Aims. After intercalary resection of a bone tumour from the femur, reconstruction with a vascularized fibular graft (VFG) and massive allograft is considered a reliable method of treatment. However, little is known about the long-term outcome of this procedure. The aims of this study were to determine whether the morbidity of this procedure was comparable to that of other reconstructive techniques, if it was possible to achieve a satisfactory functional result, and whether biological reconstruction with a VFG and massive allograft could achieve a durable, long-lasting reconstruction. Patients and Methods. A total of 23 patients with a mean age of 16 years (five to 40) who had undergone resection of an intercalary bone tumour of the femur and reconstruction with a VFG and allograft were reviewed clinically and radiologically. The mean follow-up was 141 months (24 to 313). The mean length of the fibular graft was 18 cm (12 to 29). Full weight-bearing without a brace was allowed after a mean of 13 months (seven to 26). Results. At final follow-up, the mean Musculoskeletal Tumor Society Score of 22 evaluable patients was 94% (73 to 100). Eight major complications, five fractures (21.7%), and three nonunions (13%) were seen in seven patients (30.4%). Revision-free survival was 72.3% at five, ten, and 15 years, with fracture and nonunion needing surgery as failure endpoints. Overall survival, with removal of allograft or amputation as failure endpoints, was 94.4% at five, ten, and 15 years. Discussion. There were no complications needing surgical revision after five years had elapsed from surgery, suggesting that the mechanical strength of the implant improves with time, thereby decreasing the risk of complications. In young patients with an intercalary bone tumour of the femur, combining a VFG and massive allograft may result in a reconstruction that lasts a lifetime. Cite this article: Bone Joint J 2018;100-B:378–86

We reviewed 23 patients with a giant-cell tumour (GCT) of bone and histologically-proven lung metastases at a mean of 11.9 years (3 to 24.5) after the original diagnosis. The mean age of the patients at diagnosis was 27.3 years (9 to 61); the male to female ratio was 0.9:1. The most common primary site was the distal radius. The mean interval between the onset of the tumour and the detection of lung metastases was 4.1 years (0 to 24). There had been local recurrence in 19 (83%) either before or at the time of diagnosis of lung metastasis. Surgical resection alone was the preferred treatment for lung metastases in 70% and resulted in 18 patients (76%) being free from disease when last reviewed. One patient had the spontaneous regression of lung metastases and was free from recurrence 16 years later. Sixteen patients (69.7%) have survived and four (17.4%) have died from progression of the tumour. Three other deaths were not related to the tumour. Local recurrence and a primary lesion at the distal radius seem to be associated with an increased risk of lung metastases. Repeated surgical resection of lung metastases gave a high rate of survival

1. A case of glomus tumour of the subcutaneous tissues of the knee which presented with extreme pain is described. Successful removal of the tumour led to complete relief of pain and return to normal function. 2. The histological features are given. 3. The literature is briefly reviewed

Aims. Intercalary allografts following resection of a primary diaphyseal tumour have high rates of complications and failures. At our institution intercalary allografts are augmented with intramedullary cement and fixed using compression plating. Our aim was to evaluate their long-term outcomes. Patients and Methods. A total of 46 patients underwent reconstruction with an intercalary allograft between 1989 and 2014. The patients had a mean age of 32.8 years (14 to 77). The most common diagnoses were osteosarcoma (n = 16) and chondrosarcoma (n = 9). The location of the tumours was in the femur in 21, the tibia in 16 and the humerus in nine. Function was assessed using the Musculoskeletal Tumor Society (MSTS) scoring system and the Toronto Extremity Salvage Score (TESS). The survival of the graft and the overall survival were assessed using the Kaplan-Meier method. Results. The median follow-up was 92 months (4 to 288). The mean MSTS 87 score was 29.1 (19 to 35), the mean MSTS 93 score was 82.2 (50 to 100) and the mean TESS score was 81.2 (43 to 100). Overall survival of the allograft was 84.8%. A total of 15 patients (33%) had a complication. Five allografts were revised for complications and one for local recurrence. Conclusion. Intercalary allografts augmented with intramedullary cement and compression plate fixation provide a reliable and durable method of reconstruction after the excision of a primary diaphyseal bone tumour, with high levels of function and satisfaction. . Cite this article: Bone Joint J 2017;99-B:973–8

Amalignant peripheral nerve-sheath tumour developed in the right S1 nerve root in a man aged 30 causing back pain and sciatica. CT and MRI revealed a destructive tumour of the sacrum invading the retroperitoneal space. The tumour was not resectable with an adequate margin. Chemotherapy, consisting of high-dose ifosfamide followed by a combination of vincristine, doxorubicin and cyclophosphamide, was given with success. Malignant peripheral nerve-sheath tumours are thought to respond weakly to chemotherapy, but the response in our patient was complete

1. A twenty-six-year-old woman was paraplegic because of a benign giant-cell tumour which had destroyed the body of the twelfth thoracic vertebra completely and the bodies of the eleventh thoracic and first lumbar vertebrae partially. The tumour had expanded into both pleural cavities and displaced the aorta forward and to the left. The extent and topography of the tumour were evaluated before operation by angiography. The function of the spinal cord had not been improved significantly by laminectomy. It was therefore decided to attempt extirpation of the tumour by removing all remaining parts of the three vertebrae involved. 2. After the removal of the tumour, only the spinal cord with the thecal sac bridged the gap between the tenth thoracic and second lumbar vertebrae. The gap was bridged with struts of cortical bone from both tibiae and with two strong plates, all secured with steel wire. The metal was removed five months later because it had become loose. Gradual shortening and angulation of the spine then occurred, together with progressive resorption of the cortical grafts. Nevertheless, the operation restored the function of the spinal cord; the patient regained ability to walk and full control of the bladder

A giant cell tumour is a primary lesion of bone of intermediate severity. Its histogenesis is unclear. In a few cases pulmonary metastases have been described. Multiple skeletal metastases in the absence of sarcomatous change have been observed. We present a case report of a 25-year-old woman with a recurrent giant cell tumour of the distal fibula. After a second recurrence and six years after the initial diagnosis, she rapidly developed multiple bony metastases. The outcome was fatal

We investigated the use of hypoxia-inducible factor (HIF) proteins as prognostic markers in chondrosarcoma and the relationship of HIF to the biological characteristics of cartilage tumours. The expression of HIF-1α, HIF-2α, proliferating cell nuclear antigen (PCNA) and microvessel density (MVD) were measured immunohistochemically in 29 specimens of cartilage tumour. There was no HIF-1α and HIF-2α staining in any of the nine benign cartilage tumours. In 20 specimens of chondrosarcoma, the rate of HIF-1α and HIF-2α expression was 40% and 25%, respectively. The tumour size (≥ 8 cm), histological grade (grade 2 and grade 3) surgical margin (marginal and intralesional) and HIF-1α expression (positive) correlated significantly with a shorter disease-free survival. There was a significant association between HIF-1α and the MVD and a strong trend towards a correlation between HIF-1α and the PCNA index or histological grade. Our findings suggest that HIF-1α protein may be a useful objective marker in the assessment of the prognosis in chondrosarcoma, since it plays an important role in tumour angiogenesis and cell proliferation

We treated 98 patients with peri-acetabular tumours by resection and reconstruction with a custom-made pelvic endoprosthesis. The overall survival of the patients was 67% at five years, 54% at ten years and 51% at 30 years. One or more complications occurred in 58.1% of patients (54), of which infection was the most common, affecting 30% (28 patients). The rate of local recurrence was 31% (29 patients) after a mean follow-up of 71 months (11 to 147). Dislocation occurred in 20% of patients (19). Before 1996 the rate was 40.5% (17 patients) but this was reduced to 3.9% (two patients) with the introduction of a larger femoral head. There were six cases of palsy of the femoral nerve with recovery in only two. Revision or excision arthroplasty was performed in 23.7% of patients (22), principally for uncontrolled infection or aseptic loosening. Higher rates of death, infection and revision occurred in men. This method of treatment is still associated with high morbidity. Patients should be carefully selected and informed of this pre-operatively

We evaluated the construct validity of the Musculoskeletal Tumour Society rating scale (Enneking score) as a functional measure for patients with sarcoma involving the upper limb. We compared the Enneking score by examining the correlation between two patient-derived outcome measures, the Disability of the Arm, Shoulder, and Hand (DASH) questionnaire and the Medical Outcomes Study Short Form-36 (SF-36) as indicators of functional status in 40 patients with malignant or aggressive benign bone and soft-tissue tumours of the upper limb who had undergone surgical treatment. The frequency distributions were similar among the three scoring systems. As for the validity, Spearman’s rank correlation coefficient of the Enneking score to the DASH questionnaire was −0.79 and that of the Enneking to the SF-36 subscales ranged from 0.38 to 0.60. Despite being a measure from the surgeon’s perspective, the Enneking score was shown to be a valid indicator of physical disability in patients with malignant or aggressive benign tumours of the upper limb and reflected their opinion

We hypothesised that the use of computer navigation-assisted surgery for pelvic and sacral tumours would reduce the risk of an intralesional margin. We reviewed 31 patients (18 men and 13 women) with a mean age of 52.9 years (13.5 to 77.2) in whom computer navigation-assisted surgery had been carried out for a bone tumour of the pelvis or sacrum. There were 23 primary malignant bone tumours, four metastatic tumours and four locally advanced primary tumours of the rectum. The registration error when using computer navigation was <  1 mm in each case. There were no complications related to the navigation, which allowed the preservation of sacral nerve roots (n = 13), resection of otherwise inoperable disease (n = 4) and the avoidance of hindquarter amputation (n = 3). The intralesional resection rate for primary tumours of the pelvis and sacrum was 8.7% (n = 2): clear bone resection margins were achieved in all cases. At a mean follow-up of 13.1 months (3 to 34) three patients (13%) had developed a local recurrence. The mean time alive from diagnosis was 16.8 months (4 to 48). . Computer navigation-assisted surgery is safe and has reduced our intralesional resection rate for primary tumours of the pelvis and sacrum. We recommend this technique as being worthy of further consideration for this group of patients. Cite this article: Bone Joint J 2013;95-B:1417–24

Giant-cell tumour of the tendon sheath, also called pigmented villonodular synovitis, is a benign tumour with a high incidence of recurrence. We have tried to identify risk factors for recurrence. Of the 48 patients included in the study, 14 received radiotherapy after surgery. Only two (4%) had a recurrence. This compares favourably with previously reported incidences of between 25% and 45%

1. The clinical appearance and histological structure of a myo-epithelial tumour of a sweat gland of the hand is described. 2. A review of previously published papers is given. 3. It is suggested that trauma may play a part in the causation of these tumours

Aims. The purpose of this retrospective study was to differentiate between the MRI features of normal post-operative change and those of residual or recurrent disease after intralesional treatment of an atypical cartilage tumour (ACT)/grade I chondrosarcoma. Patients and Methods. We reviewed the case notes, radiology and histology of 75 patients, who had been treated for an ACT/grade I chondrosarcoma by curettage, phenolisation and bone allografting between 1994 and 2005. The first post-operative Gd-enhanced MRI scan was carried out within one year of surgery. Patients had a minimum of two scans and a mean follow-up of 72 months (13 to 169). Further surgery was undertaken in cases of suspected recurrence. Results. In 14 patients (18.6%) a second procedure was undertaken after a mean period of 59 months (8 to 114). Radio frequency ablation (RFA) was used in lesions of < 10 mm and curettage, phenolisation and bone grafting for those ≥ 10 mm. Only six of these (8% of total) had a histologically-proven recurrence. No increase in tumour grade was seen at time of recurrence. Conclusion. Based on this study, we have been able to classify the post-operative MRI appearances into four groups. These groups differ in follow-up, and have a different risk of recurrence of the lesion. Follow-up and treatment vary for the patients in each group. We present a flow diagram for the appropriate and safe follow-up for this specific group of patients. Cite this article: Bone Joint J 2016;98-B:1674–81

In 45 patients we assessed the functional results and complications for three different reconstructive procedures after resection of primary tumors of the proximal humerus. An osteoarticular allograft was used in 11, a clavicula pro humero operation in 15 and a tumour prosthesis in 19. The glenoid was resected with the proximal humerus in 25 patients. The axillary nerve was resected in 42 patients. The complication rate was lowest after reconstruction with a tumour prosthesis. The clavicula pro humero operation resulted in the most revisions. Cumulative survival rates for all the reconstructive procedures were similar. At follow-up at two years the functional results for the three reconstructive procedures were the same with a mean functional rating of 79% (Musculoskeletal Tumor Society). Excision of the glenoid had no influence on the functional result. Our findings indicate that the use of a tumour prosthesis is the most reliable limb-salvage procedure for the proximal humerus. The clavicula pro humero is an appropriate procedure if a prosthesis cannot be used

Despite extensive experience with prosthetic replacement for the reconstruction of limbs following juxta-articular resection of tumours, there are few reports of prosthetic replacement of the distal radius. We present two cases of massive bone defects of the distal radius in which alumina ceramic prosthetic replacements were used. We evaluated the patients more than ten years after the procedure. Both patients had degenerative changes to the wrist. This, however, was not associated with pain or decreased function, and both had returned to their previous occupation after surgery. When a patient has a massive defect of the distal radius, reconstruction using a ceramic prosthesis is a reasonable alternative to using autograft. This method of treatment results in little pain, a moderate range of movement and satisfactory function

A 15-year-old girl presented with a very large desmoid tumour in her buttock; it extended into the pelvis and thigh and would have required hindquarter amputation for its removal. This was not performed and the tumour underwent spontaneous regression. Fourteen years later the patient is alive and well. The suggestion is made that in some cases a more expectant approach to treatment might be justified for what is essentially a non-malignant condition

The best method of reconstruction after resection of malignant tumours of the tibial diaphysis is unknown. In the absence of any long-term studies analysing the results of intercalary endoprosthetic replacement, we present a retrospective review of 18 patients who underwent limb salvage using a tibial diaphyseal endoprosthetic replacement following excision of a malignant bone tumour. There were ten men and eight women with a mean age of 42.5 years (16 to 76). Mean follow-up was 58.5 months (20 to 141) for all patients and 69.3 months (20 to 141) for the 12 patients still alive. Cumulative patient survival was 59% (95% confidence interval (CI) 32 to 84) at five years. Implant survival was 63% (95% CI 35 to 90) at ten years. Four patients required revision to a proximal tibial replacement at a mean follow-up of 29 months (10 to 54). Complications included metastases in five patients, aseptic loosening in four, peri-prosthetic fracture in two, infection in one and local recurrence in one. The mean Musculoskeletal Tumor Society score and the mean Toronto Extremity Salvage Score were 23 (17 to 28) and 74% (53 to 91), respectively. Although rates of complication and revision were high, custom-made tibial diaphyseal replacement following resection of malignant bone tumours enables early return to function and provides an attractive alternative to other surgical options, without apparent compromise of patient survival

Giant-cell tumours of the sacrum are difficult to treat. Surgery carries a high risk of morbidity, local recurrence and mortality. Radiation is effective in some patients, but has a risk of malignant change. We evaluated the effectiveness of serial arterial embolisation as an alternative to surgery. Five patients with giant-cell tumours of the sacrum which had been primarily treated by serial embolisation were retrospectively reviewed for changes in the size of the tumour. In four the symptoms resolved with full return of function and arrest in the growth of the tumour. They remained free from growth, recurrence, or metastases at follow-up (4 to 17 years). One patient died from metastatic disease within 18 months of the initial diagnosis

The level of bone resection for osteosarcoma depends on the pre-operative evaluation of the extent of intramedullary tumour. We compared the accuracy of magnetic resonance imaging (MRI), computerised tomography (CT), and isotope bone scanning with the actual extent of the tumour in the resected specimens from 34 patients with primary osteosarcoma of a long bone. The extent of medullary tumour was defined accurately in 23 of 24 MRI scans (96%) and 24 of 32 CT scans (75%). A flexion contracture of a joint close to the tumour was an important cause for inaccurate measurements from both MRI and CT scans. Isotope bone scanning was inaccurate: its role is now confined to detecting skeletal metastases and skip lesions