Aims

Although internal hemipelvectomy is associated with a high incidence of morbidity, especially wound complications, few studies have examined rates of wound complications in these patients or have identified factors associated with the consequences. The present study aimed to: 1) determine the rate of wound and other complications requiring surgery after internal hemipelvectomy; and 2) identify factors that affect the rate of wound complications and can be used to stratify patients by risk of wound complications.

Purpose

Durable fixation may be difficult to achieve when significant bone loss is present, as it occurs in pelvic sarcoma resection and revision surgery of tumor implants. Purpose of this study was to review clinical results of primary and revision surgery of the pelvis and lower extremity in the setting of severe bone loss following limb salvage procedures for bone sarcoma using modular porous tantalum implants.

Purpose

Rotationplasty was first described in 1930 by Borggreve for treatment of limb shortening with knee ankylosis after tuberculosis. In 1948, Van Nes described its use for management of congenital defects of the femur and in the 1980s, Kotz and Salzer reported on patients with malignant bone tumors around the knee treated by rotationplasty as an alternative to above-knee amputation. Currently, rotationplasty is one of the options for surgical management of lower extremity bone sarcomas in skeletally immature patients but alternative limb salvage techniques, such as the use of expandable endoprosthesis, are also available. Despite rather satisfactory functional results have been uniformly associated with rotationplasty, concern still exists about the potential psychological impact of the new body imagerelated to the strange appearance of the rotated limb. Results of rotationplasty for sarcomas of the distal femur over a 20-year period were analyzed, focusing on long-term survival, function, quality of life and mental health.

Aim

Patients treated with limb salvage surgery for bone sarcomas of the extremities (upper and lower) may have physical disability as a result of treatment. Goal of this study was to evaluate the quality of life after treatment (chemotherapy and conservative surgery) at long term.

Aim

was to analyze infections after bone tumour surgery.

Aim

Purpose of this study was to review a single Institution experience and results of management of extraskeletal osteosarcoma (OGS), with emphasis on the role of combined treatment consisting of surgery and adjuvant chemotherapy.

Introduction: Extraskeletal osteosarcoma is a rare malignant tumor of the soft tissues. Overall, this malignancy has been associated with worse local control and overall survival rates than its skeletal counterpart despite multimodal approach. Purpose of this study was to review a single Institution experience and analyse results of management to identify factors affecting the outcome.

Methods: Retrospective study of 48 patients observed between 1966 and 2007 was undertaken. Of the total, 36 patients were admitted and managed at our Institution while 12 patients were sent for consultation and therefore not included in this study. Clinico-pathologic features and details of treatment of all 36 patients were reviewed and correlated with outcome. Updated follow-up was available in all patients.

Results: There were 21 males and 15 females, mean age was 53.6+/−19.3 years (range 14–84 yrs); 23 patients (63.9%) presented with localised disease while distant metastases were present in 13 patients (36.1%). Surgery consisted of a limb-salvage procedure in 25 patients (69.4%), amputation in 9 patients (25%) and 2 patients were considered inoperable (5.6%). Postoperative radiation therapy was given to 6 patients (16.7%) and multiagent chemotherapy administered to 19 patients (52.8%). At mean follow-up of 5.8 years, 23 patients had died of disease, expected 5 and 10 year overall survival rates were 41% and 31%. Tumor size and age at presentation were the most important predictors of survival while chemotherapy showed a trend towards improved survival in patients with localised disease.

Discussion and Conclusion: Extraskeletal osteosarcoma was associated with substantially worse prognosis than skeletal osteosarcoma despite multimodal management.

Osteosarcoma is the most common second malignancy seen in retinoblastoma survivors. Risk of developing osteosarcoma in this group is estimated approximately 500 times higher than the general population. Prognosis in this setting has been reported significantly worse than conventional osteosarcoma despite multimodal management. Purpose of this study was to evaluate clinical features, molecular aspects and outcome of treatment in this subgroup of osteosarcoma patients.

Between 1985 and 2004, from a total of about 1100 osteosarcomas, 7 survivors of retinoblastoma developing high-grade osteosarcoma as second malignancy presented at the authors’ Institution. Retrospective study was undertaken to analyze presentation, tissue expression of RB1, P53, PGP and DHFR, treatment and outcome of both retinoblastoma and osteosarcoma.

Retinoblastoma was bilateral in 5 cases and unilateral in two. All the patients had been treated with a combination of surgery +/− chemotherapy +/− radiation.

None of them had evidence of retinoblastoma at the time of second malignancy diagnosis. Average age at diagnosis of osteosarcoma was 14 years (9–17 years), mean interval between the two malignancies was 155 months. All the osteosarcomas were in the appendicular skeleton, all but one around the knee. Molecular analysis showed defective RB1 gene in all cases All the seven patients received contemporary multimodal management for osteosarcoma. All but one patient died of osteosarcoma within 30 months from diagnosis. The living patient had local recurrence 9 years after limb salvage and is currently disease free following amputation.

Prognosis of osteosarcoma in retinoblastoma patients remains poor as compared to conventional high grade osteosarcoma despite multimodal management. No obvious correlation was found between poor prognosis and P53, PGP and DHFR expression.

Introduction: The role of surgery for local control in the multimodal management of Ewing’s sarcoma has substantially increased during the past 20 years. However, selection bias due to location (extremities vs axial skeleton) and relatively non-homogeneous treatment received by patients in multi-institutional trials may limit objective evaluation and comparison of the relative role of surgery and radiation therapy in this setting. Purpose of this study was to review a large series of patients homogeneously treated at a single institution.

Methods: 268 patients with non-metastatic Ewing’s sarcoma of the extremities treated by contemporary multimodal management were reviewed. Chemotherapy was administered according to 4 sequential protocols of adjuvant (1) and neoadjuvant (3) treatment. Local control consisted of surgery in 136 patients, surgery and radiation therapy in 70 patients, and radiation therapy in 60 patients. Two patients underwent only chemotherapy.

Results: The 5-year event-free survival (EFS) and overall survival (OS) were 62 and 69 per cent respectively. The rates of 5-year EFS and local control were significantly lower in patients treated with radiation therapy compared to patients treated by surgery or surgery and radiation therapy (48 vs 66 per cent, p=0.002; 80 vs 94 per cent, p= 0,0001). In group 3 (Radiation Therapy only) there were also 6 secondary malignancies.

Conclusion: Surgery was associated with better survival and local control in this series. In our opinion, surgery should always be considered in the local treatment of Ewing’s sarcoma of the extremities. Postoperative Radiation Therapy must be added in cases of inadequate surgical margins.

Introduction: Secondary sarcomas of bone are a rare group of usually high-grade malignant tumors developing over different pre-existing bone conditions, historically associated with worse prognosis than their primary counterpart. Purpose of this study was to investigate the factors affecting the outcome, with emphasis modern multi-modal management.

Methods: From approximately 30000 bone tumor cases filed at our Institution, 87 patients with secondary sarcoma were identified. Secondary chondrosarcomas were not included and all the patients had an high-grade lesion. Patients were divided in 3 groups: radiation induced sarcomas (29), sarcomas in Paget’s disease (34), and sarcomas in benign lesions (24). Of these patients, 67 were admitted for treatment while 20 were consultations; of the 67 patients admitted, 44 had stage II while 23 had stage III disease. There were 57 males and 30 females, average age was 52. Most common histotype was osteosarcoma (81). Treatment varied from none to combined multimodal management including chemotherapy and surgery.

Results: Cumulative survival is 37% at 10 years. Survival was affected by stage of disease (II=54%, III=0%; p< 0.00005), site (extremities 44%, pelvis-spine 0%; p< 0.00005), inclusion of surgery in the treatment plan (yes 51%, no 0%; p< 0.00005), and type of treatment (combined chemotherapy and surgery 56%, surgery 39%; p=0.07). No differences were noted between the 3 main etiologic groups.

Conclusions: Secondary sarcomas are a rare group malignant tumors with heterogeneous clinical features. While stage and site are still the most relevant prognostic factors, modern multimodal management including neoadjuvant chemotherapy and surgery seems to favourably affect the outcome.

Introduction: Giant Cell Tumor (GCT) is rarely associated with lung metastases (1–4%). No prognostic factors have been reliably associated with the occurrence of lung metastases.

Since high levels of urokinase-type plasminogen activation system have been associated with cancer metastasis, purpose of this study was to investigate its expression in patients with giant cell tumor and the relationship with outcome.

Materials and Methods: Expression of urokinase-type plasminogen activation system was evaluated by immunohistochemistry in the primary lesion of 65 patients with GCT. This included urokinase-type plasminogen activator (u-PA), plasminogen activator inhibitor type 1 (PAI-1), and u-PA receptor (u-PAR). Patient population consisted of 12 cases that developed lung metastases and 53 cases that did not show metastases at last follow-up. Clinical outcome of the 2 groups was retrospectively reviewed and correlated with u-PA, PAI-1 and u-PAR expression levels.

Results: Overexpression of u-PA, PAI-1 and u-PAR was more frequent in the metastatic (92%) than non-metastatic (21%) group (p< 0.0005). Incidence of local recurrence was higher in the metastatic (67%) than non-metastatic (30%) group (p=0,024). Risk of re-recurrence after 1st local recurrence was more than 4 times higher in the metastatic than non-metastatic group (p=0.05). No differences were observed in the 2 groups with respect to age, sex, site, stage, treatment, follow-up and mortality.

Conclusions: Overexpression of urokinase-type plasminogen activation system in this study associated with an increased risk of lung metastases, local recurrence and local re-recurrence. Evaluation of urokinase-type plasminogen activation system expression levels may identify a subgroup of patients with increased risk of relapse

Despite local treatment with systemic chemotherapy in Ewing’s sarcoma family tumours (ESFT), patients with detectable metastases at presentation have a markedly worse prognosis than those with apparently localised disease. We investigated the clinical, pathological and laboratory differences in 888 patients with ESFT, 702 with localised disease and 186 with overt metastases at presentation, seen at our institution between 1983 and 2006.

Multivariate analyses showed that location in the pelvis, a high level of serum lactic dehydrogenase, the presence of fever and a short interval between the onset of symptoms and diagnosis were indicative of metastatic disease. The rate of overt metastases at presentation was 10% without these four risk factors, 22.7% with one, 31.4% with two, and 50% for those with three or four factors. We concluded that in ESFT the site, the serum level of lactic dehydrogenase, fever, and the interval between the onset of symptoms and diagnosis are indicators of tumours having a particularly aggressive metastatic behaviour.

Between 1986 and 2002, 42 patients with synchronous multifocal osteosarcoma were treated with two different protocols of neoadjuvant chemotherapy. When feasible, the primary and secondary tumours were excised as a combined procedure.

After initial chemotherapy 26 patients were excluded from simultaneous excision of all their secondary bone lesions as their disease was too advanced. In 12 patients only isolated excision of the primary lesion was possible. For 16 patients simultaneous operations were conducted to excise the primary and secondary lesions. This involved two supplementary sites in 15 patients and four additional sites in one patient. Of these, 15 attained remission but 12 relapsed and died (11 within two years). Three patients remained disease-free at five, six and 17 years. The histological response to pre-operative chemotherapy of the primary and secondary lesions was concordant in 13 of the 16 patients who underwent simultaneous operations at more than one site.

The prognosis for synchronous multifocal osteosarcoma remains poor despite combined chemotherapy and surgery. The homogeneous histological responses in a large proportion of the primary and secondary lesions implies that synchronous multifocal osteosarcoma tumours are not multicentric in origin, but probably represent bone-to-bone metastases from a single tumour.

Introduction: Allograft-Prosthesis Composite represents a reliable option for proximal femur replacement after resection for bone tumor. It provides advantages over megaprostheses because of better soft tissue repair and superior abduction strength, quality of gait, hip stability, and load transfer by healed bone rather than prosthetic stem, with potential impact on implant survival. Purpose of this paper was to review details of the surgical technique and results.

Methods: A retrospective study of 62 patients who had resection of the proximal femur because of a bone tumor and reconstruction with an Allograft-Prosthesis Composite was undertaken. The basic surgical technique consisted of an uncemented tapered long stem prosthesis (i.e. Wagner or Wagner-type stem) cemented in the allograft and press-fitted in the host bone, achieving bone-bone contact through a transverse osteotomy. Details of the surgical technique included: 1) accurate preoperative planning, canal sizing and implant selection; 2) under-reaming of the proximal 5–10 mm of the host medullary canal, depending upon bone quality and diameter of the selected stem; 3) allograft preparation and prosthesis cementation in the allograft; 4) introduction of the composite implant, pressfitted in host medullary canal, until bone-bone contact is achieved; 5) careful repair of abductors and iliopsoas to corresponding allograft tendon insertions.

Key points for successful fixation are absolute rotational stability and satisfactory circumferential bone-bone contact at the time of surgery.

Postoperative regimen consisted of hip, followed by progressive bracing and toe-touch weight-bearing for 6 weeks, weight-bearing.

Results: There were 2 septic failures. Two patients developed asymptomatic nonunion. There were no dislocations. Most common complication was fracture of the allograft greater trochanter (30%), which required surgery in only 1 case and never substantially affected function. The incidence of trochanteric fracture decreased from 63% in the first 27 patients to 5% in the following 35 patients by switching implant design from a valgus 145° neck angle to 135° neck angle, improving offset and abductors function. Bone grafting of the allograft-host union was required in 10% of the cases.

According to MSTS, results were satisfactory in 90% of the patients, with average score 91% (75%–96%).

Discussion and Conclusion: Allograft-Prosthesis Composite is a successful procedure for reconstruction of the proximal femur. Careful surgical technique is the key to excellent function and low complication rate.

Multiple Hereditary Exostoses is a rare skeletal chondrodysplasia characterized by the presence of a variable number of osteochondromas, usually mostly affecting the long bones but possibly located anywhere. Appearance and growth of exostoses is parallel to the patient’s growth, essentially ending when skeletal maturity is reached.

Its clinical expression is well known and may vary from asymptomatic to severe deformities and is rarely complicated by trasformation to secondary chondrosarcoma (0.5–2%). Research in the field of genetics has lead to identification of 2 responsible genes, EXT1 and EXT2, located respectively on chromosome 8 and 11, both coding for transmembrane glycoproteins involved in the synthesis of heparan-sulfate chains.

A third rare abnormality (EXT3) has been located on chromosome 19 but the responsible gene has not been identified yet.

Seems logical to investigate the genetic basis of the disease and the correlation with clinical aspects, either severity of the deformities and consequent functional impairment and potential for chondrosarcoma.

At the authors’ Institution a total of 550 patients with Multiple Hereditary Exostoses are presently filed. Genetic screening by DHPLC (Denaturing High Performance Liquid Chromatography) and clinicoradiographic orthopedic evaluation has been carried out on 200 patients. So far, 45 mutations have been identified (35 in EXT1 and 10 in EXT2) in 167 patients, 20 of which presented with negative family history and are therefore considered “de-novo” mutations.

Comparison of the clinical data and prospective long term follow-up will possibly clarify different prognosis and risk of secondary chondrosarcoma for different genotypes.

Aims: Purpose of this study was to obtain long term follow-up in patients with Osteofibrous Dysplasia (OFD), in order to investigate natural history of the disease, late results of treatment, and potential risk of Adaman-tinoma development in this setting. Methods: A retrospective study of 48 patients with histologically proven OFD observed at our Institution between 1900 and 1997 was undertaken. Clinico-pathologic features of all cases were reviewed and found consistent with OFD. A clinical status update and current radiographs were obtained in all patients. A subgroup of 21 patients with minimum follow-up of 20 years (21 to 44 years, average 27) was analysed for functional result and adamantinoma development. Functional result according to MTS-ISOLS score correlated with surgical aggressiveness. Results: Best results were observed in patients that received a single biopsy or curettage; worse results were seen after multiple resections or osteotomies and associated with complications as infection or compartment syndrome. No patients had current symptoms or significant symptoms changes nor physical findings, radiographic clues or subsequent radiographic changes suggesting adamantinoma development. Conclusions: OFD is a benign condition; the natural history of the disease has minimal consequences in the adult life. Surgical treatment is usually not necessary and may actually worsen the result because of the potential for severe complications.

The relationship with adamantinoma remains unclear, follow-up is suggested.

Aims: Purpose of this study was to evaluate the results of a staged revision technique in the treatment of deep infection after limb salvage surgery for bone tumors and to identify factors possibly affecting the outcome. Methods: A retrospective study of 19 consecutive patients with an infected bone tumor reconstruction treated at our Institution in the period 1986–1997 was undertaken. All the patients underwent staged revision (two stages in 13 cases, three stages in 5, four stages in 1) using one or more antibiotic loaded cement spacers after debridement and partial (10 cases) or complete (9 cases) removal of the original implant. Postoperatively, all the patients received oral or parenteral antibiotics for a minimum of 4 weeks. Delayed reimplantation was performed in 15 cases, average time to reimplantation being 7 months (4–14). A minimum follow up of 3 years was available in all patients. Cultures identiþed S. Epider-midis in 12 cases (63%), S. Aureus in 4 (21%), mixed organisms in 2 (11%), and were negative in 1 case (5%) despite clinical evidence of infection. Results: At a minimum follow-up of 3 years, 13 patients were continuously infection-free (68%) while 6 relapsed (32%). Two of the 6 relapses were cleared by amputation while 4 remained infected. Average functional result of infection-free patients according to the International Society Of Limb Salvage (ISOLS) was 71% (21.2 points), ranging from 60% to 80% (18 to 24 points). Conclusions: Staged revision with antibiotic loaded cement spacer for infected bone tumor reconstruction is a demanding and expensive technique requiring prolonged inability. Overall success rate in this series approaches 70%. Complete removal of the infected implant, microbiology, appropriate antibiotic selection, and negative cultures before reimplantation are crucial.