencing the graft incorporation and functional capacity. It is unknown if mechanical failure is associated with a gradual loss of bulk material properties of the bone (strength and modulus), loss of bone mineral density, osteoclastic resorption of the allograft, unrepaired allograft microfractures or microcracks, and/or local stress concentration within the tissue. Allograft material properties, bone mineral density, microcrack prevalence, and cortical porosity were quantified in 13 failed human allograft retrievals ranging in longevity from 1 to 13 years in vivo. Nonimplanted allograft tissue (n = 27) served as the baseline for comparison. A 50% loss in strength of allograft tissue was noted after 10 years in vivo. Loss of strength was correlated with an increase in microfracture prevalence and decrease in bone mineral density within the retrieved allograft cortex. This study suggests functional failure of allograft limb salvage procedures may, in part, be attributed to degradation of the tissue's material properties, bone mineral density and prevalence of microcracks.
Historically, an adequate surgical procedure has been the most effective means of treating the majority of primary musculoskeletal sarcomas, and amputation has figured prominently in the surgical ...armamentarium. 4, 7, 9, 19, 21, 29, 41 The recent evidence that certain chemotherapeutic agents may have significant anti-sarcoma activity 2, 15, 17, 38 and coincident technical advances in irradiation therapy, radiographic localization, and reconstructive surgery have fostered enthusiastic interest in extremity-saving treatments. Almost all such treatments emphasize limb salvage as an alternative to amputation and are usually performed under a protective cloak of adjunctive chemotherapy, irradiation or immunoactive agents. 20, 23, 24, 30, 37, 39 Since neither chemotherapy nor irradiation therapy alone has been shown to assure long-term local control of bulk disease, surgical intervention remains an essential step in the overall management of musculoskeletal sarcomas. 3, 9, 17, 18, 29 Questions concerning the magnitude and timing of the surgical procedure are as unanswered as those relating to the most appropriate use of the adjuncts themselves. Increasingly, the surgeon and his patient are confronted with a bewildering array of therapeutic options, the long-term outcomes of which are unknown. These relatively rare sarcomas increasingly are distributed among a variety of treatment protocols in which multiple parameters differ. This trend necessitates interinstitutional cooperation if sufficient numbers of patients are to be available for the timely evaluation of treatments in clinical use. Such cooperation and even effective interinstitutional communication are seriously hampered by the lack of uniform language, so that meaningful comparison of treatments is currently impossible. Prime factors include the lack of a consistent definition of the surgery performed and a serviceable surgical staging system encompassing bone and soft tissue. Standard terminology will assure that like and unlike treatments are appropriately compared. Although an effective staging system should serve all members of the multidisciplinary team, the biologic behavior of musculoskeletal sarcomas suggests that the most useful staging system will articulate with the surgical procedure.
The need for a standardized system of end result reporting of various surgical alternatives after limb salvaging and ablative procedures for musculoskeletal tumors was clearly recognized during the ...first International Symposium on Limb Salvage (ISOLS) in 1981. During the ensuing four biannual symposia, there has been an ongoing developmental experience with a system extensively field tested in 1989 by the Musculoskeletal Tumor Society (MSTS). This system of functional evaluation has been adopted by the MSTS and ISOLS for their joint studies and program presentation. In brief, the system assigns numerical values (0-5) for each of six categories: pain, and function and emotional acceptance in upper and lower extremities; supports, and walking and gait in the lower extremity; and hand positioning, and dexterity and lifting ability in the upper extremity. Demographic information and a patient satisfaction component is included. A numerical score and percent rating is calculated to allow for comparison of results. The system has been field tested in 220 patients with low (+/-) interobserver variability. It was well accepted by the participants, and its usage is recommended by the MSTS to facilitate valid comparative end result studies of musculoskeletal tumor reconstructions.
The management of adult soft tissue sarcomas Mendenhall, William M; Indelicato, Daniel J; Scarborough, Mark T ...
American journal of clinical oncology
32, Issue:
4
Journal Article
Peer reviewed
Soft tissue sarcomas are a relatively rare, heterogeneous group of tumors arising from mesenchymal tissues and occurring almost anywhere in the body. The rate of progression and likelihood of ...hematogenous dissemination, usually to the lung, is determined primarily by tumor grade. The likelihood of regional spread is low. Pretreatment evaluation includes computed tomography of the primary site and chest and magnetic resonance imaging of the primary tumor. The mainstay of treatment is surgery; wide excision for low-grade lesions and wide or radical (compartmental) resection for high-grade tumors. Often, these procedures cannot be achieved either because of the location and extent of the sarcoma or anticipated functional deficit. Adjuvant preoperative and/or postoperative radiotherapy improves the likelihood of local control and preserves function when adequate margins cannot be achieved with surgery alone. The role of adjuvant chemotherapy is unclear; however, some data suggest the doxorubicin containing regimens may improve the likelihood of cure for high-grade lesions, particularly large tumors arising in the extremities. Prognosis is influenced by a variety of factors, including age, tumor size, histologic grade, depth (superficial or deep), histologic subtype, and site. Approximately 90% and 98% of recurrences are observed within 5 years and 10 years, respectively. Five-year overall survival rates range from approximately 60% to 80%.
For giant cell tumors of bone, does radiotherapy provide a safe and effective treatment? This retrospective review includes 24 patients with 26 histologically diagnosed tumors treated with ...megavoltage radiotherapy between March 1972 and July 1996. Of the 10 recurrent tumors, five had an intralesional resection, two had a biopsy, and three had no biopsy before radiotherapy. Of the 16 previously untreated tumors, one was irradiated after a marginal resection, five after an intracapsular resection, and 10 after biopsy alone. The total doses ranged from 35 to 55 Gy (median, 43 Gy) in fractions of 1.67 to 2.33 Gy per day. Twenty of 26 tumors (77%) were controlled locally. All of the local recurrences occurred within the irradiated field. Five of six patients with local recurrence were treated successfully with additional surgery. Salvage surgery after local recurrence required amputation of an extremity in three patients and a total knee replacement in one patient. The ultimate local control rate was 96% with one patient alive with progressive disease. Lung metastases in one patient were treated successfully with surgery, chemotherapy, and radiotherapy. In one patient a radiation-induced sarcoma developed 22 years after treatment. The authors conclude that radiation therapy is a safe and effective treatment option for benign giant cell tumors of bone. A total dose greater than 40 Gy is the only variable found to significantly influence local control.
Background
Complications are frequent with osteoarticular allografts, and their long-term survivorship in the distal femur is unclear. Thus, the benefits of osteoarticular allografting remain ...controversial.
Questions/purposes
We therefore determined the frequency of complications in osteoarticular allografts of the distal femur relative to their potential long-term survival.
Methods
We retrospectively reviewed 26 patients who had osteoarticular allograft reconstruction of the distal femur after resection of a malignant or aggressive benign tumor of bone. The minimum followup was 15 months (average, 156 months; range, 15–283 months) for all patients and 98 months (average, 191 months; range, 98–283 months) for the surviving patients.
Results
At last followup, 16 of the 26 original allografts were still in place. The overall 5-year and 10-year allograft survival rates were 69% and 63%, respectively. The 5-year and 10-year survival rates of the joint surface were 79% and 65%, respectively. Eleven patients retained their original osteoarticular allograft without a resurfacing procedure, and nine had been converted to allograft-prosthetic composites. Five patients were converted to megaprostheses and one had an amputation for local recurrence. At last followup, 25 of 26 patients retained a functional limb.
Conclusions
Osteoarticular allograft reconstructions of the distal femur can provide long term survival and restore function but the risk of complications and their physical and monetary costs for patients are not trivial. Lacking the benefit of improved soft tissue attachments inherent in other anatomic sites, we believe this option is most appropriate for restoring bone stock in young patients with expectations of long-term survival.
Level of Evidence
Level IV, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.
Purpose
: To review a large single-institution experience in the management of aggressive fibromatosis to determine the effectiveness of external beam radiotherapy (EBRT) and identify the ...presentation and treatment variables predictive of locoregional control.
Methods and Materials
: Between 1975 and 2000, 72 patients were treated with EBRT for a pathologically confirmed diagnosis of aggressive fibromatosis. Thirty patients were treated at the primary presentation and 42 at the time of a locoregional recurrence. Minimal 2-year follow-up data were available for 65 patients (median 6 years). Megavoltage irradiation with
60Co to 20 MV photons or electron therapy was used for all patients. Most patients were treated after attempted complete surgical resection; 16 patients underwent pretreatment biopsy alone. The prescribed treatment was standard (1.8 Gy) daily fractions in 42 cases and 1.2 Gy fractions b.i.d. in 23 cases. The median prescribed dose was 54 Gy. The prognostic variables and treatment results were evaluated by Kaplan-Meier actuarial analysis.
Results
: Locoregional control was achieved in 52 of 65 patients. The 5-year actuarial locoregional control was 83%. Locoregional failure occurred in 13 patients (11 in patients with recurrent tumors). Only two failures occurred within the irradiation fields; nine failures occurred at the field margins. Eleven patients were salvaged by surgery: wide excision in nine and amputation in two. The only prognostic factor significant for locoregional control was primary vs. recurrent presentation (
p = 0.0193). The 5-year locoregional control rates for irradiation at initial presentation and at recurrence were 96% and 75%, respectively. The variables without significance for locoregional control included primary tumor location, surgical procedures performed, resection margins, and gross vs. microscopic residual tumor at irradiation. Lymphedema was the most common late effect, occurring in 7 patients, 5 with prior treatment. Bone fracture occurred in 3 patients; all 3 had fibromatosis involving the bone at presentation but without recurrence at the time of fracture.
Conclusion
: EBRT is effective treatment for aggressive fibromatosis. The probability of locoregional control decreases with multiple prior recurrences.
Orthopedic oncology in the United States has its roots in European medicine of the 1800s in which sarcomas were first classified on the basis of their gross characteristics (1804) and amended on the ...basis of their histologic features (1867). Surgical management, local excision, with unacceptable mortality gave way to amputation in the 1870s and remained so, until limb-sparing resection was cautiously embarked upon in the mid 1900s. Nonsurgical adjuvant was first devised in the 1880s (as Coley's toxins) but remained largely ineffective until the advent of chemotherapy in the 1970s. The combination of these in the last 30 years, together with vastly improved staging and reconstructive techniques has led to the current preponderance of limb-salvaging surgery and greatly improved survival rates. Their application has been greatly enhanced by the development of Orthopedic oncology fellowships, formation of Orthopedic oncology societies, and the institution of federally funded regional cancer centers with the formation of multidisciplinary sarcoma treatment teams.
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