Outcome of expandable prostheses in children.

BACKGROUND: Expandable prostheses offer the advantages of limb-salvage and limb-length equality at skeletal maturity. However, what is the cost for achieving that goal, and in how many children this is achieved? MATERIALS AND METHODS: We present 32 children (16 boys and 16 girls; mean age, 9 y) with bone sarcomas of the femur treated with limb salvage using expandable prostheses. The Kotz Growing prosthesis and the noninvasive Repiphysis and Stanmore expandable prostheses were used. The mean follow-up was 49 months. Survival analysis of the children and primary implants and functional evaluation were performed. RESULTS: Survival of the children was 94% and 84% at 48 and 72 months. Survival of the primary prostheses was 78% and 66% at 48 and 72 months; survival was significantly higher only for the Kotz when compared with the Repiphysis prostheses (P=0.026). The rate of implant-related complications was 51.3%; 9 prostheses (23%) were revised because of aseptic loosening, infection, and breakage. A mean total lengthening of 28 mm (4 to 165 mm) was achieved by 84 procedures (2.6 procedures/patient). Three of the 9 children who reached skeletal maturity had limb-length equality and 6 discrepancy of 15 to 30 mm. The mean Musculoskeletal Tumor Society score was excellent (79%) without a significant difference between the type of prostheses (P=0.934). CONCLUSIONS: The Kotz Growing prosthesis, although it requires an open lengthening procedure, has shown higher survival when compared with the noninvasive Repiphysis prosthesis. However, the total lengthening remains small, and the complications rates are high even with the noninvasive prostheses.

Outcome of the intramedullary diaphyseal segmental defect fixation system for bone tumors.

BACKGROUND: Resection of diaphyseal malignant bone tumors is indicated for local control and impending pathological fracture or failure of prophylactic internal fixation. However, there are no large, long-term studies analyzing the results of intercalary reconstruction using segmental metallic spacers. MATERIALS AND METHODS: We present 24 patients treated with wide resection for primary or metastatic bone tumors involving the diaphysis of the femur, tibia, or humerus and reconstruction using a modular intramedullary diaphyseal segmental defect fixation system. The mean length of bone resection was 10 cm. The postoperative complications and outcome were evaluated. RESULTS: At a mean follow-up of 29 months, 17 patients were alive and 7 patients were dead of disease; no patient had local recurrence. Implant-related complications occurred in 8 patients, the most common being mechanical loosening and rotational instability. Loosening was most common in reconstructions of more than 10 cm length of bone resection. In all femoral reconstructions, mechanical failure occurred at the proximal stem. True limb length discrepancy of 2.0 cm was observed in one patient. Wound healing complications were not observed; range of motion and function of the adjacent joints was within normal limits. CONCLUSIONS: The modularity, ease of application and preservation of the adjacent joints are major advantages of segmental modular prostheses; however, the complications' rate is high. Complications occur most often at the proximal stem in femoral reconstructions and reconstructions for more than 10 cm length of bone resection. In these cases, the use of these implants should be reconsidered or not recommended.

Gorham-Stout disease: the experience of the Rizzoli Institute and review of the literature.

Gorham-Stout disease (also known as "disappearing bone disease") was first described by Jackson in 1838, but was properly defined by Gorham and Stout in a series of 24 patients in 1954-1955. It is a rare disease of unknown etiology (about 200 cases reported in the literature) characterized by spontaneous progressive resorption of bone without malignant proliferation of vascular structures. The diagnosis is one of exclusion and it is based on combined histological, radiological, and clinical features. Benign vascular proliferation with fatty bone marrow and thinning of bony trabeculae is a typical histological feature. Standard radiographs of disappearing bone disease show progressive bony resorption with adjacent soft tissue involvement. Most cases of Gorham-Stout disease resolve spontaneously, but prognosis remains unpredictable. This study reports 13 cases of Gorham-Stout disease treated in our institution from 1968 to 2008. The aim of the work was to review our series and the literature on this rare disease, as well as to evaluate whether or not an optimal treatment can be identified and recommended.

Evolution of surgical treatment for sarcomas of proximal humerus in children: retrospective review at a single institute over 30 years.

BACKGROUND: Proximal humerus, although a common site for primary bone sarcomas, finds scant mention in literature as far as options and outcome of reconstruction in the skeletally immature skeleton are concerned. Reconstruction after resection of proximal humeral sarcomas in the immature skeleton poses specific challenges to the surgeon, and there has been a definite evolution of these techniques over the decades. We studied the evolution and compared the outcome of various techniques for such reconstruction over 3 decades at a single institution. METHODS: All 61 children younger than 13 years of age and treated for a primary sarcoma of the proximal humerus at Department of Musculoskeletal Oncology, Rizzoli Orthopedic Institute, from 1976 to 2006 were studied for techniques of resection and reconstruction, complications, surgical procedures needed during follow up, and functional and radiologic outcomes during and at final follow-up. The functional outcomes after various procedures were compared using the Musculoskeletal Tumor Society scoring system. RESULTS: A definite trend from amputation in the first decade, to the use of nonbiological reconstruction (endoprostheses, K nail cement spacer) in the second and biological reconstruction (vascular proximal fibula autograft, osteoarticular allograft, and allograft prosthesis composite) in the third decade was seen. There was a trend of improvement in the functional outcome over the 3 decades, although the complication rates and the need for repeated surgical procedures remained a major problem in all the techniques. CONCLUSIONS: Reconstruction of proximal humerus after resection for sarcomas is a challenging task. Although endoprostheses do have a definite role to play in reconstruction of proximal humerus in children, the use of biological techniques in well-selected patients is being carried out more often now than before, as is reflected in this series, with a potentially improved functional outcome. LEVEL OF EVIDENCE: Level III-Retrospective comparative study.

Post traumatic myositis ossificans: Sonographic findings.

PURPOSE: The aim of this paper is to describe the sonographic (US) features of post traumatic myositis ossificans (PTMO). METHODS: Fifteen patients with histopathological diagnosis of PTMO were included. The following US parameters were evaluated: presence of a mass, calcifications, power Doppler signals, and relationship with adjacent structures. RESULTS: Five patients (33.3%) were diagnosed as early PTMO and 10 (66.6%) as mature PTMO. In both early and mature PTMO, US showed an oval hypoechoic mass, without infiltrative borders, located in a muscular belly. In the early phase, a center of less echogenicity was observed, associated with an outer sheet-like hyperechoic peripheral rim, while, in the mature phase, peripheral calcification created more reflections and distal acoustic shadowing was observed. CONCLUSIONS: US is highly sensitive in the detection of PTMO, helps differentiate between PTMO and malignant soft tissue tumors, and can be used for follow-up of the lesions.

Dedifferentiated chondrosarcoma revisited.

This article reviews recent molecular, biologic, therapeutic, and clinical findings in dedifferentiated chondrosarcoma. Dedifferentiated chondrosarcoma is one of the most malignant primary bone tumors characterized by two distinct histopathologic components: a well-differentiated chondral lesion sharply juxtaposed with a high-grade noncartilaginous component. Wide or radical surgical resection is mandatory. High-dose radiation therapy is confined to inaccessible sites and palliation. Chemotherapy is administered whenever the dedifferentiated component is chemosensitive and the patient is in good general condition. Despite this approach, metastases will result in poor survival of these patients.

Chordoma of the mobile spine and sacrum: a retrospective analysis of a series of patients surgically treated at two referral centers.

BACKGROUND: Chordoma is a rare tumor, and its natural history is still not well known. MATERIALS AND METHODS: All patients affected by localized chordoma surgically treated at Istituto Ortopedico Rizzoli, Bologna, and Istituto Nazionale Tumori, Milan, Italy, between 1980 and 2008 were reviewed. Local recurrence, distant metastasis, and overall survival (OS) were analyzed both from time of diagnosis and from time of local recurrence/distant metastasis. A multivariable analysis to identify independent prognostic factors was carried out. RESULTS: A total of 138 consecutive patients were identified (sacrum 78%, lumbar spine 15%, cervical-dorsal spine 7%). Of these, 130 underwent surgical resection. Median follow-up was 142 months. The 5- and 10-year OS, local relapse-free survival (LRFS), and distant relapse-free survival (DRFS) were, respectively, 78% and 54%, 52% and 33%, and 86% and 72%. Size was an independent prognostic factor for OS (P value < .001), LRFS (P value: .038), and DRFS (P value: .004), while surgical margins independently predicted LRFS (P value: .003) with a trend for OS. The 5- and 10-year OS, LRFS, and DRFS after the first local relapse were 50% and 26%, 47% and 31%, and 64% and 61%. The size of the recurrence and quality of surgical margins did not influence postrelapse OS. The 5-year OS after the second local relapse was 19%. 22% of patients developed distant metastases with a 5-year post-metastases OS of 33%. CONCLUSIONS: Tumor size and surgical margins affected outcome only on initial presentation. However, wide surgery was feasible in a minority of cases. Most patients died of local-regional disease even when metastases occurred. Indeed, long-term prognosis was such that disease-free survival at 10 years was only 26%.

Desarthrodesis and prosthetic reconstruction of the knee after resection of bone tumors.

BACKGROUND AND OBJECTIVES: The aim of the present study was to review the long-term results in patients who had undergone modular prosthetic reconstruction of the knee, following a failed arthrodesis performed after the resection of bone tumors. METHODS: We reviewed 16 desarthrodeses and prosthetic reconstructions of the knee following a failed artificial arthrodesis after bone tumor resection. Desarthrodeses and subsequent knee megaprosthesis were performed after breakage of the arthrodesis implant in 11 cases and following presumed eradication of infection in five cases. RESULTS: The mean follow-up was 15.9 years. The mean post-operative range of knee flexion was 70°, but only four patients had active extension of the knee. Functional results, according to the MSTS system, were satisfactory in 11 patients. Complications included deep infections in five patients, aseptic loosening in three patients, breakage of the joint hinge in one patient, and patellar tendon avulsion in one patient. Despite a high rate of complications, all but two patients were satisfied. CONCLUSION: Our findings indicate that conversion of oncological knee arthrodesis to total knee arthroplasty should be taken into consideration only after giving the patient extensive information about the high risk of serious complications.

Clinically significant thromboembolic disease in orthopedic oncology: an analysis of 986 patients treated with low-molecular-weight heparin.

BACKGROUND AND OBJECTIVES: Thromboembolic risk in orthopedic oncology is high due to several factors. The aim of this study was to assess clinically significant thromboembolic disease in 986 patients operated on with a prosthetic reconstruction of the lower limbs after the resection of bone tumors and prophylactically treated with low-molecular-weight heparin (LMWH). METHODS: Between 1983 and 2006, 986 patients had uncemented megaprostheses after a resection of the lower limbs for bone tumors. Antithromboembolic prophylaxis was always administered with LMWH from the immediate postoperative time until the time of complete weight-bearing. Phlebographies and vascular ecodoppler were not performed postoperatively on a regular basis. Patients were followed in the clinic with imaging studies for several years (lower limb CT, MRI, CT of the chest for malignant tumors). The diagnosis of symptomatic venous thromboembolism (VTE) was established or excluded on clinical evidence and MRI study. RESULTS: Among the 986 cases treated, only 11 patients (1.1%) showed a major thromboembolic event confirmed clinically and through imaging. One of these patients died with pulmonary embolism a few days after surgery. Two cases of thromboembolism occurred in patients with a vascular bypass. CONCLUSIONS: Despite general oncologic and orthopedic factors favoring VTE, the clinical occurrence of this event was extremely low in this series, probably due to a consistent and careful prophylaxis, prolonged until the time of complete weight-bearing.

The use of prolonged peripheral neural blockade after lower extremity amputation: the effect on symptoms associated with phantom limb syndrome.

BACKGROUND: Phantom limb syndrome (PLS) is common after limb amputations, involving up to 90% of amputees. Although many different therapies have been evaluated, none has been found to be highly effective. Therefore, we evaluated the efficacy of a prolonged perineural infusion of a high concentration of local anesthetic solution in preventing PLS. METHODS: A perineural catheter was placed immediately before or during surgery in 71 patients undergoing lower extremity amputation. A continuous infusion of 0.5% ropivacaine was started intraoperatively at 5 mL/h using an elastomeric (nonelectronic) pump, and continued for 4 to 83 days after surgery. PLS was evaluated on the first postoperative day and then 1, 2, 3, and 4 weeks, and 3, 6, 9, and 12 months after surgery. To evaluate the presence and severity of PLS while the patient was receiving the ropivacaine infusion, it was discontinued for 6 to 12 hours before each assessment period (i.e., until the sensation in the extremity returned). The severity of phantom limb and stump pain was assessed using a 5-point verbal rating scale (VRS), with 0 = no pain to 4 = intolerable pain, and "phantom" sensations were recorded as present or absent. If the VRS score was >1 or significant phantom sensations were present, the ropivacaine infusion was immediately restarted at 5 mL/h. If the VRS score remained at 0 to 1 and the patient had not experienced phantom sensations for 48 hours, the infusion was permanently discontinued and the catheter was removed. RESULTS: Median duration of the local anesthetic infusion was 30 days (95% confidence interval, 25-30 days). On postoperative day 1, 73% of the patients complained of severe-to-intolerable pain (visual analog scale >2). However, the incidence of severe-to-intolerable phantom limb pain was only 3% at the end of the 12-month evaluation period. At the end of the 12-month period, the percentage of patients with VRS pain scores were 0 = 84%, 1 = 10%, 2 = 3%, 3 = 3%, and 4 = none. However, phantom limb sensations were present in 39% of patients at the end of the 12-month evaluation period. All patients were able to manage the elastomeric catheter infusion system at home. CONCLUSION: Use of a prolonged postoperative perineural infusion of ropivacaine 0.5% seems to be an effective therapy for the treatment of phantom limb pain and sensations after lower extremity amputation.

The role of surgery and adjuvants to survival in Pagetic osteosarcoma.

BACKGROUND: Osteosarcoma is a rare complication of Paget's disease with a very poor prognosis. Treatment is controversial: the older age of the patients affected by Paget's disease may limit the use of chemotherapy and axial involvement may limit the practicality of surgery. QUESTIONS/PURPOSES: The purposes of this study are (1) to report the survival in patients treated for osteosarcoma in Paget's disease; (2) to identify correlations between type of treatment and survival comparing our data with those in the literature; (3) to determine if the extent of Paget's disease and risk of malignant transformation are associated; (4) to assess if prognosis is related with site; and (5) to identify the variations of histologic subtypes of these osteosarcomas. METHODS: We retrospectively reviewed the medical records of 26 patients treated between 1961 and 2006 who had bone sarcoma arising from a site of Paget's disease. Twenty two of the 26 patients had surgery. In six surgery only was performed; three had surgery, adjuvant chemotherapy, and radiotherapy; one surgery and radiotherapy; 12 underwent surgery and chemotherapy, adjuvant in 10 patients and neoadjuvant in two; two had only radiotherapy and two had only chemotherapy. We performed survival analyses between various combinations of treatment. RESULTS: At last followup four patients had no evidence of disease (NED) at a minimum followup of 42.6 months (mean, 139 months; range, 42.6-257.4 months) and 22 died with disease (DWD) at a minimum time of 1 month (mean, 20.2 months; range, 1-84 months). One of the six patients (11%) treated with surgery only had NED at 10 years; the other five died from disease at a mean of 30 months. Three of 12 patients (25%) treated with surgery and chemotherapy are NED at a mean followup of 12 years; nine died of disease at a mean of 24 months. All patients treated without surgery died at a mean of 7.5 months (range, 1-13.7 months). CONCLUSIONS: Despite improvements in surgery and medical treatments the prognosis remains poor in patients with Paget's sarcoma.

Local recurrence, survival and function after total femur resection and megaprosthetic reconstruction for bone sarcomas.

BACKGROUND: The choices of treatment for patients with extensive tumors of the femur include total femur megaprosthesis or large allograft-prosthetic composites. Previous reports suggest variable survival ranging from 60-70% at 1 to 2 years. However, these studies described earlier prostheses and techniques. QUESTIONS/PURPOSES: To confirm previous reports we determined (1) risk of local recurrence; (2) overall survivorship; and (3) function in patients with total femur reconstructions for tumors. METHODS: We retrospectively reviewed 23 patients with total femur megaprostheses implanted between 1987 and 2006 after resection of bone tumors. Two patients lost at followup were excluded; the remaining 21 included 15 males and six females with a mean age of 21 years. The mean followup was 48 months (range, 1 month 17 years). Function was assessed according to the MSTS System II. RESULTS: No patient developed a local recurrence during followup. At last followup, six patients were continuously disease-free at a mean of 148 months, one patient had no evidence of disease after treatment of a recurrence, one patient was alive with disease, and 13 patients died of their disease at a mean time of 17 months. In 15 patients evaluated with the MSTS score, the mean score was 66%; four patients had over 75%, eight from 51% to 75%, three from 26% to 50%. Four patients (19%) had complications requiring further surgery in absence of trauma. A fifth patient had a posttraumatic periprosthetic fracture. CONCLUSIONS: A total femur prosthesis allows a limb-preserving procedure in tumors with extensive femoral involvement or in the presence of a skip lesion along the femur. The prognosis of these tumors is poor, but this reconstruction provides function with a relatively low rate of major complications.

Surgical margins and local control in resection of sacral chordomas.

BACKGROUND: The treatment of choice in sacral chordoma is surgical resection, although the risk of local recurrence and metastasis remains high. The quality of surgical margins obtained at initial surgery is the primary factor to improve survival reducing the risk of local recurrence, but proximal sacral resections are associated with substantial perioperative morbidity. QUESTIONS/PURPOSES: We considered survivorship related to local recurrence in terms of surgical margins, level of resection, and previous surgery. METHODS: We retrospectively reviewed 56 patients with sacral chordomas treated with surgical resection. Thirty-seven were resected above S3 by a combined anterior and posterior approach and 19 at or below S3 by a posterior approach. Nine of these had had previous intralesional surgery elsewhere. The minimum followup was 3 years (mean, 9.5 years; range, 3-28 years). RESULTS: Overall survival was 97% at 5 years, 71% at 10 years, and 47% at 15 years. Survivorship to local recurrence was 65% at 5 years and 52% at 10 years. Thirty percent of patients developed metastases. Wide margins were associated with increased survivorship to local recurrence. We found no differences in local recurrence between wide and wide-contaminated margins (that is, if the tumor or its pseudocapsule was exposed intraoperatively, but further tissue was removed to achieve wide margins). Previous intralesional surgery was associated with an increased local recurrence rate. We observed no differences in the recurrence rate in resections above S3 or at and below S3. CONCLUSIONS: Surgical margins affect the risk of local recurrence. Previous intralesional surgery was associated with a higher rate of local recurrence. Intraoperative contamination did not affect the risk of local recurrence when wide margins were subsequently attained.

Recurrence after and complications associated with adjuvant treatments for sacral giant cell tumor.

BACKGROUND: The best treatment of giant cell tumor of the sacrum is controversial. It is unclear whether adjuvant treatment with intralesional surgery reduces recurrences or increases morbidity. QUESTIONS/PURPOSES: We therefore asked whether adjuvants altered recurrence rates and complications after intralesional surgery for sacral giant cell tumors. METHODS: We retrospectively studied 31 patients with sacral giant cell tumors treated with intralesional surgery with and without adjuvants. Survival to local recurrence was evaluated using Kaplan-Meier analysis. The differences in survival to local recurrence with and without adjuvants were evaluated using multivariate Cox regression analysis. Complications were recorded from clinical records and images. The minimum followup was 36 months (median, 108 months; range, 36-276 months). RESULTS: Overall survival to local recurrence was 90% at 60 and 120 months. Survival to local recurrence with and without radiation was 91% and 89%, with and without embolization was 91% and 86%, and with and without local adjuvants was 88% and 92%, respectively. Adjuvants had no influence on local recurrence. Mortality was 6%: one patient died at 14 days postoperatively from a massive pulmonary embolism and another patient had radiation and died of a high-grade sarcoma. Fifteen of the 31 patients (48%) had one or more complications: eight patients (26%) had wound complications and seven patients (23%) had massive bleeding during curettage with hemodynamic instability. L5-S2 neurologic deficits decreased from 23% preoperatively to 13% postoperatively; S3-S4 deficits increased from 16% to 33%. CONCLUSIONS: Adjuvants did not change the likelihood of local recurrence when combined with intralesional surgery but the complication rate was high.

Selective arterial embolization of 36 aneurysmal bone cysts of the skeleton with N-2-butyl cyanoacrylate.

BACKGROUND: Aneurysmal bone cyst (ABC) is a lytic benign bone lesion representing about 1% of all primary bone tumors. The lesion causes pain and swelling, which are generally present for less than 3 months. METHODS: From April 2003 to April 2008 36 patients affected by aneurysmal bone cysts were treated by selective arterial embolization with N-2-butyl cyanoacrylate. The study population comprised 20 male and 16 female patients with an age range of 3.3-60.8 years. Nine lesions were localized in the appendicular skeleton (1 in the upper and 8 in the lower limb), 4 in the thoracic cage (1 rib lesion and 3 scapular lesions), 17 in the pelvis and 6 in the spine (1 thoracic and 5 sacral localizations). RESULTS: A total of 55 embolizations were performed: in 22 cases (61%) only one embolization was needed, whilst two embolizations were necessary in 9 cases (25%) and 3 in the remaining 5 patients (14%). The treatment was effective in 32 patients (94% ): follow-up was 0.9-5 years. In one patient, previously surgically treated, only the cyanoacrylate embolization turned out to be useful for healing the lesion. Another 7 patients underwent surgery during the study period. In the 55 procedures we performed we had 3 complications (5%): 2 cases of skin necrosis and 1 of transient paresis. CONCLUSIONS: Arterial embolization with cyanoacrylate may be the treatment of choice for aneurysmal bone cysts. Embolization is a less invasive, lower cost, simpler procedure than surgery and is easily repeatable.

Vincristine, doxorubicin, cyclophosfamide, actinomycin D, ifosfamide, and etoposide in adult and pediatric patients with nonmetastatic Ewing sarcoma. Final results of a monoinstitutional study.

AIMS AND BACKGROUND: To investigate a six-drug combination in patients with nonmetastatic Ewing sarcoma, focusing on chemotherapy-induced necrosis and chemotherapy toxicity in adult and pediatric patients. METHODS AND STUDY DESIGN: Alternating cycles of vincristine (1.5 mg/m2), doxorubicin (80 mg/m2) and cyclophosfamide (1200 mg/m2) (weeks 0, 6, 13, 22 and 31), ifosfamide (9 g/m2), vincristine (1.5 mg/m2), and actinomycin D (1.5 mg/m2) (weeks 3, 16, 25 and 34), and ifosfamide (9 g/m2) and etoposide (450 mg/m2) (weeks 9, 19, 28 and 37) were administered. Primary chemotherapy-induced necrosis was graded: G3 (complete necrosis), G2 (microfoci of tumor cells) and G1 (macrofoci of tumor cells). RESULTS: From 1996 to 1999, 50 patients with Ewing sarcoma were enrolled. The median age was 23.5 years (range, 4-56). Chemotherapy-induced necrosis (in 28 patients) was G3 in 36%, G2 in 21% and G1 in 43%. At a median follow-up of 110 months (range, 36-129), 5-year overall survival and event-free survival were 72% and 66%, respectively. According to histologic response, 5-year event-free survival was 90% in G3, 83% in G2, and 42% in G1 (P = 0.02). In adult and pediatric (<18 years) patients, the incidence of G4 leukopenia was 62% and 74%, respectively, with febrile neutropenia in 13% and 21%, respectively. G4 thrombocytopenia occurred in 3% of cycles in adults and in 7% in pediatric patients. Platelet and red blood cell transfusions were required respectively in 1% and 11% of cycles in adults and in 6% and 24% of cycles in pediatric patients. CONCLUSIONS: The six-drug combination can be administered safely in adult and pediatric populations. About 40% of patients have a poor chemotherapy-induced tumor necrosis, leading to poor probability of survival. New strategies are recommended to improve survival of poor responders to the six-drug combination.

Long-term results in children with massive bone osteoarticular allografts of the knee for high-grade osteosarcoma.

BACKGROUND: Reconstruction of distal femur or proximal tibia in growing patients is a challenge for the high rate of complications and limb length discrepancy at the end of growth. The purpose of this study was to evaluate the long-term outcome of children affected by high-grade osteosarcoma of the knee region, reconstructed by osteoarticular bone allograft of distal femur, and proximal tibia. METHODS: We retrospectively reviewed 25 patients treated for high-grade osteosarcoma, 13 in the distal femur and 12 in the proximal tibia. The mean follow-up was 124 months. Clinical and radiologic evaluation was carried out in the 20 long-term survivors with a minimum follow-up of 7 years from surgery. The rates of survival of the implants were estimated with use of the Kaplan-Meier method. Functional and radiographic evaluation was done according to the Musculoskeletal Tumor Society scoring system at the time of the latest follow-up in all the patients that still had the allograft. RESULTS: Five patients died during the first 2 years of follow-up for disease-related causes. Of the remaining 20 osteoarticular allografts (10 of the distal femur and 10 of the proximal tibia), 12 failed: 4 in the distal femur and 8 in the proximal tibia. All the failures were related to a graft fracture, but in 4 patients with subchondral collapse the graft was maintained and converted into an allograft prosthetic composite. No deep infection of the primary reconstruction was observed. The overall rate of allograft survival was 70% at 5 years and 58% at 10 years in the distal femur, and 45% at 5 years and 20% at 10 years in the proximal tibia. At final follow-up, 8 patients still walked on the primary implant, 6 in the distal femur, and 2 in the proximal tibia. The functional outcome of these patients was evaluated as good in 5 patients (3 with distal femoral and 2 with proximal tibial allograft), and poor in 3. CONCLUSIONS: Although mechanical complications significantly affect the outcome, osteoarticular allografts may represent a viable option for reconstruction in children older than 8 with high-grade sarcomas about the knee. LEVEL OF EVIDENCE: Level IV.

The treatment of nonmetastatic high grade osteosarcoma of the extremity: review of the Italian Rizzoli experience. Impact on the future.

The Bone Tumor Center of the "Istituto Ortopedico Rizzoli" was established in 1955 with the aim of studying and treating the musculoskeletal tumors. Between 1959 and 2006, 1245 patients with high grade nonmetastatic osteosarcoma of the extremity were treated at our Institute. Most of them were enrolled in study protocols. In the "prechemotherapy era", the cure rate was 11%, with an amputation rate of 90%. Our first experience with adjuvant chemotherapy was in 1972. A total of 223 patients received adjuvant chemotherapy, with a disease-free survival (DFS) ranging from 45% to 53%, according to the chemotherapy protocol used. With the introduction of neoadjuvant chemotherapy, the resection rate increased and reached 94%, when high dose fosfamide was added to standard doses of methotrexate, cisplatin, and adriamycin. In the last few years, the results of treatment of nonmetastatic osteosarcoma of the extremity have reached a plateau (64% five-year DFS), and strategies of dose intensification are not able to improve the prognosis. Not only new active drugs, but also different approaches to the disease, are needed. In this regard, we are now investigating tumor microenvironment-targeted agents and chemotherapy protocols based on prospective biological stratification of patients. Collaborative projects with international groups and institutions are crucial for this rare disease.

Treatment of nonmetastatic Ewing's sarcoma family tumors of the spine and sacrum: the experience from a single institution.

The objective of this study is to determine the best local treatment combined with neoadjuvant chemotherapy for ESFT of the spine and sacrum, for the best local treatment for Ewing sarcoma family tumors (ESFT) according to the primary site is still unclear. Nowadays surgery is used in local treatment of ESFT, but literature is scarce on the best local treatment in sites where surgery is problematic, such as the spine. This study evaluates the outcome and the rate of local recurrence of ESFT in the spine and sacrum when treated with neoadjuvant chemotherapy, and locally by radiotherapy alone or surgery, followed by reduced doses of radiotherapy. Forty-three patients with nonmetastatic ESFT located in the spine and sacrum were treated at our institution between 1983 and 2000 with neoadjuvant chemotherapy, and locally by radiotherapy alone in 26 cases, and surgery followed by radiotherapy at reduced doses in 17. The 5- and 10-year event-free survival (EFS) was 37 and 30%, and the 5- and 10-year overall survival was (OS) 42 and 32%. The prognosis was unrelated to gender and age, tumor volume, chemotherapy protocol, and local treatment. The outcome seemed worse for patients with primary tumors located in the sacrum than for patients with tumors located in the rest of the spine (5-year EFS = 23 vs. 46%). For these patients the results were significantly worse than for those we achieved with neoadjuvant treatment for ESFT located in other sites. However, no differences were observed between patients locally treated with radiotherapy alone and those treated by radiotherapy followed by surgery. We concluded that regardless of the type of local treatment even when associated with neoadjuvant therapy, ESFT in the spine and sacrum has a poor outcome and prognosis is significantly worse than that of primary ESFT in other sites.

The use of unicondylar osteoarticular allografts in reconstructions around the knee.

Unicondylar osteoarticular allografts (UOA) of the knee are mainly used after bone tumour resections for benign aggressive tumours or small malignant tumours with clearly defined margins. They are also used less often in large posttraumatic condylar defects. Between 1989 and 2004, 12 deep-frozen UOA reconstructions (in 11 patients) were performed at our Institute. The diagnosis was chondrosarcoma in four cases, giant cell tumour in three, osteosarcoma in three, posttraumatic defect in one, and one failed UOA. The involved site was the medial femoral condyle in six cases, the lateral femoral condyle in three, the medial side of the tibial plateau in two, and the lateral in one case. One allograft was removed after 29 months because of an intra-articular displaced fracture, and substituted with a new UOA. One patient died of metastatic disease at 24 months. We report the functional and radiographical outcome of the remaining 10 UOAs with a minimum follow-up of 4 years (average 11 years). Two of the 10 patients had excellent results, five were good and three were fair. Radiographically, five patients had "mild" and five had "severe" degenerative changes. One patient with severe degenerative changes had pain and stiffness, therefore the UOA was converted into a prosthesis allograft composite, using a conventional total knee prosthesis. In selected cases of distal femoral and proximal tibial tumours, UOA reconstructions give good functional outcomes with relatively few major complications.