Dedifferentiated chordoma is a rare and aggressive variant of the conventional tumour in which an area undergoes transformation to a high-grade lesion, typically fibrous histiocytoma, fibrosarcoma, and rarely, osteosarcoma or rhabdomyosarcoma. The dedifferentiated component dictates overall survival, with smaller areas of dedifferentiation carrying a more favourable prognosis. Although it is more commonly diagnosed in recurrences and following radiotherapy, there have been a few reports of spontaneous development. We describe four such cases, which were diagnosed de novo following primary excision, and discuss the associated clinical and radiological features

Skeletal metastases from hepatocellular carcinoma are highly destructive vascular lesions which severely reduce the quality of life. Pre-existing liver cirrhosis presents unique challenges during the surgical management of such lesions. We carried out a retrospective study of 42 patients who had been managed surgically for skeletal metastases from hepatocellular carcinoma affecting the appendicular skeleton between January 2000 and December 2006. There were 38 men and four women with a mean age of 60.2 years (46 to 77). Surgery for a pathological fracture was undertaken in 30 patients and because of a high risk of fracture in 12. An intralesional surgical margin was achieved in 36 and a wide margin in six. Factors influencing survival were determined by univariate and multivariate analyses. The survival rates at one, two and three years after surgery were 42.2%, 25.8% and 19.8%, respectively. The median survival time was ten months (95% confidence interval 6.29 to 13.71). The number of skeletal metastases and the Child-Pugh grade were identified as independent prognostic factors by Cox regression analysis. The method of management of the hepatocellular carcinoma, its status in the liver, the surgical margin for skeletal metastases, the presence of a pathological fracture and adjuvant radiotherapy were not found to be significantly related to the survival of the patient, which was affected by hepatic function, as represented by the Child-Pugh grade

We have investigated the oncological outcome of 63 patients with soft-tissue sarcomas of the hand managed at three major centres in the United Kingdom. There were 44 males and 19 females with a mean age of 45 years (11 to 92). The three most common diagnoses were synovial sarcoma, clear cell sarcoma and epithelioid sarcoma. Local excision was carried out in 45 patients (71%) and amputation in 18 (29%). All those treated by amputation had a wide margin of excision but this was only achieved in 58% of those treated by local excision. The risk of local recurrence was 6% in those treated by amputation compared with 42% for those who underwent attempted limb salvage. An inadequate margin of excision resulted in a 12 times greater risk of local recurrence when compared with those in whom a wide margin of excision had been achieved. We were unable to demonstrate any role for radiotherapy in decreasing the risk of local recurrence when there was an inadequate margin of excision. Patients with an inadequate margin of excision had a much higher risk of both local recurrence and metastasis than those with wide margins. The overall survival rate at five years was 87% and was related to the grade and size of the tumour and to the surgical margin. We have shown that a clear margin of excision is essential to achieve local control of a soft-tissue sarcoma in the hand and that failure to achieve this results in a high risk of both local recurrence and metastastic disease

We investigated whether our policy of routine re-excision of the tumour bed after an unplanned excision of a soft-tissue sarcoma was justified. Between April 1982 and December 2005, 2201 patients were referred to our hospital with the diagnosis of soft-tissue sarcoma, of whom 402 (18%) had undergone an unplanned excision elsewhere. A total of 363 (16.5%) were included in this study. Each patient was routinely restaged and the original histology was reviewed. Re-excision was undertaken in 316 (87%). We analysed the patient, tumour and treatment factors in relation to local control, metastasis and overall survival. Residual tumour was found in 188 patients (59%). There was thus no residual disease in 128 patients of whom 10% (13) went on to develop a local recurrence. In 149 patients (47%), the re-excision specimen contained residual tumour, but it had been widely excised. Local recurrence occurred in 30 of these patients (20%). In 39 patients (12%), residual tumour was present in a marginal resection specimen. Of these, 46% (18) developed a local recurrence. A final positive margin in a high-grade tumour had a 60% risk of local recurrence even with post-operative radiotherapy. Metastases developed in 24% (86). The overall survival was 77% at five years. Survival was related to the grade of the tumour and the finding of residual tumour at the time of re-excision. We concluded that our policy of routine re-excision after unplanned excision of soft-tissue sarcoma was justified in view of the high risk of finding residual tumour

1. Five cases of fibrosarcoma arising after radiotherapy in cases of ankylosing spondylitis are reported. 2. The relationship of the tumours to irradiation is discussed. 3. Objective neurological signs arising in the lower limbs of patients with ankylosing spondylitis after a latent period following radiotherapy may indicate an underlying sarcoma

We evaluated the results of fibular centralisation as a stand alone technique to reconstruct defects that occurred after resection of tumours involving the tibial diaphysis and distal metaphysis. Between January 2003 and December 2006, 15 patients underwent excision of tumours of the tibial diaphysis or distal metaphysis and reconstruction by fibular centralisation. Their mean age was 17 years (7 to 40). Two patients were excluded; one died from the complications of chemotherapy and a second needed a below-knee amputation for a recurrent giant-cell tumour. A total of 13 patients were reviewed after a mean follow-up of 29 months (16 to 48). Only 16 of 26 host graft junctions united primarily. Ten junctions in ten patients needed one or more further procedure before union was achieved. At final follow-up 12 of the 13 patients had fully united grafts; 11 walked without aids. The mean time to union at the junctions that united was 12 months (3 to 36). The mean Musculoskeletal Tumor Society Score was 24.7 (16 to 30). Fibular centralisation is a durable reconstruction for defects of the tibial diaphysis and distal metaphysis with an acceptable functional outcome. Stable osteosynthesis is the key to successful union. Additional bone grafting is recommended for patients who need postoperative radiotherapy

We reviewed 20 patients who had undergone a Coonrad-Morrey total elbow arthroplasty after resection of a primary or metastatic tumour from the elbow or distal humerus between 1980 and 2002. Eighteen patients underwent reconstruction for palliative treatment with restoration of function after intralesional surgery and two after excision of a primary bone tumour. The mean follow-up was 30 months (1 to 192). Five patients (25%) were alive at the final follow-up; 14 (70%) had died of their disease and one of unrelated causes. Local control was achieved in 15 patients (75%). The mean Mayo Elbow Performance Score improved from 22 (5 to 45) to 75 points (55 to 95). Four reconstructions (20%) failed and required revision. Seven patients (35%) had early complications, the most frequent being nerve injury (25%). There were no infections or wound complications although 18 patients (90%) had radiotherapy, chemotherapy or both. The Coonrad-Morrey total elbow arthroplasty provides good relief from pain and a good functional outcome after resection of tumours of the elbow. The rates of complications involving local recurrence of tumour (25%) and nerve injury (25%) are of concern

Aims

Iliosacral sarcoma resections have been shown to have high rates of local recurrence (LR) and poor overall survival. There is also no universal classification for the resection of pelvic sarcomas invading the sacrum. This study proposes a novel classification system and analyzes the survival and risk of recurrence, when using this system.

We evaluated 31 patients who were treated with a non-vascularised fibular graft after resection of primary musculoskeletal tumours, with a median follow-up of 5.6 years (3 to 26.7 years). Primary union was achieved in 89% (41 of 46) of the grafts in a median period of 24 weeks. All 25 grafts in 18 patients without additional chemotheraphy and/or radiotherapy achieved primary union, compared with 16 of the 21 grafts (76%; 13 patients) with additional therapy (p = 0.017). Radiographs showed an increase in diameter in 70% (59) of the grafts. There were seven fatigue fractures in six patients, but only two needed treatment. Non-vascularised fibular transfer is a simpler, less expensive and a shorter procedure than the use of vascularised grafts and allows remodelling of the fibula at the donor site. It is a biological reconstruction with good long-term results, and a relatively low donor site complication rate of 16%

Aims

Patients with soft-tissue sarcoma (STS) who undergo unplanned excision (UE) are reported to have worse outcomes than those who undergo planned excision (PE). However, others have reported that patients who undergo UE may have similar or improved outcomes. These discrepancies are likely to be due to differences in characteristics between the two groups of patients. The aim of the study is to compare patients who underwent UE and PE using propensity score matching, by analyzing data from the Japanese Bone and Soft Tissue Tumor (BSTT) registry.

1. Problems of management of a pathological fracture of a major long bone through a metastatic cancer deposit are outlined. The relatively short life expectancy of these patients is stressed. 2. The results of treatment of thirty-six consecutive fractures of this type by internal fixation and radiotherapy, and of similar treatment used "prophylactically" on seven occasions, are reviewed. 3. Four-fifths of the patients with lower limb fractures became ambulant with crutches and were able to return home. 4. "Prophylactic" internal fixation and radiotherapy appear to be helpful in selected cases. 5. The theoretical dangers and difficulties of the technique are discussed. They are easily surmountable and have probably been over-emphasised in the past. 6. It is concluded that this is the best method at present available for dealing with this difficult problem

Between 1955 and 1989 we treated 24 patients (17 women and seven men) with giant-cell tumours of the spine at the Mayo Clinic. Their mean age was 30 years and the mean follow-up time was 12.4 years. Pain was the presenting symptom in all and half had a neurological deficit. The cervical, thoracic, and lumbar spines were equally involved. The tumours recurred in five of the 14 patients treated by one-stage surgery and in five of the ten treated by two-stage surgery. Seven patients received adjuvant radiotherapy, one for the primary lesion and six for recurrent lesions. Surgical management was by curettage or en bloc excision depending on the location and the extent of the tumour. Because of the risk of sarcomatous transformation, radiation therapy should be reserved for patients with incomplete excision or for those with local recurrence

1. Fifty-five giant-cell tumours of bone are described in fifty-two patients of whom thirty-five were women. The highest incidence was in the third and fourth decades. 2. The tumours were characteristic radiologically: they all occurred in skeletally mature patients, and chiefly in the expanded extremities of long bones. 3. Histological grading was not done, the tumours being designated typical or malignant. 4. Curettage and graft, resection, radiotherapy and amputation were the basic forms of treatment. Amputation has an occasional place in primary treatment and radiotherapy should be confined to tumours in surgically inaccessible sites. 5. Resection is the method of choice for tumours in suitable sites, such as the upper end of the fibula ; careful curettage and grafting should be used to preserve the function of a large joint. 6. Recurrences within two years should be treated by limited resection without preliminary biopsy. Late recurrences must have a biopsy taken to establish the diagnosis before planning treatment

Aims

Current literature suggests that survival outcomes and local recurrence rates of primary soft-tissue sarcoma diagnosed in the very elderly age range, (over 90 years), are comparable with those in patients diagnosed under the age of 75 years. Our aim is to quantify these outcomes with a view to rationalizing management and follow-up for very elderly patients.

Aims

Giant cell tumour of bone (GCTB) treatment changed since the introduction of denosumab from purely surgical towards a multidisciplinary approach, with recent concerns of higher recurrence rates after denosumab. We evaluated oncological, surgical, and functional outcomes for distal radius GCTB, with a critically appraised systematic literature review.

Aims

BoneMaster is a thin electrochemically applied hydroxyapatite (HA) coating for orthopaedic implants that is quickly resorbed during osseointegration. Early stabilization is a surrogacy marker of good survival of femoral stems. The hypothesis of this study was that a BoneMaster coating yields a fast early and lasting fixation of stems.

Aims

While a centralized system for the care of patients with a sarcoma has been advocated for decades, regional variations in survival remain unclear. The aim of this study was to investigate regional variations in survival and the impact of national policies in patients with a soft-tissue sarcoma (STS) in the UK.

Breast cancer is generally managed surgically with adjuvant agents which include hormone therapy, chemotherapy, radiotherapy and bisphosphonate therapy. However, some of these adjuvant therapies may cause adverse events, including wound infection, neutropenia, bone marrow suppression and fever. The simultaneous presentation of osteonecrosis and osteomyelitis has not previously been described in patients with breast cancer undergoing hormone therapy and chemotherapy. We report a patient with breast cancer who developed bone infarcts in both legs as well as osteomyelitis in the right distal tibia after treatment which included a modified radical mastectomy, hormone therapy and chemotherapy. Simultaneous osteonecrosis and osteomyelitis should be considered in patients with breast cancer who are receiving chemotherapy and hormone therapy who present with severe bone pain, especially if there have been infective episodes during treatment

Aims

The aim of this study was to inform the epidemiology and treatment of slipped capital femoral epiphysis (SCFE).