Complete resolution and remodeling of chronic recurrent multifocal osteomyelitis on MRI and radiographs.

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare condition thought to be under-diagnosed, with a true prevalence of more than the 1 in 10,000 estimated. It is a condition that is classically described as polyostotic with a relapsing and remitting course, preferentially affecting the metaphyses of tubular bones in the pediatric population. Lesions have characteristic appearances of cortical hyperostosis and mixed lytic/sclerotic medullary appearances radiographically, with active osteitis and periostitis best seen with fluid-sensitive sequences on magnetic resonance imaging (MRI). There are reports of lesions resolving on follow-up radiographs and MRI scans, but no supporting images. In particular, although the marrow appearances and degree of osteitis have been shown to improve on MRI, complete resolution and remodeling back to normal has never been demonstrated. We present a case of a lesion that has completely healed and remodeled back to normal appearances on both radiographs and MRI, and consider this the standard for the often loosely used terms "normalization" and "resolution". We discuss the implications of this for our understanding of the natural history of CRMO, and how this adds weight to the condition being significantly under-diagnosed. It provides a "gold standard" to be aimed for when assessing treatments for CRMO, and the optimal outcomes that are possible. It also provides further insight into the potential of pediatric bone to recover and remodel when affected by inflammatory conditions.

Distal tibial giant cell tumour treated with curettage and stabilisation with an Ilizarov frame.

A 36-year-old man presented with a long history of pain in his left ankle. Imaging and biopsy confirmed this to be a giant cell tumour of bone. This was treated with curettage of the lesion and stabilisation with an Ilizarov frame. After a prolonged course of rehabilitation he has excellent function and has returned to hill walking and there is no evidence of recurrence of the giant cell tumour.

Prognostic indicators of outcome for patients with skeletal metastases from carcinoma of the prostate.

AIMS: The purpose of this study was to identify prognostic indicators of outcome at presentation to the orthopaedic surgeon, in patients with metastatic prostate cancer. Our aim was to use this information in a pragmatic, clinic-based approach so that surgical decision making could be optimized to benefit the patient in their remaining lifetime. PATIENTS AND METHODS: A cohort analysis was undertaken of all patients with metastatic disease of the prostate who presented to a regional orthopaedic centre in the United Kingdom between 2003 and 2016. Biochemical data were collected in addition to disease and demographic data. These included: prostate-specific antigen (PSA) at orthopaedic presentation; haemoglobin (Hb); platelets (plt); alkaline phosphatase (ALP); albumin (Alb); and corrected calcium (CaC). Statistical analysis included Kaplan-Meier survival analysis, and a Cox proportional hazards model was fitted to the data. RESULTS: From the departmental database, 137 episodes were identified in 136 patients with a median age at presentation of 72 years (interquartile range (IQR) 66 to 78). Most patients had stage IV disease (n = 98, 72%), and most did not undergo surgical intervention. At one-year follow-up, 50% of patients had died. Biomarkers found to be independently associated with poor survival were: low Hb, low Alb, relatively low PSA (< 30 mmol/l), and a raised ALP. Patients who needed surgical intervention had a poorer survival rate than patients who were managed nonoperatively. CONCLUSION: The study findings are important for orthopaedic clinical practice in the management of patients with metastatic prostate cancer. The interpretation of routine blood tests can help to predict survival in patients who present with orthopaedic manifestations of prostate cancer. A lower PSA is not necessarily a good prognostic sign. We believe that simple blood testing should be carried out routinely when assessing a patient, guiding potential surgical management and palliative care in the future.

The validity of the Mirels score for predicting impending pathological fractures of the lower limb.

Aims: The aim of this study was to validate the Mirels score in predicting pathological fractures in metastatic disease of the lower limb. Patients and Methods: A total of 62 patients with confirmed metastatic disease met the inclusion criteria. Of the 62 patients, 32 were female and 30 were male. The mean age of patients was 65 years (35 to 89). The primary malignancy originated from the breast in 27 (44%) patients, prostate in 15 (24%) patients, kidney in seven (11%), and lung in four (6%) of patients. One patient (2%) had metastatic carcinoma from the lacrimal gland, two patients (3%) had multiple myeloma, one patient (2%) had lymphoma of bone, and five patients (8%) had metastatic carcinoma of unknown primary. Plain radiographs at the time of initial presentation were scored using Mirels system by the four authors. The radiographic components of the score (anatomical site, size, and radiographic appearance) were scored two weeks apart. Inter- and intraobserver reliability were calculated with Fleiss' kappa test. Bland-Altman plots were created to compare the variances of the individual components of the score and the total Mirels score. Results: Kappa values for the interobserver variability of the components of the Mirels score were k = 0.554 (95% CI 0.483 to 0.626) for site, k = 0.342 (95% CI 0.285 to 0.400) for size, k = 0.443 (95% CI 0.387 to 0.499) for radiographic appearance, and k = 0.294 (95% CI 0.258 to 0.331)for the total score. Kappa values for the intra-observer reliability were k = 0.608 (95% CI 0.506 to 0.710) for site, k = 0.579 (95% CI 0.487 to 0.670) for size, k = 0.614 (95% CI 0.522 to 0.703) for radiographic appearance, and k = 0.323 (95% CI 0.266 to 0.379) for total score. Conclusion: Our study showed fair to moderate agreement between authors when using the Mirels score, and moderate to substantial agreement when authors rescored radiographs. The Mirels score is subjective and lacks reproducibility in predicting the risk of pathological fracture. Cite this article: Bone Joint J 2018;100-B:1100-5.

Hemiarthroplasty proximal femoral endoprostheses following tumour reconstruction: is acetabular replacement necessary?

AIMS: Dislocation rates are reportedly lower in patients requiring proximal femoral hemiarthroplasty than for patients undergoing hip arthroplasty for neoplasia. Without acetabular replacement, pain due to acetabular wear necessitating revision surgery has been described. We aimed to determine whether wear of the native acetabulum following hemiarthroplasty necessitates revision surgery with secondary replacement of the acetabulum after proximal femoral replacement (PFR) for tumour reconstruction. PATIENTS AND METHODS: We reviewed 100 consecutive PFRs performed between January 2003 and January 2013 without acetabular resurfacing. The procedure was undertaken in 74 patients with metastases, for a primary bone tumour in 20 and for myeloma in six. There were 48 male and 52 female patients, with a mean age of 61.4 years (19 to 85) and median follow-up of two years (interquartile range (IQR) 0.5 to 3.7 years). In total, 52 patients presented with a pathological fracture and six presented with failed fixation of a previously instrumented pathological fracture. RESULTS: All patients underwent reconstruction with either a unipolar (n = 64) or bipolar (n = 36) articulation. There were no dislocations and no acetabular resurfacings. Articular wear was graded using the criteria of Baker et al from 0 to 3, where by 0 is normal; grade 1 represents a narrowing of articular cartilage and no bone erosion; grade 2 represents acetabular bone erosion and early migration; and grade 3 represents protrusio acetabuli. Of the 49 patients with radiological follow-up greater than one year, six demonstrated grade 1 acetabular wear and two demonstrated grade 2 acetabular wear. The remainder demonstrated no radiographic evidence of wear. Median medial migration was 0.3 mm (IQR -0.2 to 0.7) and superior migration was 0.3 mm (IQR -0.2 to 0.6). No relationship between unipolar versus bipolar articulations and wear was evident. CONCLUSION: Hemiarthroplasty PFRs for tumour reconstruction eliminate joint instability and, in the short to medium term, do not lead to native acetabular wear necessitating later acetabular resurfacing. Cite this article: Bone Joint J 2018;100B:101-8.

Observations on the natural history of massive lumbar disc herniation.

We have treated 15 patients with massive lumbar disc herniations non-operatively. Repeat MR scanning after a mean 24 months (5 to 56) showed a dramatic resolution of the herniation in 14 patients. No patient developed a cauda equina syndrome. We suggest that this condition may be more benign than previously thought.

The impact and efficacy of surveillance in patients with sarcoma of the extremities.

OBJECTIVES: In this cross sectional study, the impact and the efficacy of a surveillance programme for sarcomas of the extremities was analysed. METHODS: All patients who had treatment with curative intent for a high-grade sarcoma and were diagnosed before 2014 were included and followed for a minimum of two years. RESULTS: Of the 909 patients who had a review appointment in 2014, 131 were under review for a high-grade sarcoma of the extremities following treatment with curative intent. Of these patients, three patients died of disease, two patients died of other causes, 12 are alive, with disease, and 114 have no evidence of disease. The surveillance programme accounts for 14% of all review appointments.Four of five patients (80%) who developed local recurrence identified the recurrence themselves. Chest radiographs are adequate in identifying metastatic disease and 11 (73%) of metastases were diagnosed during a routine follow up visit. However, the chance of cure is small and only two patients were referred for a metastatectomy. Of these only one survived for more than two years.The mean time for developing metastatic disease and local recurrence was 2.0 and 3.9 years respectively. Once identified, the mean time to death was 2.1 years for patients with metastatic disease. CONCLUSIONS: Surveillance of sarcoma patients makes up a substantial amount of the workload of a sarcoma unit. The chance of cure following identification of local recurrence or metastatic disease, however, is small. Alternative methods of surveillance that allow better evaluation of the patient's needs are recommended.Cite this article: P. Cool, G. Cribb. The impact and efficacy of surveillance in patients with sarcoma of the extremities. Bone Joint Res 2017;6:224-230. DOI: 10.1302/2046-3758.64.BJR-2016-0253.R1.

Prognostic factors for patients with skeletal metastases from carcinoma of the breast.

AIMS: Surgical intervention in patients with bone metastases from breast cancer is dependent on the estimated survival of the patient. The purpose of this paper was to identify factors that would predict survival so that specific decisions could be made in terms of surgical (or non-surgical) management. METHODS: The records of 113 consecutive patients (112 women) with metastatic breast cancer were analysed for clinical, radiological, serological and surgical outcomes. Their median age was 61 years (interquartile range 29 to 90) and the median duration of follow-up was 1.6 years (standard deviation (sd) 1.9, 95% confidence interval (CI) 0 to 5.9). The cumulative one- and five-year rates of survival were 68% and 16% (95% Cl 60 to 77 and 95% CI 10 to 26, respectively). RESULTS: Linear discriminant analysis identified a 'quadruple A' predictor of survival by reclassifying the sum of the albumin, adjusted calcium, alkaline phosphatase and age covariates each multiplied by a determined factor. The accuracy of this 'quadruple A' predictor was 90% with a sensitivity of 100% and a specificity of 88%. A receiver operating characteristic (ROC) curve revealed an area under the curve of 79%. Survival analysis for this 'quadruple A' predictor (< = one or > one year survival) was statistically significant using the log rank test (p = 0.0004) and Cox proportional hazard (p = 0.001). Multivariate analysis showed the 'quadruple A' predictor to be the only independent predictor of survival (p = 0.01). DISCUSSION: The 'quadruple A' predictor, together with other positive predictors of survival, can be used by oncologists, orthopaedic and breast surgeons to estimate survival and therefore guide management.

Surveillance of intramedullary cartilage tumours in long bones.

AIMS: The purpose of this study was to determine if clinical and radiological surveillance of cartilage tumours with low biological activity is appropriate. PATIENTS AND METHODS: A total of 98 patients with an intramedullary cartilage neoplasm in a long bone met our inclusion criteria and were included in the study. These patients had undergone a total of 384 scans. Patients with radiological follow-up of more than three years (46 patients) were divided into two groups: an active group (11 patients) and a latent group (35 patients). RESULTS: Active lesions had a total growth in all three planes that was > 6 mm, whilst latent lesions had < 6 mm of growth. Most latent lesions were heavily calcified: active lesions were calcified less than 50% (p = 0.025). CONCLUSION: Clinico-radiological surveillance can identify growing cartilage lesions: MRI is the surveillance modality of choice. A CT scan is recommended, in addition, at presentation to assess the amount of calcification within the lesion. A first follow-up MRI is suggested one year from diagnosis. If the total growth in the cartilage lesion is > 6 mm, surgical treatment should be considered. Otherwise, a second surveillance scan can be performed at three years to determine further management. Cite this article: Bone Joint J 2016;98-B:1542-7.

Whole-body magnetic resonance imaging in myxoid liposarcoma: A useful adjunct for the detection of extra-pulmonary metastatic disease.

Myxoid liposarcomas (MLS) are a subgroup of soft-tissue sarcomas which have a propensity for extra-pulmonary metastases. Conventional radiological staging of soft-tissue sarcomas consists of chest radiographs (CXR) and thoracic computed tomography (CT) for possible chest metastases, supplemented by magnetic resonance imaging (MRI) for local disease. The optimal radiological modality to detect extra-pulmonary metastases for systemic staging has not been proven. We reviewed the efficacy of Whole-Body MRI (WBMRI) for this purpose. 33 WBMRI and simultaneous CT scans were performed in 28 patients suffering from MLS between 2007 and 2015. 38 metastases were identified in seven patients via WBMRI. Osseous lesions predominated (spine, pelvis, chest-wall and long bones), followed by soft-tissue and abdominal lesions. Of the 29 soft-tissue or osseous metastases that were within the field-of-view of the simultaneous CT scans, five soft-tissue and zero osseous metastases were identified using CT. Metastatic disease was detected in three patients solely using WBMRI, which directly influenced their management. WBMRI is a useful adjunct in the detection of extra-pulmonary metastatic disease, which directly alters patient management. WBMRI has demonstrated an ability to identify more sites of metastatic disease compared to CT. WBMRI should be used in two situations. Firstly, at diagnosis where ablative treatment will be required e.g. amputation, when the diagnosis of occult metastasis would change treatment planning. Secondly, at diagnosis of relapse to confirm if it is a solitary site of relapse prior to consideration of metastectomy.

Giant lipomatous tumours of the hand and forearm.

This study examines the presentation, management and outcomes of a series of 10 patients with giant lipomatous tumours (defined as greater than 5 cm diameter) of the hand and forearm who presented to our orthopaedic oncology service. All patients underwent local staging and were discussed at our multidisciplinary tumour meeting prior to definitive surgery. In all cases, neurovascular structures required mobilization in order to excise the tumour. Seven of the tumours were benign lipomas and one was a neural fibrolipoma. The other two were well differentiated lipoma-like liposarcomas/atypical lipomatous tumours. Giant lipomas and well differentiated lipoma-like liposarcomas/atypical lipomatous tumours of the hand and forearm present infrequently and a multidisciplinary approach is recommended in the investigation and surgical management of these patients.

Subcutaneous and lung metastases from chondrosarcoma of the thumb.

We present a rare case of metacarpal chondrosarcoma with cutaneous metastases in the ipsilateral upper arm. Chondrosarcomas of the small bones of the hand rarely metastasise unlike chondrosarcomas elsewhere in the body. Excision/ray amputation rather than curettage may be preferable in the treatment of high-grade chondrosarcomas in the hand.

Metastatic bone disease--the orthopaedic workload.

BACKGROUND: In 1999, the British Association of Surgical Oncology (BASO) published its guidelines for the management of metastatic bone disease in breast cancer in the UK, highlighting the role of the orthopaedic surgeon as an integral part of the multidisciplinary team. AIM: To establish the workload to our department of metastatic bone disease. METHODS: A prospective study was performed over a 6-month period aiming to study factors affecting length of stay, whether BASO recommendations were being adhered to for metastatic breast disease and whether BASO recommendations could be extended to other forms of metastatic bone disease. RESULTS: 36 patients were referred to the orthopaedic department during the study period. The majority of the patients were referred as emergencies, 83% of patients were seen by an orthopaedic consultant on the day of referral but only 27% were seen by the orthopaedic cancer liaison consultant. Only 16 patients required a therapeutic operative intervention and length of in-patient stay was increased by not having a known primary carcinoma at admission. Patients with bone metastases discussed at the breast cancer multidisciplinary meeting were also studied as were patients with bone metastases presenting to the oncology service. It was found that there was little overlap between these groups and our study group. CONCLUSIONS: Metastatic bone disease does form a workload to the orthopaedic department and BASO recommendations are generally followed for breast disease. The BASO recommendations could be extended to other tumours; however, it should be recognised that there is a significant workload involved and a need for sufficient resources to allow a high quality and timely service.

Diffuse pigmented villonodular synovitis (diffuse-type giant cell tumour) of the foot and ankle.

Pigmented villonodular synovitis (PVNS) is a rare benign disease of the synovium of joints and tendon sheaths, which may be locally aggressive. We present 18 patients with diffuse-type PVNS of the foot and ankle followed for a mean of 5.1 years (2 to 11.8). There were seven men and 11 women, with a mean age of 42 years (18 to 73). A total of 13 patients underwent open or arthroscopic synovectomy, without post-operative radiotherapy. One had surgery at the referring unit before presentation with residual tibiotalar PVNS. The four patients who were managed non-operatively remain symptomatically controlled and under clinical and radiological surveillance. At final follow-up the mean Musculoskeletal Tumour Society score was 93.8% (95% confidence interval (CI) 85 to 100), the mean Toronto Extremity Salvage Score was 92 (95% CI 82 to 100) and the mean American Academy of Orthopaedic Surgeons foot and ankle score was 89 (95% CI 79 to 100). The lesion in the patient with residual PVNS resolved radiologically without further intervention six years after surgery. Targeted synovectomy without adjuvant radiotherapy can result in excellent outcomes, without recurrence. Asymptomatic patients can be successfully managed non-operatively. This is the first series to report clinical outcome scores for patients with diffuse-type PVNS of the foot and ankle.

Limb salvage for soft-tissue sarcomas of the foot and ankle.

We evaluated the oncological and functional outcome of 27 patients who had limb salvage for a soft-tissue sarcoma of the foot or ankle between 1992 and 2007, with a mean follow-up of 7.5 years (1.05 to 16.2). There were 12 men and 15 women, with a mean age at presentation of 47 years (12 to 84). Referrals came from other hospitals for 16 patients who had previous biopsy or unplanned excision, and 11 presented de novo. There were 18 tumours located in the foot and nine around the ankle. Synovial sarcoma was the most frequent histological diagnosis. Excision was performed in all cases, with 16 patients requiring plastic surgical reconstruction with 13 free and three local flaps. Adjuvant treatment was undertaken in 20 patients, 18 with radiotherapy and two by chemotherapy. Limb salvage was successful in 26 of the 27 patients. There have been two local recurrences and two mesenchymal metastases. Four patients have died of their sarcoma and two of other causes. Function was evaluated with the Toronto Extremity Salvage Score and a mean overall score of 89.40 (52.1 to 100) was obtained. A questionnaire revealed that all surviving patients are able to wear normal shoes and none require a walking aid. Limb salvage can achieve good oncological and functional results with additional treatment.

Percutaneous radiofrequency thermocoagulation of osteoid osteomas: factors affecting therapeutic outcome.

OBJECTIVE: To examine factors which affect local recurrence of osteoid osteomas treated with percutaneous CT-guided radiofrequency thermocoagulation. DESIGN AND PATIENTS: A prospective study was carried out on 45 patients with osteoid osteoma who underwent percutaneous radiofrequency thermocoagulation with a minimum follow-up of 12 months. RESULTS: There were seven local recurrences (16%); all occurred within the first year. Local recurrence was significantly related to a non-diaphyseal location (P<0.01). There was no significant relationship (P=0.05) between local recurrence and age of the patient, duration of symptoms, previous treatment, size of the lesion, positive biopsy, radiofrequency generator used or the number of needle positions. There were no complications. CONCLUSIONS: Osteoid osteomas in a non-diaphyseal location are statistically more likely to recur than those in a diaphyseal location when treated with CT-guided percutaneous radiofrequency thermocoagulation. This relationship between local recurrence and location has not been previously reported.

Mountain biking injuries in rural England.

BACKGROUND: Off road mountain biking is now an extremely popular recreation and a potent cause of serious injury. AIM: To establish the morbidity associated with this sport. METHODS: Data were collected prospectively over one year on all patients presenting with an injury caused by either recreational or competitive off road mountain biking. RESULTS: Eighty four patients were identified, 70 males and 14 females, with a mean age of 22.5 years (range 8-71). Most accidents occurred during the summer months, most commonly in August. Each patient had an average of 1.6 injuries (n = 133) and these were divided into 15 categories, ranging from minor soft tissue to potentially life threatening. Operative intervention was indicated for 19 patients (23%) and several required multiple procedures. The commonest injuries were clavicle fractures (13%), shoulder injuries (12%), and distal radial fractures (11%). However, of a more sinister nature, one patient had a C2/3 dislocation requiring urgent stabilisation, one required a chest drain for a haemopneumothorax, and another required an emergency and life saving nephrectomy. CONCLUSION: This sport has recently experienced an explosion in popularity, and, as it carries a significant risk of potentially life threatening injury across all levels of participation, the use of protective equipment to reduce this significant morbidity may be advisable.