Elastofibroma dorsi is an uncommon, benign, slow-growing soft-tissue tumour of uncertain aetiology. It classically presents as an ill-defined mass at the inferior pole of the scapula with symptoms which include swelling, discomfort, snapping, stiffness and occasionally pain. We report the symptoms, function and outcome after treatment of 21 elastofibromas in 15 patients. All were diagnosed by MRI and early in the series four also underwent CT-guided biopsy to confirm the diagnosis. In all, 18 tumours were excised and three were observed. After excision, the mean visual analogue score for pain decreased from 4.6 (0 to 10) pre-operatively to 2.4 (0 to 8) post-operatively (p = 0.04). The mean shoulder function, at a mean follow-up of 4.2 years (3 months to 16 years), was 78.1% (30 to 100) using the Stanmore percentage of normal shoulder assessment scoring system. The mean range of forward flexion improved from 135° (70° to 180°) to 166° (100° to 180°) after excision (p = 0.005). In four patients a post-operative haematoma formed; one required evacuation. Three patients developed a post-operative seroma requiring needle aspiration and one developed a superficial infection which was treated with antibiotics. Our findings support previous reports suggesting that a pre-operative tissue diagnosis is not necessary in most cases since the lesion can be confidently diagnosed by MRI, when interpreted in the light of appropriate clinical findings. Surgical excision in symptomatic patients, is helpful. It has been suggested that elastofibroma is caused by a local tissue reaction and is not a true neoplastic process. A strong association has been noted between elastofibroma and repetitive use of the shoulder, which is supported by our findings

Aims

Orthopaedic and reconstructive surgeons are faced with large defects after the resection of malignant tumours of the sacrum. Spinopelvic reconstruction is advocated for resections above the level of the S1 neural foramina or involving the sacroiliac joint. Fixation may be augmented with either free vascularized fibular flaps (FVFs) or allograft fibular struts (AFSs) in a cathedral style. However, there are no studies comparing these reconstructive techniques.

Aims

The aim of this study was to analyze the complications and outcomes of treatment in a series of previously untreated patients with a primary aneurysmal bone cyst (ABC) who had been treated by percutaneous sclerosant therapy using polidocanol.

Aims

The primary aim of this study was to determine the effect of the duration of symptoms (DOS) prior to diagnosis on the overall survival in patients with a primary bone sarcoma.

Aims

Following the resection of an extensive amount of bone in the treatment of a tumour, the residual segment may be insufficient to accept a standard length intramedullary cemented stem. Short-stemmed endoprostheses conceivably have an increased risk of aseptic loosening. Extra-cortical plates have been added to minimise this risk by supplementing fixation. The aim of this study was to investigate the survivorship of short-stemmed endoprostheses and extra-cortical plates.

Previous classification systems of failure of limb salvage focused primarily on endoprosthetic failures and lacked sufficient depth for the effective study of the causes of failure. In order to address these inadequacies, the International Society of Limb Salvage (ISOLS) formed a committee to recommend revisions of the previous systems. The purpose of this study was to report on their recommendations. The modifications were prepared using an earlier, evidence-based model with subclassification based on the existing medical literature. Subclassification for all five primary types of failure of limb salvage following endoprosthetic reconstruction were formulated and a complementary system was derived for the failure of biological reconstruction. An additional classification of failure in paediatric patients was also described.

Limb salvage surgery presents a complex array of potential mechanisms of failure, and a complete and precise classification of types of failure is required. Earlier classification systems lacked specificity, and the evidence-based system outlined here is designed to correct these weaknesses and to provide a means of reporting failures of limb salvage in order to allow the interpretation of outcome following reconstructive surgery.

Cite this article: Bone Joint J 2014;96-B:1436–40.

Avascular necrosis (AVN) is a serious complication of high-dose chemotherapy for haematological malignancy in childhood. In order to describe its incidence and main risk factors and to evaluate the current treatment options, we reviewed 105 children with a mean age of 8.25 years (1 to 17.8) who had acute lymphoblastic or acute myeloid leukaemia, or a non-Hodgkin’s lymphoma. Overall, eight children (7.6%) developed AVN after a mean of 16.8 months (8 to 49). There were four boys and four girls with a mean age of 14.4 years (9.8 to 16.8) and a total of 18 involved sites, 12 of which were in the femoral head. All these children were aged > nine years (p < 0.001). All had received steroid treatment with a mean cumulative dose of prednisone of 5967 mg (4425 to 9599) compared with a mean of 3943 mg (0 to 18 585) for patients without AVN (p = 0.005). No difference existed between genders and no thrombophilic disorders were identified. Their initial treatment included 11 core decompressions and two bipolar hip replacements. Later, two salvage osteotomies were done and three patients (four hips) eventually needed a total joint replacement. We conclude that AVN mostly affects the weight-bearing epiphyses. Its risk increases with age and higher steroid doses. These high-risk patients may benefit from early screening for AVN.

Cite this article: Bone Joint J 2013;95-B:1708–13.

Rarely, the extent of a malignant bone tumour may necessitate resection of the complete humerus to achieve adequate oncological clearance. We present our experience with reconstruction in such cases using a total humeral endoprosthesis (THER) in 20 patients (12 male and eight female) with a mean age of 22 years (6 to 59). We assessed the complications, the oncological and functional outcomes and implant survival. Surgery was performed between June 2001 and October 2009. The diagnosis included osteosarcoma in nine, Ewing’s sarcoma in eight and chondrosarcoma in three. One patient was lost to follow-up. The mean follow-up was 41 months (10 to 120) for all patients and 56 months (25 to 120) in survivors. There were five local recurrences (26.3%) and 11 patients were alive at time of last follow-up, with overall survival for all patients being 52% (95% confidence interval (CI) 23.8 to 74) at five years. The mean Musculoskeletal Tumor Society score for the survivors was 22 (73%; 16 to 23). The implant survival was 95% (95% CI 69.5 to 99.3) at five years.

The use of a THER in the treatment of malignant tumours of bone is oncologically safe; it gives consistent and predictable results with low rates of complication.

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 identified 42 patients who presented to our unit over a 27-year period with a secondary radiation-induced sarcoma of bone. We reviewed patient, tumour and treatment factors to identify those that affected outcome. The mean age of the patients at presentation was 45.6 years (10 to 84) and the mean latent interval between radiotherapy and diagnosis of the sarcoma was 17 years (4 to 50). The median dose of radiotherapy given was estimated at 50 Gy (mean 49; 20 to 66). There was no correlation between radiation dose and the time to development of a sarcoma. The pelvis was the most commonly affected site (14 patients (33%)). Breast cancer was the most common primary tumour (eight patients; 19%). Metastases were present at diagnosis of the sarcoma in nine patients (21.4%). Osteosarcoma was the most common diagnosis and occurred in 30 cases (71.4%). Treatment was by surgery and chemotherapy when indicated: 30 patients (71.4%) were treated with the intention to cure. The survival rate was 41% at five years for those treated with the intention to cure but in those treated palliatively the mean survival was only 8.8 months (2 to 22), and all had died by two years. The only factor found to be significant for survival was the ability to completely resect the tumour. Limb sarcomas had a better prognosis (66% survival at five years) than central ones (12% survival at five years) (p = 0.009).

Radiation-induced sarcoma is a rare complication of radiotherapy. Both surgical and oncological treatment is likely to be compromised by the treatment received previously by the patient.

We describe the treatment by subperiosteal resection of an aneurysmal bone cyst in the distal fibula in eight patients and highlight the role of the periosteum in the regeneration of bone defects. The mean age of the patients was 13.5 years (12 to 17). Seven had an open growth plate. The mean size of the resected specimen was 5.12 cm (3.5 to 8.0). None of the patients received instillation of bone marrow, autogenous bone graft, allograft or any synthetic bone substitutes.

All had complete regeneration of the bone defect within three to nine months, with no joint instability or recurrence.

The mean length of follow-up was 11.5 years (2 to 18). At the final follow-up there was no difference in the range of movement, alignment or stability of the ankle when compared with the opposite side. The periosteum played a major role in the complete filling of the bone defects and avoided the morbidity of other techniques.

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 report our experience of treating 17 patients with benign lesions of the proximal femur with non-vascularised, autologous fibular strut grafts, without osteosynthesis. The mean age of the patients at presentation was 16.5 years (5 to 33) and they were followed up for a mean of 2.9 years (0.4 to 19.5). Histological diagnoses included simple bone cyst, fibrous dysplasia, aneurysmal bone cysts and giant cell tumour. Local recurrence occurred in two patients (11.7%) and superficial wound infection, chronic hip pain and deep venous thrombosis occurred in three. Pathological fracture did not occur in any patient following the procedure.

We conclude that non-vascularised fibular strut grafts are a safe and satisfactory method of treating benign lesions of the proximal femur.

Open surgery is rarely justified for the initial treatment of a unicameral bone cyst, but there is some debate concerning the relative effectiveness of closed methods. This study compared the results of steroid injection with those of autologous bone marrow grafting for the treatment of unicameral bone cysts. Between 1990 and 2001, 30 patients were treated by steroid injection and 28 by grafting with autologous bone marrow. The overall success rates were 86.7% and 92.0%, respectively (p > 0.05). The success rate after the initial procedure was 23.3% in the steroid group and 52.0% in those receiving autologous bone marrow (p < 0.05), and the respective cumulative success rates after second injections were 63.3% and 80.0% (p > 0.05). The mean number of procedures required was 2.19 (1 to 5) and 1.57 (1 to 3) (p < 0.05), the mean interval to healing was 12.5 months (4 to 32) and 14.3 months (7 to 36) (p > 0.05), and the rate of recurrence after the initial procedure was 41.7% and 13.3% in the steroid and in the autologous bone marrow groups, respectively (p < 0.05).

Although the overall rates of success of both methods were similar, the steroid group had higher recurrence after a single procedure and required more injections to achieve healing.

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.

We report positive and negative factors associated with the most commonly-used methods of reconstruction after pathological fracture of the proximal femur. The study was based on 142 patients treated surgically for 146 metastatic lesions between 1996 and 2003. The local rate of failure was 10.3% (15 of 146). Of 37 operations involving osteosynthetic devices, six failed (16.2%) compared with nine (8.3%) in 109 operations involving endoprostheses. Of nine cases of prosthetic failure, four were due to periprosthetic fractures and three to recurrent dislocation. In the osteosynthesis group, three (13.6%) of 22 reconstruction nails failed. The two-year risk of re-operation after any type of osteosynthesis was 0.35 compared with 0.18 after any type of endoprosthetic reconstruction (p = 0.07). Endoprosthetic reconstructions are preferable to the use of reconstruction nails and other osteosynthetic devices for the treatment of metastatic lesions in the proximal third of the femur.