We identified eight patients of 2900 with a primary malignant bone tumour who had coexisting neurofibromatosis type 1. This was a much higher incidence than would be expected by chance. The patients had a mean age of 22.4 years (9 to 54): five were male. Two patients subsequently developed a second bone sarcoma, one of which was radiation induced. Four of the primary tumours were osteosarcomas, four were spindle-cell sarcomas and one a Ewing’s sarcoma. All the patients were treated with chemotherapy and surgery: six of the eight appear to be cured.

This study suggests a possible relationship between neurofibromatosis type 1 and the development of a bone sarcoma, the increased risk being estimated at eight times that of the normal population. We recommend that further research into this possible link should be considered.

The aim of this study was to investigate the occurrence of tissue hypoxia and apoptosis at different stages of tendinopathy and tears of the rotator cuff.

We studied tissue from 24 patients with eight graded stages of either impingement (mild, moderate and severe) or tears of the rotator cuff (partial, small, medium, large and massive) and three controls. Biopsies were analysed using three immunohistochemical techniques, namely antibodies against HIF-1α (a transcription factor produced in a hypoxic environment), BNip3 (a HIF-1α regulated pro-apoptotic protein) and TUNEL (detecting DNA fragmentation in apoptosis).

The HIF-1α expression was greatest in mild impingement and in partial, small, medium and large tears. BNip3 expression increased significantly in partial, small, medium and large tears but was reduced in massive tears. Apoptosis was increased in small, medium, large and massive tears but not in partial tears.

These findings reveal evidence of hypoxic damage throughout the spectrum of pathology of the rotator cuff which may contribute to loss of cells by apoptosis. This provides a novel insight into the causes of degeneration of the rotator cuff and highlights possible options for treatment.

The dismal outcome of tuberculosis of the spine in the pre-antibiotic era has improved significantly because of the use of potent antitubercular drugs, modern diagnostic aids and advances in surgical management. MRI allows the diagnosis of a tuberculous lesion, with a sensitivity of 100% and specificity of 88%, well before deformity develops. Neurological deficit and deformity are the worst complications of spinal tuberculosis. Patients treated conservatively show an increase in deformity of about 15°. In children, a kyphosis continues to increase with growth even after the lesion has healed. Tuberculosis of the spine is a medical disease which is not primarily treated surgically, but operation is required to prevent and treat the complications. Panvertebral lesions, therapeutically refractory disease, severe kyphosis, a developing neurological deficit, lack of improvement or deterioration are indications for surgery. Patients who present with a kyphosis of 60° or more, or one which is likely to progress, require anterior decompression, posterior shortening, posterior instrumented stabilisation and anterior and posterior bone grafting in the active stage of the disease. Late-onset paraplegia is best prevented rather than treated. The awareness and suspicion of an atypical presentation of spinal tuberculosis should be high in order to obtain a good outcome. Therapeutically refractory cases of tuberculosis of the spine are increasing in association with the presence of HIV and multidrug-resistant tuberculosis.

Metallosis is a rare cause of failure after total knee replacement and has only previously been reported when there has been abnormal metal-on-metal contact. We describe 14 patients (15 knees) whose total knee replacement required revision for a new type of early failure caused by extensive metallosis. A modification of a cementless rotating platform implant, which had previously had excellent long-term survival, had been used in each case. The change was in the form of a new porous-beaded surface on the femoral component to induce cementless fixation, which had been used successfully in the fixation of acetabular and tibial components. This modification appeared to have resulted in metallosis due to abrasive two-body wear. The component has subsequently been recalled and is no longer in use. The presentation, investigation, and findings at revision are described and a possible aetiology and its implications are discussed.

The August 2014 Oncology Roundup³⁶⁰ looks at: Anaesthesic modality does not affect outcomes in tumour surgery; infection predictors in orthopaedic oncology; sarcoma depth unimportant in survival; photon/proton radiotherapy surprisingly effective in chondrosarcoma control; total humerus replacement a success!; LDH simple predictor of survival in sarcoma; Denosumab again! and Oops procedures in triplicate.

Objectives

Matrix-assisted autologous chondrocyte transplantation (MACT) has been developed and applied in the clinical practice in the last decade to overcome most of the disadvantages of the first generation procedures. The purpose of this systematic review is to document and analyse the available literature on the results of MACT in the treatment of chondral and osteochondral lesions of the knee.

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.

The integrity of the spinal accessory nerve is fundamental to thoracoscapular function and essential for scapulohumeral rhythm. This nerve is vulnerable along its superficial course. This study assessed the delay in diagnosis and referral for management of damage to this nerve, clarified its anatomical course and function, and documented the results of repair. From examination of our records, 111 patients with lesions of the spinal accessory nerve were treated between 1984 and 2007. In 89 patients (80.2%) the damage was iatropathic. Recognition and referral were seldom made by the surgeon responsible for the injury, leading to a marked delay in instituting treatment. Most referrals were made for painful loss of shoulder function. The clinical diagnosis is straightforward. There is a characteristic downward and lateral displacement of the scapula, with narrowing of the inferior scapulohumeral angle and loss of function, with pain commonly present. In all, 80 nerves were explored and 65 were repaired. The course of the spinal accessory nerve in relation to the sternocleidomastoid muscle was constant, with branches from the cervical plexus rarely conveying motor fibres. Damage to the nerve was predominantly posterior to this muscle.

Despite the delay, the results of repair were surprising, with early relief of pain, implying a neuropathic source, which preceded generally good recovery of muscle function.

Vancomycin-supplemented allografts provide biological restoration of bone stock and sound fixation with a low incidence of re-infection. Experimental incorporation of these grafts is similar to allografts without vancomycin. However, the underlying biology remains unknown.

We report the first histological observations of vancomycin-supplemented impacted bone allografts in two reconstructions performed 14 and 20 months after revision surgery because of a periprosthetic fracture.

Areas of active bone remodelling (creeping substitution), as well as calcified bone trabeculae and graft particles embedded in dense fibrous tissue, were observed with osteoid and fibroconnective tissue surrounding polymethylmethacrylate particles.

These pathological findings are similar to those reported in allografts without vancomycin and support the hypothesis that high levels of vancomycin do not affect the incorporation of bone graft.

Aneurysmal bone cyst is a rare tumour-like lesion which develops during growth. Our aim was to determine the efficacy of the administration of percutaneous intralesional 3% polidocanol (hydroxypolyaethoxydodecan) as sclerotherapy.

Between July 1997 and December 2004 we treated 72 patients (46 males, 26 females) with a histologically-proven diagnosis of aneurysmal bone cyst, at various skeletal sites using this method. The sclerotherapy was performed under fluoroscopic guidance and general anaesthesia or sedation and local anaesthesia. The mean follow-up period was 34 months (26.5 to 80). The patients were evaluated using the Enneking system for functional assessment and all the lesions were radiologically quantified into four grades.

The mean age of patients was 15.6 years (3 to 38) and the mean number of injections was three (1 to 5). Ten patients were cured by a single injection. The mean reduction in size of the lesion (radiological healing) was found to be 76.6% (61.9% to 93.2%) with a mean clinical response of 84.5% (73.4% to 100%). Recurrence was seen in two patients (2.8%) within two years of treatment and both were treated successfully by further sclerotherapy.

Percutaneous sclerotherapy with polidocanol is a safe alternative to conventional surgery for the treatment of an aneurysmal bone cyst. It can be used at surgically-inaccessible sites and treatment can be performed on an out-patient basis.

Allografts of bone from the femoral head are often used in orthopaedic procedures. Although the donated heads are thoroughly tested microscopically before release by the bone bank, some surgeons take additional cultures in the operating theatre before implantation. There is no consensus about the need to take these cultures. We retrospectively assessed the clinical significance of the implantation of positive-cultured bone allografts. The contamination rate at retrieval of the allografts was 6.4% in our bone bank. Intra-operative cultures were taken from 426 femoral head allografts before implantation; 48 (11.3%) had a positive culture. The most frequently encountered micro-organism was coagulase-negative staphylococcus. Deep infection occurred in two of the 48 patients (4.2%). In only one was it likely that the same micro-organism caused the contamination and the subsequent infection.

In our study, the rate of infection in patients receiving positive-cultured allografts at implantation was not higher than the overall rate of infection in allograft surgery suggesting that the positive cultures at implantation probably represent contamination and that the taking of additional cultures is not useful.

We review the treatment of pelvic Ewing’s sarcoma by the implantation of extracorporeally-irradiated (ECI) autografts and compare the outcome with that of other reported methods.

We treated 13 patients with ECI autografts between 1994 and 2004. There were seven males and six females with a median age of 15.7 years (interquartile range (IQR) 12.2 to 21.7). At a median follow-up of five years (IQR 1.8 to 7.4), the disease-free survival was 69% overall, and 75% if one patient with local recurrence after initial treatment elsewhere was excluded. Four patients died from distant metastases at a mean of 17 months (13 to 23). There were three complications which required operative intervention; one was a deep infection which required removal of the graft. The functional results gave a mean Musculoskeletal Tumor Society score of 85% (60% to 97%), a mean Toronto extremity salvage score of 86% (69% to 100%) and a mean Harris hip score of 92 (67 to 100).

We conclude that ECI grafting is a suitable form of treatment for localised and resectable pelvic Ewing’s sarcoma.

Autologous chondrocyte implantation (ACI) and mosaicplasty are methods of treating symptomatic articular cartilage defects in the knee. This study represents the first long-term randomised comparison of the two techniques in 100 patients at a minimum follow-up of ten years. The mean age of the patients at the time of surgery was 31.3 years (16 to 49); the mean duration of symptoms pre-operatively was 7.2 years (9 months to 20 years). The lesions were large with the mean size for the ACI group being 440.9 mm² (100 to 1050) and the mosaicplasty group being 399.6 mm² (100 to 2000). Patients had a mean of 1.5 previous operations (0 to 4) to the articular cartilage defect. Patients were assessed using the modified Cincinnati knee score and the Stanmore-Bentley Functional Rating system. The number of patients whose repair had failed at ten years was ten of 58 (17%) in the ACI group and 23 of 42 (55%) in the mosaicplasty group (p < 0.001).

The functional outcome of those patients with a surviving graft was significantly better in patients who underwent ACI compared with mosaicplasty (p = 0.02).

Fungal peri-prosthetic infections of the knee and hip are rare but likely to result in devastating complications. In this study we evaluated the results of their management using a single-stage exchange technique. Between 2001 and 2011, 14 patients (ten hips, four knees) were treated for a peri-prosthetic fungal infection. One patient was excluded because revision surgery was not possible owing to a large acetabular defect. One patient developed a further infection two months post-operatively and was excluded from the analysis. Two patients died of unrelated causes.

After a mean of seven years (3 to 11) a total of ten patients were available for follow-up. One patient, undergoing revision replacement of the hip, had a post-operative dislocation. Another patient, undergoing revision replacement of the knee, developed a wound infection and required revision 29 months post-operatively following a peri-prosthetic femoral fracture.

The mean Harris hip score increased to 74 points (63 to 84; p < 0.02) in those undergoing revision replacement of the hip, and the mean Hospital for Special Surgery knee score increased to 75 points (70 to 80; p < 0.01) in those undergoing revision replacement of the knee.

A single-stage revision following fungal peri-prosthetic infection is feasible, with an acceptable rate of a satisfactory outcome.

Cite this article: Bone Joint J 2014;96-B:492–6.

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.

While primary squamous-cell carcinoma of the hand is common, metastasis of a squamous-cell carcinoma to the hand is very rare. It has been reported to arise from carcinoma of the lung and oesophagus and, rarely, from other tumours. We describe a patient with metastatic squamous-cell carcinoma occurring in the first web space of the hand from primary lung cancer, which remained undetected for 30 months after treatment of the metastasis.

Smoking is known to have an adverse effect on wound healing and musculoskeletal conditions. This case-controlled study looked at whether smoking has a deleterious effect in the outcome of autologous chondrocyte implantation for the treatment of full thickness chondral defects of the knee.

The mean Modified Cincinatti Knee score was statistically significantly lower in smokers (n = 48) than in non-smokers (n = 66) both before and after surgery (p < 0.05). Smokers experienced significantly less improvement in the knee score two years after surgery (p < 0.05). Graft failures were only seen in smokers (p = 0.016). There was a strong negative correlation between the number of cigarettes smoked and the outcome following surgery (Pearson’s correlation coefficient −0.65, p = 0.004).

These results suggest that patients who smoke have worse pre-operative function and obtain less benefit from this procedure than non-smokers. The counselling of patients undergoing autologous chondrocyte implantation should include smoking, not only as a general cardiopulmonary risk but also because poorer results can be expected in smokers following this procedure.

We describe 22 cases of bizarre parosteal osteochondromatous proliferation, or Nora’s lesion. These are surface-based osteocartilaginous lesions typically affecting the hands and feet. All patients were identified from the records of a regional bone tumour unit and were treated between 1985 and 2009. Nine lesions involved the metacarpals, seven the metatarsals, one originated from a sesamoid bone of the foot and five from long bones (radius, ulna, tibia, and femur in two). The mean age of the patients was 31.8 years (6 to 66), with 14 men and eight women. Diagnosis was based on the radiological and histological features. The initial surgical treatment was excision in 21 cases and amputation of a toe in one. The mean follow-up was for 32 months (12 to 162). Recurrence occurred in six patients (27.3%), with a mean time to recurrence of 49 months (10 to 120). Two of the eight patients with complete resection margins developed a recurrence (25.0%), compared with four of 14 with a marginal or incomplete resection (28.6%).

Given the potential surgical morbidity inherent in resection, our data suggest that there may be a role for a relatively tissue-conserving approach to the excision of these lesions.

We report 17 patients (20 hips) in whom metal-on-metal resurfacing had been performed and who presented with various symptoms and a soft-tissue mass which we termed a pseudotumour. Each patient underwent plain radiography and in some, CT, MRI and ultrasonography were also performed. In addition, histological examination of available samples was undertaken.

All the patients were women and their presentation was variable. The most common symptom was discomfort in the region of the hip. Other symptoms included spontaneous dislocation, nerve palsy, a noticeable mass or a rash. The common histological features were extensive necrosis and lymphocytic infiltration. To date, 13 of the 20 hips have required revision to a conventional hip replacement. Two are awaiting revision.

We estimate that approximately 1% of patients who have a metal-on-metal resurfacing develop a pseudotumour within five years. The cause is unknown and is probably multifactorial. There may be a toxic reaction to an excess of particulate metal wear debris or a hypersensitivity reaction to a normal amount of metal debris. We are concerned that with time the incidence of these pseudotumours may increase. Further investigation is required to define their cause.