Stefano Boriani

Giant cell tumor of bone.

True giant-cell tumors are quite rare. Solitary bone cysts occur with about the same frequency, while sarcomata of bone are found five times as often as either. We agree with Geschickter and Copeland that. there is a close clinical kinship between giant-cell tumor and solitary bone cyst., and tlsat they probably have a common origin. Giant-cell tumors are neoplasms and as such can be expected to recur in sites of incomplete extirpation. Moreover, they may undergo malignant changes, either through trauma or as a result of inadequate treatment. The number and size of the giant cells, together with the number of nuclei pet cell, furnish the best criterion for determining the prognosis. The small, sparsely nucleated giant cells with a tendency for collection of many cells in each high-powered field indicate more activity and rapid growth. These tumors have a greater tendency to recur or become malignant. X-irradiation should be reserved for those lesions inaccessible to surgery. The danger of incomplete cures, recurrence, and of an overdosage of roentgen rays are not as important as the hazard of late sarcomatous changes. Resection of the involved portion of a bone where feasible is the treatment of choice. The distal portion of the radius can be sipplanted successfully by use Proximal end of the fibula. The femoral condyle can be replaced by a section from the ilium. If resection is not practical, the next best method of treatment is thorough curettage, with or without cauterization, together with multiple bone grafts or chips to fill the cavity. The most important single factor in this method applies to the treatment of solitary bone cyst as well as giant-cell tumor: this is thorough curettage, breaking through and destroying the limiting wall and thus opening into the normal cancellous bone of the shaft. Bone chips or grafts should he inserted when large defects are created. The so-called giant-cell variants in most instances are probably separate and distinct pathological lesions resembling this tumor only through the presence of giant cells.Of 327 patients who had a giant-cell tumor of bone and were seen at the Istituto Rizzoli, 293 were treated at the Institute, and 280 of these were followed for two to forty-four years. The distribution according to sex and age of the patient and site of the tumor was similar to the distributions in major reports of large series. The tumor usually involved the metaphysis and the epiphysis, but was occasionally limited to the metaphysis, and in only 2 per cent of the patients was it adjacent to an open growth plate. The tumor on occasion invaded the articular space, also involving the ligaments and the synovial membrane. Extension to an adjacent bone through the joint occurred in 5 per cent of the tumors. Our radiographic grading, which is roughly comparable with the staging system of Enneking et al., was Grade I in 4 per cent, II in 74 per cent, and III in 22 per cent of 266 patients before treatment. A pathological fracture was apparent on the first radiograph in 9 per cent of the patients. In the 280 patients with adequate follow-up, 331 surgical procedures were performed. The rate of local recurrence was 27 per cent in the 151 intralesional procedures, 8 per cent in the 122 marginal excisions, and zero in the fifty-eight wide or radical procedures. These results did not correlate with the radiographic grade of the lesion. Of the fifty-one local recurrences that were seen after treatment at our institution, 90 per cent appeared in the first three years after surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

Primary bone tumors of the spine. Terminology and surgical staging.

Appropriate application of an oncologic staging system is required to evaluate the relationship among histologic types, management, and outcome of primary bone tumors. A commonly accepted terminology for surgical procedures and for definition of tumor extent is needed for surgical planning and clinical reviews. The principles of the Enneking system for classifying stages of tumors are emphasized and applied to the spine using a practical approach for surgical staging.

A novel classification system for spinal instability in neoplastic disease: an evidence-based approach and expert consensus from the Spine Oncology Study Group.

Study Design. Systematic review and modified Delphi technique. Objective. To use an evidence-based medicine process using the best available literature and expert opinion consensus to develop a comprehensive classification system to diagnose neoplastic spinal instability. Summary of Background Data. Spinal instability is poorly defined in the literature and presently there is a lack of guidelines available to aid in defining the degree of spinal instability in the setting of neoplastic spinal disease. The concept of spinal instability remains important in the clinical decision-making process for patients with spine tumors. Methods. We have integrated the evidence provided by systematic reviews through a modified Delphi technique to generate a consensus of best evidence and expert opinion to develop a classification system to define neoplastic spinal instability. Results. A comprehensive classification system based on patient symptoms and radiographic criteria of the spine was developed to aid in predicting spine stability of neoplastic lesions. The classification system includes global spinal location of the tumor, type and presence of pain, bone lesion quality, spinal alignment, extent of vertebral body collapse, and posterolateral spinal element involvement. Qualitative scores were assigned based on relative importance of particular factors gleaned from the literature and refined by expert consensus. Conclusion. The Spine Instability Neoplastic Score is a comprehensive classification system with content validity that can guide clinicians in identifying when patients with neoplastic disease of the spine may benefit from surgical consultation. It can also aid surgeons in assessing the key components of spinal instability due to neoplasia and may become a prognostic tool for surgical decision-making when put in context with other key elements such as neurologic symptoms, extent of disease, prognosis, patient health factors, oncologic subtype, and radiosensitivity of the tumor.

Chordoma of the Mobile Spine : Fifty Years of Experience

Study Design. A consecutive series of 52 chordomas of the mobile spine observed over a 50-year period includes a retrospective review of 15 cases treated prior to 1991 and a prospective group of 37 cases treated from 1991 to 2002. Objectives. This series reviews epidemiologic issues as well as clinical patterns of spinal chordomas. We attempt to correlate tumor extent, treatment, and outcomes over time. Summary of Background Data. Chordoma is the most frequent primary tumor of the mobile spine. Due to slow growth, both initial symptoms and recurrences after treatment arise later, making it difficult to evaluate the effectiveness of treatment protocols. Methods. A prospective series of 37 cases is compared with a retrospective group of 15 patients observed between 1954 and 1991. In the prospective study, all patients had imaging studies, and oncologic and surgical staging. When en bloc resection was not feasible, intralesional extracapsular excision was combined with radiation therapy. The prospective patients were clinically evaluated and imaged. Patients in the retrospective group were evaluated by chart and available images; of these, only one en bloc resection (intralesional margin) was performed. Survivors were all evaluated clinically and had radiographic studies. Results. Forty-eight patients were available for long-term follow-up. Four died due to post-operative complications, and six due to disease less than 2 years after treatment. Forty-two patients were followed over 2 years; 26 patients had over 5 years follow-up. All patients having radiation alone, intralesional excision, or a combination had recurrences in less than 2 years, and died in some cases after a long survival with symptomatic disease. Intralesional extracapsular excision with radiation had a high rate of recurrence (12 of 16 at average 30 months), but 3 patients are continuously disease-free (CDF) at mean 52 months and 5 are alive with disease at average 69 months (ranging 24 to 146). Twelve of 18 patients having en bloc resection are CDF at average 8 years (48 to 155 months). The remaining 6 recurred and of these 1 died. All of these (6) had been previously treated and/or had en bloc resections with contaminated margins. Conclusions. The only treatment protocol associated with CDF at follow-up longer than 5 years is margin-free en bloc resection.

Spinal Instability Neoplastic Score: An Analysis of Reliability and Validity From the Spine Oncology Study Group

PURPOSE Standardized indications for treatment of tumor-related spinal instability are hampered by the lack of a valid and reliable classification system. The objective of this study was to determine the interobserver reliability, intraobserver reliability, and predictive validity of the Spinal Instability Neoplastic Score (SINS). METHODS Clinical and radiographic data from 30 patients with spinal tumors were classified as stable, potentially unstable, and unstable by members of the Spine Oncology Study Group. The median category for each patient case (consensus opinion) was used as the gold standard for predictive validity testing. On two occasions at least 6 weeks apart, each rater also scored each patient using SINS. Each total score was converted into a three-category data field, with 0 to 6 as stable, 7 to 12 as potentially unstable, and 13 to 18 as unstable. RESULTS The κ statistics for interobserver reliability were 0.790, 0.841, 0.244, 0.456, 0.462, and 0.492 for the fields of location, pain, bone quality, alignment, vertebral body collapse, and posterolateral involvement, respectively. The κ statistics for intraobserver reliability were 0.806, 0.859, 0.528, 0.614, 0.590, and 0.662 for the same respective fields. Intraclass correlation coefficients for inter- and intraobserver reliability of total SINS score were 0.846 (95% CI, 0.773 to 0.911) and 0.886 (95% CI, 0.868 to 0.902), respectively. The κ statistic for predictive validity was 0.712 (95% CI, 0.676 to 0.766). CONCLUSION SINS demonstrated near-perfect inter- and intraobserver reliability in determining three clinically relevant categories of stability. The sensitivity and specificity of SINS for potentially unstable or unstable lesions were 95.7% and 79.5%, respectively.

Long‐term results in 144 localized Ewing's sarcoma patients treated with combined therapy

The results of 144 previously untreated cases of primary Ewings sarcoma of bone are reported with a minimum follow‐up of 5 years. This series was treated between 1972 and 1982 at Istituto Ortopedico Rizzoli with a combined therapy. The local control of the disease consisted of amputation (ten cases), resection followed by radiation therapy (35‐45 Gy) (48 cases) and radiation therapy alone (40‐60 Gy) (86 cases). Adjuvant chemotherapy, rigorously standardized, was performed according two different protocols: the first (85 cases treated in the period 1972–1978) consisted of vincristine (VCR) Adriamycin (doxorubicin) (ADM), and cyclophosphamide (EDX); the second (59 cases treated in the period 1979‐1982) of VCR, ADM, EDX and dactinomycin (DACT). At a follow‐up of 5 to 16 years (median, 9), 59 patients (41%) are continuously disease‐free (CDF), 81 (56%) developed metastatic disease and/or local recurrence, and four (3%) had a second malignancy. Three factors seem to be correlated to prognosis: the site of the initial lesion (only 23% of the pelvic lesions are represented in the CDF group versus 46% of the other locations); the chemotherapy protocol (32% of the cases in the first protocol are CDF versus 54% in the second); the type of local treatment (60% of the patients treated with amputation or resection plus radiotherapy versus 28% of those treated with radiation therapy alone are CDF). A local recurrence was observed in 24% of the patients (8% in the group locally treated with surgery or surgery plus radiation therapy versus 36% in the group treated with radiation therapy alone). These data suggest that even though adjuvant chemotherapy can improve the long‐term results in localized Ewings sarcoma patients, this disease still represents, in a high percentage of cases, a lethal process whose final prognosis widely depends on the local control of the lesion. Due to the questionable effect of the radiation therapy alone in controlling the primary lesion and its important side effects, the role of surgery in treating Ewings sarcoma of bone should be extended.

Aneurysmal Bone Cyst of the Mobile Spine: Report on 41 Cases

Study Design. Forty-one cases of aneurysmal bone cyst of the mobile spine were retrospectively reviewed. Objectives. To evaluate the role of surgical and nonsurgical treatment of aneurysmal bone cyst of the spine. Summary of Background Data. Ten to 30% of aneurysmal bone cysts arise from the mobile spine, frequently occurring in pediatric patients. The course of the disease depends on the aggressiveness of the tumor, as well as the treatment. Intralesional surgery seems to be an effective treatment, as well as radiotherapy and embolization. Methods. All charts, radiographs, and images were reviewed. The composite information provided by this review allowed for oncologic and surgical staging of these cases. Thirty-two patients underwent curettage (14 of them followed by radiotherapy), four were submitted to selective arterial embolization, three received radiotherapy alone, and two underwent en bloc excision. Results. All patients were found alive and disease free at final follow-up evaluation. Two recurrences followed one incomplete curettage and one embolization. The combination of curettage and radiotherapy, although effective, showed the greatest incidence of late axial deformity. Selective arterial embolization was curative in three of four cases and did not affect the possibility of surgery in case of local recurrence. Conclusions. If confirmed on larger series, selective arterial embolization seems to be the first treatment option for spine aneurysmal bone cyst, because of the low cost-to-benefit ratio. Diagnosis must be certain, based on pathognomonic radiographic pattern or on histologic study.— In case of neurologic involvement, pathologic fracture, technical impossibility of performing embolization, or local recurrence after at least two embolization procedures, complete intralesional excision would be the therapy of choice.

Chordoma of the spine above the sacrum. Treatment and outcome in 21 cases.

Study Design Twenty‐one cases of chordoma arising in the mobile spine were retrospectively reviewed. Objectives All the cases were submitted to oncologic and surgical staging to correlate treatment and outcome. Summary of Background Data Excluding plasmacytomas, chordoma is the most frequent primary malignant tumor of the spine, occurring mainly in elderly men. The course of the disease is slow, metastases occur late, and death can result from complications related to local extension of the disease. Complete excision of the tumor according to oncologic criteria can be hampered by extension of the tumor and by anatomic constraints in the mobile spine. Methods All charts, radiographs, and images were reviewed. The composite information provided by this review allowed for oncologic and surgical of these cases. Treatment was defined according to Ennekings criteria. All the patients were followed for determination of their status clinically and radiographically. Results Ten patients died (1 to 137 months after treatment, mean 65 months); four patients are alive with the disease; only seven patients (33%) are symptom free at the final follow‐up (39 to 112 months after treatment, mean 65 months). Conventional radiation therapy was not effective in eradicating the tumor, even if associated with palliative or debulking surgery: of 15 cases, 12 were associated with recurrence or progression. Intralesional surgery also was not effective (two recurrences in two cases, 18 to 41 months later). En bloc excision of the lesion, sometimes combined with radiation therapy as an adjuvant, obtained the best results (four patients disease free at 39 to 112 months, mean 77 months). Conclusions En bloc excision‐even if marginal‐is the treatment of choice of chordomas of the spine. Early diagnosis and careful surgical staging and planning are necessary. Megavoltage radiation can be administered as an adjuvant.

Chondrosarcoma of the mobile spine: report on 22 cases.

STUDY DESIGN A retrospective review of 22 cases of chondrosarcoma arising from the mobile spine. OBJECTIVE To evaluate the role of oncologic and surgical staging in correlating management and outcome of chondrosarcoma involving the spine. SUMMARY OF BACKGROUND DATA Approximately 10% of chondrosarcomas arise from the mobile spine, occurring mainly in adults, particularly elderly men. The course of the disease depends on the aggressiveness of the tumor, but also is influenced by the management. Intralesional surgery is followed almost constantly by local recurrence even with adjuvant therapy. METHODS All charts, radiographs, and images were reviewed. The composite information provided by this review allowed for oncologic and surgical staging of the reviewed cases. According to Enneking criteria, the surgical procedures were defined as curettage (piecemeal excision) or en blocexcision. The margins were submitted to histologic study and reported as intralesional, marginal, or wide. - As primary management, 10 intralesional curettages (follow-up period, 2-119 months; average, 61 months) and 12 en bloc excisions (follow-up period, 39-207 months; average, 97 months) were performed. A total of 33 procedures were performed, including the management of the recurrences (18 curettages and 15 en bloc excisions: one for soft tissue recurrence). A clinical and radiographic follow-up period of of 2 to 236 months (average, 81 months; minimal follow-up period for survivors, 30 months; average follow-up period for survivors, 115 months) was available for all the patients. RESULTS Three recurrences occurred in 14 patients treated by en bloc excision at onset or for recurrence, two in cases of histologically proven contaminated or intralesional margins. All but one patient were alive at final follow-up evaluation. Conversely, all the patients treated by one or more curettages (with or without adjuvant radiation therapy) had at least one recurrence, and 8 of 10 of these patients died of the disease. At final follow-up evaluation, nine patients had died of the disease; nine were continuously disease free (but one had died of another unrelated malignancy); and four were symptom free after management for recurrences (one was found alive 155 months after a soft-tissue metastasis en-bloc excision). CONCLUSIONS En bloc excision, with wide or marginal histologic margins, is the suggested management for chondrosarcomas of the spine. Early diagnosis and careful surgical staging and planning are necessary for conducting adequate management. However, tumor contamination of the specimen margins, even in a small area, or spreading of the tumor myxoid content can worsen the prognosis.

A system for surgical staging and management of spine tumors: A clinical outcome study of giant cell tumors of the spine

Study Design. This study developed and independently applied a spine tumor classification system, referred to as the Weinstein‐Boriani‐Biagini system, in a retrospective analysis of a series of patients with spinal giant cell tumors from three institutions. Objective. To evaluate factors of potential prognostic significance for recurrence of spinal giant cell tumors. Summary of Background Data. No prior reviews of patients treated with modern surgical techniques are available. Methods. Charts and radiographs for 36 cases of spinal giant cell tumors were reviewed by an independent investigator. All patients had had recent clinical follow‐up examinations. All patients were classified according to the Enneking system. A subgroup of 24 patients for whom preoperative computed tomography scans were available were classified using the Weinstein‐Boriani‐Biagini staging system. Outcome measures included pain, neurologic status, and tumor recurrence. Results. Recurrence rates were substantially higher among patients treated with attempted surgical excision before referral to a tertiary care center (83% vs. 18%). There was a higher recurrence rate for tumors that involved the vertebral body and posterior elements in comparison with lesions residing in only anterior elements (24% vs. 0%). Tumors that had extra‐osseous extension into the canal and into the paraspinous musculature had a higher recurrence rate than tumors either confined to the osseous compartment or with extension either into the spinal canal or externally into paraspinous planes, but not both (21% vs. 10%). Conclusions. These results indicate that the Weinstein‐Boriani‐Biagini system may prove useful in developing treatment algorithms and in assessing outcome for these rare and difficult lesions. At least in the case of giant cell tumors, the musculoskeletal tumor staging system as developed by Enneking for long bones suggests the ideal surgical margin and may provide information relevant to tumor recurrence rates. Additional aspects of tumor extent and location, however, may berelevant to primary tumor recurrence rates when theselesions occur in the spine.