Clinical and oncological outcomes after surgical excision of pigmented villonodular synovitis at the foot and ankle.

BACKGROUND: Pigmented villonodular synovitis (PVNS) is a rare benign neoplastic disease of the synovium of joints and tendon sheaths, which may be locally aggressive. It can be broadly classified into localised disease or more diffuse forms, with the latter more prone to recurrence after surgical excision. We describe our experience in the management of foot and ankle PVNS, focusing on the diffuse type. METHODS: Patients with PVNS were identified from a histology database from 2000 to 2010 at the University Hospitals of Leicester. The primary aim was to determine oncological outcomes and evaluate clinical outcomes with the Toronto Extremity Salvage Score (TESS) and the American Academy of Foot and Ankle Surgeons (AOFAS) scores. RESULTS: 30 patients, 16 males and 14 females with a mean age of 37±15 years, who underwent surgery, were identified. There were 22 nodular PVNS and 8 diffuse PVNS. The diffuse PVNS was more likely to be in the hindfoot (75%, 6/8), of which 50% (3/6) had osteoarthritis at presentation. The localised PVNS was mostly located in the forefoot (91%, 20/22). None of the localised PVNS had a recurrence. The surgical recurrence rate in this series was similar to the pooled recurrence rate from the literature [12.5% (1/8) compared to 12.2% (6/49)]. The mean TESS and AOFAS scores were 86 and 78, respectively. CONCLUSIONS: Diffuse PVNS is more likely to occur in the hindfoot and nodular PVNS is more common in the forefoot. Aggressive synovectomy alone is an effective treatment for diffuse PVNS, with good oncological and clinical outcomes.

Tumour-to-tumour metastasis of laryngeal leiomyosarcoma to an axillary hibernoma.

Tumour-to-tumour metastasis is a rare, but well-recognised occurrence. This case report documents the metastasis of a primary laryngeal leiomyosarcoma to a hibernoma. We believe that this is the first recorded case of leiomyosarcoma metastasising to another neoplasm, and the first recorded case of a hibernoma acting as a recipient tumour for metastasis. This case study emphasises the importance of re-imaging a known benign mass in the presence of new symptoms in a patient with underlying malignancy, to ensure prompt diagnosis and management of potentially treatable metastasis. The imaging findings including whole body magnetic resonance imaging (MRI) staging, macroscopic and histological features are presented.

Benign soft tissue tumours of the foot & ankle: A pictorial review.

The purpose of this pictorial review is to aid the clinician in distinguishing different benign lesions within the foot and ankle. We discuss the typical clinical and radiological findings as well as management options for intra and extra-compartmental lesions. Differentiation between sarcoma and benign lesions is imperative when presented with any mass in the foot or ankle.

Spine and Non-spine Bone Metastases - Current Controversies and Future Direction.

Bone is a common site of metastases in advanced cancers. The main symptom is pain, which increases morbidity and reduces quality of life. The treatment of bone metastases needs a multidisciplinary approach, with the main aim of relieving pain and improving quality of life. Apart from systemic anticancer therapy (hormonal therapy, chemotherapy or immunotherapy), there are several therapeutic options available to achieve palliation, including analgesics, surgery, local radiotherapy, bone-seeking radioisotopes and bone-modifying agents. Long-term use of non-steroidal analgesics and opiates is associated with significant side-effects, and tachyphylaxis. Radiotherapy is effective mainly in localised disease sites. Bone-targeting radionuclides are useful in patients with multiple metastatic lesions. Bone-modifying agents are beneficial in reducing skeletal-related events. This overview focuses on the role of surgery, including minimally invasive treatments, conventional radiotherapy in spinal and non-spinal bone metastases, bone-targeting radionuclides and bone-modifying agents in achieving palliation. We present the clinical data and their associated toxicity. Recent advances are also discussed.

Risk factors for intramedullary nail breakage in proximal femoral fractures: a 10-year retrospective review.

INTRODUCTION Intramedullary nailing is a common treatment for proximal femoral fractures. Fracture of the nail is a rare but devastating complication that exposes often frail patients to complex revision surgery. We investigated which risk factors predict nail failure. METHODS We reviewed all cases of nail breakage seen over a 10-year period in a single busy trauma unit; 22 nail fractures were seen in 19 patients. Comparison was made with a group of 209 consecutive patients who underwent intramedullary fixation of a proximal femur fracture with no nail breakage over a 2-year period. RESULTS In the fractured nail group, mean age was 70.4 years (range 55-88 years).The mean time to fracture was 10 months (range 2.5-23 months). Logistical regression was used to show that low American Society of Anesthesiologists (ASA) score, subtrochanteric fracture and pathological fracture were independent risk factors for nail fracture. CONCLUSIONS Young patients with a low ASA score are at highest risk of nail breakage. We advise close follow-up of patients with these risk factors until bony union has been achieved. In addition, there may be merit in considering other treatment options, such as proximal femoral replacement, especially for those with pathological fracture with a good prognosis.

Surgical biopsy with intra-operative frozen section. An accurate and cost-effective method for diagnosis of musculoskeletal sarcomas.

The most appropriate protocol for the biopsy of musculoskeletal tumours is controversial, with some authors advocating CT-guided core biopsy. At our hospital the initial biopsies of most musculoskeletal tumours has been by operative core biopsy with evaluation by frozen section which determines whether diagnostic tissue has been obtained and, if possible, gives the definitive diagnosis. In order to determine the accuracy and cost-effectiveness of this protocol we have undertaken a retrospective audit of biopsies of musculoskeletal tumours performed over a period of two years. A total of 104 patients had biopsies according to this regime. All gave the diagnosis apart from one minor error which did not alter the management of the patient. There was no requirement for re-biopsy. This protocol was more labour-intensive and 38% more costly than CT-guided core biopsy (AU$1804 vs AU$1308). However, the accuracy and avoidance of the anxiety associated with repeat biopsy outweighed these disadvantages.

The investigation and management of suspected malignant pathological fractures: a review for the general orthopaedic surgeon.

The management of malignant pathological fractures necessitates careful diagnostic work-up, pre-operative investigation, planning and multidisciplinary input from specialists in the fields of radiology, pathology, oncology, trauma and orthopaedics. Malignant and non-malignant conditions including metabolic disorders, benign tumours and pharmacological therapies can be implicated. The majority of patients who present with suspected pathological fractures will be managed by general orthopaedic and trauma surgeons rather than specialists in orthopaedic oncology. Skeletal metastases can result in considerable morbidity and predispose to pathological fractures. With advances in the medical management of malignancy, life expectancy in cancer patients is increasing, leading to an increasing risk of skeletal metastasis and the potential for pathological fractures. Conventional modes of trauma fixation for pathological fractures may not be appropriate. The aim of this review is to outline diagnostic and management strategies for patients who present with a long bone fracture that is potentially pathological in nature.

Diagnostic triage for sarcoma: an effective model for reducing referrals to the sarcoma multidisciplinary team.

OBJECTIVE: Soft-tissue lesions are common and often benign. Owing to the rarity of soft-tissue sarcomas (STSs), evidence has shown that patients are increasingly referred urgently onto the 2-week wait pathway, which may have a detrimental impact on the management of patients with a proven STS. Imaging plays a vital role in lesion characterization and can be used to triage referrals to reduce the caseload of a sarcoma multidisciplinary team (MDT). In our institution, we established a sarcoma diagnostic triage meeting (SDTM). This study aimed to determine the effectiveness of the SDTM in reducing non-sarcomatous referrals to the main sarcoma MDT. METHODS: A retrospective review of the SDTM minutes from July 2011 to June 2012 was performed. Data collected for each case included details of referrer, referral modality and referral outcome. RESULTS: 165 cases were reviewed. 58% of referrals underwent a core biopsy or surgical excision with 85% benign pathology, the commonest being lipoma. 15% of referrals were sarcomatous lesions and were referred onwards to the main MDT. CONCLUSION: A total of 82% of the patients referred urgently with a suspicious soft-tissue mass was managed by the SDTM and hence not referred onwards to the East Midlands Sarcoma Service MDT. A diagnostic triage is effective in reducing the caseload burden of the main MDT and allowing it to be more effective. ADVANCES IN KNOWLEDGE: Referrals based on imaging can be prioritized by diagnostic triage. Diagnostic triage established in our institution reduced 82% of MDT referrals allowing a more focused MDT discussion on patients with a proven sarcoma.

Nationwide trends in the current management of desmoid (aggressive) fibromatosis.

AIMS: The optimal management of desmoid fibromatosis remains unclear, leading to significant variability in patient management. To assess this problem, the current approach of clinicians managing this complex condition in the UK was investigated. MATERIALS AND METHODS: A hypothetical case of intramuscular limb girdle desmoid fibromatosis in a fit 65-year-old patient was devised. Surgical and non-surgical oncology members of the British Sarcoma Group were questioned on how they would manage this case in three scenarios: primary disease with function-sparing surgery possible, primary disease with neurovascular involvement and disease recurrence after a previous R0 resection. Initial management, management of symptomatic disease progression, follow-up preferences and any differences in respondents' management choices in a younger case were investigated. RESULTS: The responses from 14 sarcoma surgeons and 23 oncologists (14 clinical, nine medical) were analysed. Desmoid fibromatosis management is generally shared by surgeons and oncologists within sarcoma multidisciplinary teams in the UK. Variation exists in the chosen initial management of primary desmoid fibromatosis in the UK, with function-sparing surgery possible (observation 51%, resection 51%), primary desmoid fibromatosis with neurovascular involvement (hormone therapy with non-steroidal anti-inflammatory drugs 51%, radiotherapy 27%, observation 22%) and for cases of desmoid fibromatosis recurrence (radiotherapy 41%, hormone therapy and non-steroidal anti-inflammatory drugs 27%, observation 24%). There was a clear preference of surgical resection of symptomatic disease progression in cases of primary desmoid fibromatosis without neurovascular involvement (60%). By contrast, radiotherapy was the preferred treatment for progression in cases with neurovascular involvement (47%) or cases of recurrence after a previous R0 resection (34%). Clinical follow-up was selected 3 months after intervention in 68% of scenarios. Follow-up imaging was selected 3 or 6 months after intervention in 57% and 21% of cases, respectively. Most respondents would not change their chosen management in younger patients. DISCUSSION: Several groups have issued formal guidelines for clinicians managing desmoid fibromatosis, including the British Sarcoma Group, the National Comprehensive Cancer Network and the European Society for Medical Oncology. However, these are in some ways contradictory and may not reflect recent publications, potentially explaining the significant variation in the management of desmoid fibromatosis in the UK shown by this survey. We propose a review of current evidence; a national consensus or a desmoid fibromatosis registry may help to standardise desmoid fibromatosis care.

Atypical familial Paget's disease of bone.

We report unusual clinical and radiological features of Paget's disease of bone in three family members. All three patients had satisfactory biochemical and symptomatic response to treatment with an intravenous bisphosphonate (clodronate).

Counting health care: is there aF in CE?

The consultant episode is widely used by health economists as a measure of delivery of care. The clinical notes of 174 consecutive patients undergoing elective orthopaedic procedures were reviewed. Just over half of the cases (89/174, 51%) were patients referred by their general practitioner, but under half of the available operating time (47%) was expended on these patients, the remainder being on patients already within the system. Under standard NHS accounting, these latter patients are not registered as new consultant referrals and, thus, the episodes of patient care of which their operation is a part, do not constitute finished consultant episodes. Our view is that the episode of care is an inappropriate measure of health care delivery applied to orthopaedic surgery in general.

Extra-abdominal desmoid fibromatosis--a sarcoma unit review of practice, long term recurrence rates and survival.

INTRODUCTION: Desmoid fibromatosis (DF) carries a significant morbidity and a recognised mortality. Despite this there are currently limited diagnostic or treatment algorithms specific to cases of extra-abdominal DF. Historically surgical excision has formed the cornerstone of treatment. Recently however a paradigm shift has meant many practitioners now adopt a more conservative approach, placing emphasis on active surveillance, function preserving resections, and non-surgical oncologic therapies. METHODS: We performed an 8-year retrospective review of all cases of extra-abdominal DF managed within our region to assess the consistency of diagnostics, management and long-term outcome. RESULTS: 47 eligible cases were identified. Mean age at diagnosis was 41.3 years (1-81 years). Disease location and speciality of diagnosing practitioners were varied. Management was generally inconsistent. Variation was seen in imaging, biopsy techniques, MDT involvement and management. At a median follow up of 4.9 years our local recurrence rate was 19%. DISCUSSION: The optimal management of DF is unknown. This has led to a lack of formalised guidance for practitioners managing this challenging condition, resulting in inconsistencies and areas for improvement in current management. We propose a diagnostic pathway which may improve consistency of care, reduce potentially unnecessary surgery and the associated morbidity, and significantly increase the rate of complete (R0) surgical resections when surgery is deemed appropriate whilst not significantly worsening oncological outcome. Specifically we propose all cases should be imaged appropriately (usually with MRI), undergo a planned biopsy (by radiologically guided core needle biopsy) and be managed centrally in conjunction with multidisciplinary sarcoma units.

Inadvertent surgical resection of soft tissue sarcomas.

BACKGROUND: The National Institute of Clinical Excellence (NICE) published Improving Outcome Guidance in 2006 defining urgent referral criteria for soft tissue sarcoma (STSs) with the twin aims of improving diagnostic accuracy and overall outcome. Despite these guidelines inadvertent excisions of soft tissue sarcomas continue to occur with alarming frequency, potentially compromising patient outcomes. OBJECTIVE: We reviewed the East Midlands Sarcoma Service experience of treating inadvertent excision of STSs and highlight the patient profile, referral pattern, subsequent management and oncological outcome associated with inadvertent resection. METHODS: Patients were identified from our sarcoma database and a retrospective case note review performed. RESULTS: Over a 3-year period, 42 patients presented to our specialist centre after unplanned excision of soft tissue sarcomas. There were 29 men and 13 women, with a mean age at presentation of 59 years (19-90). 50% of the tumours were located in lower extremity, 33% around the trunk and 17% in the upper extremity. The unplanned surgery was most commonly from general surgeons, plastic surgeons, orthopaedic surgeons, general practitioners followed by vascular surgeons. Re-resection was undertaken in 40 cases to achieve clear margins with residual tumour present in 74% of cases. Limb salvage surgery was not possible in 5 cases. CONCLUSION: Unplanned excision of sarcoma by non-oncologic surgeons remains a problem. It appears that it is equally prevalent in varied surgical community and general practitioners. Excision of large or deep solid soft tissue masses without tissue diagnosis is unacceptable.

Pigmented villonodular synovitis of the talonavicular joint: a case report and review of the literature.

Pigmented villonodular synovitis (PVNS) is a locally aggressive synovial proliferative disorder of unknown aetiology affecting the linings of joints, tendon sheaths, and bursae. A 22-year-old female patient presented with a 3-year history of an increasingly painful swelling on the dorsum of her right foot. Examination revealed a 4 cm x 2 cm swelling that was fluctuant, tender on palpation, unattached to overlying skin and partially mobile. A firm, pedunculated intra-articular lesion from the talonavicular joint was removed at surgery. Histology revealed a nodular lesion of stromal cells and numerous giant cells with villous architecture as well as abundant haemosiderin deposition with foamy macrophages (in keeping with PVNS). The patient is currently under review by the orthopaedic oncology team. Talonavicular joint PVNS is rare. MRI scanning is the optimum investigation. Complete excision is necessary to minimise high risk of recurrence.

Current practice regarding re-use of trauma instrumentation: results of a postal questionnaire survey.

A total of 248 Orthopaedic Units in the UK were surveyed following two newspaper articles about broken drill bits being retained in patients' bones. Questions were asked about re-use of drills, guide wires and taps, as well as about informing patients when drill bit fragments were left in situ during trauma surgery. The response rate was 79.0%. The majority of hospitals are not using new drill bits for each case nor new guide wires. In the current medicolegal climate, the fact that the majority of consultants are informing patients soon after surgery that a drill bit has fragmented in their bone is applauded.

Septic arthritis of the knee caused by Edwardsiella tarda after a catfish puncture wound.

Anglers are wary of catfish as their sharp fin spines may cause injury and, in some species, envenomation. We describe another complication of catfish spine injury--septic arthritis caused by Edwardsiella tarda. We believe this is the first report of this organism causing septic arthritis.

Surgical treatment of chondrosarcoma of the sternum.

PURPOSE: We reviewed all tumours of the sternum referred to The London Bone and Soft Tissue Tumour Service between 1956 and 1997 inclusive. PATIENTS AND RESULTS: There were eight patients with this pathology, the male to female ratio was 3:1 and their mean age was 53 years. Of these patients, three are alive and disease free, one is alive with recurrence, and four have died, two of the consequences of the disease and two of unrelated causes. Surgery is the principal treatment of these tumours both for excision and subsequent reconstruction. DISCUSSION: Extended disease-free survival is possible with correct diagnosis, complete excision at the first operation, appropriate skeletal reconstruction, adequate skin cover and appropriate postoperative support and follow-up.

Metacarpal morphometry using a semi-automated technique in the assessment of osteoporosis and vertebral fracture risk.

Metacarpal morphometry represents a potentially cheap and widely available non-invasive assessment of skeletal status. In two cross-sectional studies, we compared the performance characteristics of a semiautomated technique (the Teijin Bonalyzer) with an in-house manual measurement, and with measures of skeletal strength at other sites. The metacarpal cortical index (mCI) was measured on hand radiographs of 178 osteoporotic women using both the Teijin Bonalyzer and a digitizing tablet. Measurements on the latter were consistently lower than with the Bonalyzer except for mCI (0.443+/-0.080 vs 0.364+/-0.060, p<0.001), although correlation coefficients between these two methods were highly significant (r = 0.62-0.83, p<0.001). The reproducibility errors of metacarpal bone mineral density (mBMD) were constant (1.1-1.2%) whilst those for mCI showed a marked operator-dependency (2.0-7.9%). In 379 elderly community-dwelling women, Bonalyzer mCI and mBMD showed a significant decline with age (r = -0.30 and -0.27 respectively, p<0.05). Both mCI and mBMD correlated significantly with forearm BMD (r = 0.50 and 0.57 respectively, p<0.001) and hip BMD (r = 0.48 and 0.53 respectively, p<0.001). After adjustment for age and weight, hip BMD demonstrated the best discrimination for prevalent vertebral fractures as judged by the gradient of risk for a 1 SD decrease in measurement (odds ratio (OR) 2.17, 95% CI 1.56-3.01). Similar but smaller gradients of risk were shown by Bonalyzer mCI (OR 1.32, 95% CI 1.00-1.75), mBMD (OR 1.35, 95% CI 1.02-1.78) and forearm BMD (OR 1.39, 95% CI 1.08-1.80). MCI, and in particular mBMD, may be useful assessments of bone mass and fracture risk. In our study, it is comparable to peripheral assessment of skeletal status by forearm densitometry.

Studies of bone density, quantitative ultrasound, and vertebral fractures in relation to collagen type I alpha 1 alleles in elderly women.

Previous studies have demonstrated that an Sp1 binding site polymorphism in the collagen type I gene (COLIA1) is related to reduced bone mineral density (BMD) and osteoporotic fractures in certain populations, particularly in the elderly. We have examined the relationship among these COLIA1 Sp1 alleles, BMD, quantitative ultrasound properties of bone, and fractures in a population-based cohort of elderly women from the UK. The study group comprised 314 women aged 75 years and over who agreed to participate in a clinical study of bisphosphonate therapy in preventing bone loss at the hip. Women were enrolled regardless of the presence or absence of osteoporosis, but those with other diseases that might affect skeletal metabolism were excluded. The genotype distribution for the Sp1 polymorphism was in Hardy-Weinberg equilibrium (SS - 78%; Ss - 20%; ss - 2%) but the proportion of individuals who carried the "s" allele (22%) was significantly lower than previously observed in another study of the UK population (37.1%) (P < 0.001). There were no significant associations between COLIA1 genotypes and metacarpal cortical index, BMD of the forearm, tibial SOS, calcaneal SOS, or calcaneal BUA. While there was a trend towards lower BMD values at the hip in patients with Ss and ss genotypes, this was not statistically significant (SS = 0.721 +/- 0.14; Ss = 0.704 +/- 0.13; ss = 0.683 +/- 0.20 P = 0.6). Prevalent vertebral fractures occurred in 22% of subjects and prior fractures of the wrist, ankle, and hip were reported by 20%, but there was no significant difference in COLIA1 genotype distribution between fracture patients and controls. We conclude that COLIA1 Sp1 alleles are not significantly associated with BMD, ultrasound properties of bone, or fractures in this population-based sample of elderly women.