Introduction. Angiosarcomas are rare aggressive sarcomas of vascular endothelial origin. These tumours have the potential to be multicentric and are associated with high rates of local recurrence, which makes treatment challenging. The gold-standard is that these patients are managed in specialist centres by a multidisciplinary team. We present our experience of managing patients with angiosarcoma in the North of England Bone and Soft Tissue Tumour Service and a review of the literature. Methods. A prospectively collated electronic database was used to identify patients with angiosarcoma treated between 2000 and 2008, and an analysis performed of demographics, anatomical site, surgical excision and reconstruction, local disease recurrence and metastatic disease. Results. Fifteen patients (ten female, ?ve male, mean age 71 years) were identi?ed. Eight patients developed tumours in a previously irradiated area, after a mean of 11 years. Six patients had metastatic disease at presentation. Fourteen patients underwent wide surgical excision of the tumour, of which nine required defect reconstruction(?ve free latissimus dorsi ?aps, two free anterolateral thigh ?aps, two pedicled latissimus dorsi ?aps). One patient was treated with chemotherapy only. Five of 14 patients received adjuvant radiotherapy, and one received adjuvant chemotherapy. Two out of 14 patients developed local recurrence. Eight patients developed metastases, the majority of which were pulmonary. Estimated ?ve-year survival was calculated as 33% in our patient cohort. Conclusions. Angiosarcomas are aggressive, difficult to treat tumours, which can occur secondary to a multitude of causes. Clinical suspicion, biopsy and early diagnosis are essential to allow optimum treatment, which currently consists of radical surgery, together with adjuvant radiotherapy and chemotherapy

Aims. Clear cell sarcoma (CCS) of soft-tissue is a rare melanocytic subtype of mesenchymal malignancy. The aim of this study was to investigate the clinical and therapeutic factors associated with increased survival, stratified by clinical stage, in order to determine the optimal treatment. Methods. The study was a retrospective analysis involving 117 patients with histologically confirmed CCS, between July 2016 and November 2017, who were enrolled in the Bone and Soft Tissue Tumour Registry in Japan. Results. The five- and ten-year survival rates were 41% (95% confidence interval (CI) 29 to 52) and 37% (95% CI 25 to 49), respectively. On multivariable analysis, the size of the tumour of > 10 cm (p = 0.006), lymph node metastasis at the time of diagnosis (p < 0.001), distant metastases at the time of diagnosis (p < 0.001), and no surgery for the primary tumour (p = 0.019) were independently associated with a poor survival. For N0M0 CCS (n = 68), the development of distant metastases was an independent prognostic factor for survival (early (< 12 months), hazard ratio (HR) 116.78 (95% CI 11.69 to 1,166.50); p < 0.001; late (> 12 months), HR 14.79 (95% CI 1.66 to 131.63); p = 0.016); neoadjuvant/adjuvant chemotherapy (p = 0.895) and/or radiotherapy (p = 0.216) were not significantly associated with survival. The five-year cumulative incidence of local recurrence was 19% (95% CI 8 to 35) and the size of the tumour was significantly associated with an increased rate of local recurrence (p = 0.012). For N1M0 CCS (n = 18), the risk of mortality was significantly lower in patients who underwent surgery for both the primary tumour and lymph node metastases (HR 0.03 (95% CI 0.00 to 0.56); p = 0.020). For M1 CCS (n = 31), excision of the primary tumour was independently associated with better survival (HR 0.26 (95% CI 0.09 to 0.76); p = 0.013). There was no significant difference in survival between the different types of systemic treatment (p = 0.523). Conclusion. Complete excision of the primary tumour and lymph nodes is associated with a better survival in patients with CCS. Systemic treatment appears to provide limited benefits, demonstrating a pressing need for novel systemic agents. Cite this article: Bone Joint J 2023;105-B(11):1216–1225

Aim. Bumps and lumps of the hand are a common cause for consultation in general practice. However not all of these lesions are of true neoplastic nature and malignant tumours are a rarity in this location. Method. The records of all tumours of the hand and wrist treated surgically at our institution in the period 1994 to 2009 were reviewed. Because of their non-neoplastic nature typical lesions of the hand such as ganglion cysts or palmar fibromatosis and the like were not included in this study. Histological entity, location, radiographic and clinical findings were analysed; malignant tumours were followed up by X-ray and MRI. Results. Out of 142 cases which were further investigated, there were 86 bone tumours and 56 soft tissue lesions. The mean age was 41 years (range 8 to 85), there were 57 male and 85 female patients.122 cases (86%) were benign lesions, 71 cases located in the bone and 52 cases in the soft tissue respectively. There were twenty malignant lesions, sixteen of which were bone tumours; the most common being chondrosarcoma (11), followed by metastases (3) and osteosarcoma (2). The group of malignant soft tissue tumours (4) was comprised of synovial sarcoma (2), Ewing sarcoma (1) and extraosseus myxoid chondrosarcoma (1). Out of 52 benign soft tissue tumours, pigmented villonodular synovitis (32), haemangioma (6) and lipomatous tumours (6) have been the most frequent entities. Chondroma (48), osteoid-osteoma (4) and giant cell tumour (3) were the most common benign bone tumours. Conclusion. Knowledge of tumour prevalence under consideration of the patient's age and location of the tumour is an important prerequisite for identifying neoplastic lesions of the hand

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We performed a systematic literature review to define features of patients, treatment, and biological behaviour of multicentric giant cell tumour (GCT) of bone.

Aims

The modified Glasgow Prognostic Score (mGPS) uses preoperative CRP and albumin to calculate a score from 0 to 2 (2 being associated with poor outcomes). mGPS is validated in multiple carcinomas. To date, its use in soft-tissue sarcoma (STS) is limited, with only small cohorts reporting that increased mGPS scores correlates with decreased survival in STS patients.

Introduction. General Practitioners will be confronted with a sarcoma rarely in their working lives. Most will never see a bone sarcoma although most will see at least one soft tissue sarcoma. Guidelines designed to lead to earlier diagnosis of the most common cancers were introduced by NICE in 2000 and were updated in 2005, containing basic guidance on the earlier diagnosis of bone and soft tissue tumours. Referral criteria include: masses greater than 5cm in diameter, masses deep to fascia, masses which are fixed or immobile, masses which are painful, those which are increasing in size and recurrence after previous excision. Methods. We examined 350 General Practitioner referrals over the past 3 years to our service and examined the correlation between the above criteria and the likelihood of malignancy. Results. 33.3% of all tumours were malignant. The likelihood of a painful tumour proving malignant was 33.1% and therefore pain was not a valid predictor of malignancy (p = 0.86). Effectively, pain did not confer a relative risk. The likelihood of a growing tumour proving malignant was 38.5% and therefore increasing size is a predictor of malignancy (p = 0.03). Increasing size conferred a relative risk of 1.42 on a growing tumour. 67% of GP's said that they felt uncertain in determining whether a lesion was deep to fascia. Discussion. Whilst the introduction of the NICE guidelines has provided a clearer indication for referral than existed previously, we did not find all the referral criteria to be of particular value. In particular, pain did not correlate strongly with the likelihood of a tumour proving malignant. Specifically, GPs need further education to improve their working knowledge of this rare disease

Aim. Grade is the most important predictor of the biological behaviour of soft tissue sarcomas. Assigning a pathologic grade is always a difficult task as discordance rate is 30-40% even among experienced sarcoma pathologists. Many of these tumours are heterogeneously large and only small fractions are sampled for biopsy. This emphasizes the need for an objective and accurate assessment of histology. Our aim is to evaluate the role of Choline as a tumour marker in (i) differentiating benign from malignant soft tissue tumour, (ii) to distinguish recurrent/residual tumours using in-vivo MR spectroscopy. Methods. PMRS Study was performed at 1.5Tesla MRI machine of the lesions in 25 patients. Single-voxel (SVS) study has been done in 10 cases and chemical shift imaging (CSI) study characterised the heterogeneity of the tumour in 15 cases by using point – resolved spectroscopic sequence (PRESS) with echo time TR=2000/TE = 30, 135 & 270 msec. The choline peak, identified at 3.2 ppm in spectra was considered significant. MRS results and histopathologic findings were correlated and P < 0.001, considered being significant. Results. Choline peak was found in 17 out of 17 patients with sarcomas where as three patient with benign and five treated sarcomas patients with no residual disease did not show any choline. In vivo spectroscopy here shows sensitivity, specificity, positive predictive value, negative predictive value and accuracy of 100% each. In vivo spectroscopy shows sensitivity, specificity, positive predictive value, negative predictive value and accuracy of 100% each where as preoperative biopsy shows 75%, 100%, 100%, 72.7% and 85% respectively. Conclusion. Choline peak in PMRS study can predict the grade, margin status and tumour activity in recurrent and/or residual tumour. A major study should be done to validate its efficacy for routine use in oncology

Aim. Grade is the most important predictor of the biological behaviour of soft tissue sarcomas. Assigning a pathologic grade is always a difficult task as discordance rate is 30-40% even among experienced sarcoma pathologists. Many of these tumours are heterogeneously large and only small fractions are sampled for biopsy. This emphasizes the need for an objective and accurate assessment of histology. Our aim is to evaluate the role of Choline as a tumour marker in (i) differentiating benign from malignant soft tissue tumour, (ii) to distinguish recurrent/residual tumours using in-vivo MR spectroscopy. Methods. PMRS Study was performed at 1.5Tesla MRI machine of the lesions in 25 patients. Single-voxel (SVS) study has been done in 10 cases and chemical shift imaging (CSI) study characterised the heterogeneity of the tumour in 15 cases by using point – resolved spectroscopic sequence (PRESS) with echo time TR=2000/TE = 30, 135 & 270 msec. The choline peak, identified at 3.2 ppm in spectra was considered significant. MRS results and histopathologic findings were correlated and P < 0.001, considered being significant. Results. Choline peak was found in 17 out of 17 patients with sarcomas where as three patient with benign and five treated sarcomas patients with no residual disease did not show any choline. In vivo spectroscopy here shows sensitivity, specificity, positive predictive value, negative predictive value and accuracy of 100% each. In vivo spectroscopy shows sensitivity, specificity, positive predictive value, negative predictive value and accuracy of 100% each where as preoperative biopsy shows 75%, 100%, 100%, 72.7% and 85% respectively. Conclusion. Choline peak in PMRS study can predict the grade, margin status and tumour activity in recurrent and/or residual tumour. A major study should be done to validate its efficacy for routine use in oncology

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Time to treatment initiation (TTI) is generally defined as the time from the histological diagnosis of malignancy to the initiation of first definitive treatment. There is no consensus on the impact of TTI on the overall survival in patients with a soft-tissue sarcoma. The purpose of this study was to determine if an increased TTI is associated with overall survival in patients with a soft-tissue sarcoma, and to identify the factors associated with a prolonged TTI.

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The aim of this study was to assess orthopaedic oncologic patient morbidity resulting from COVID-19 related institutional delays and surgical shutdowns during the first wave of the pandemic in New York, USA.

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Iliac wing (Type I) and iliosacral (Type I/IV) pelvic resections for a primary bone tumour create a large segmental defect in the pelvic ring. The management of this defect is controversial as the surgeon may choose to reconstruct it or not. When no reconstruction is undertaken, the residual ilium collapses back onto the remaining sacrum forming an iliosacral pseudarthrosis. The aim of this study was to evaluate the long-term oncological outcome, complications, and functional outcome after pelvic resection without reconstruction.

Aims

Limb salvage in bone tumour patients replaces the bone with massive segmental prostheses where achieving bone integration at the shoulder of the implant through extracortical bone growth has been shown to prevent loosening. This study investigates the effect of multidrug chemotherapy on extracortical bone growth and early radiological signs of aseptic loosening in patients with massive distal femoral prostheses.

Aims

Survival rates and local control after resection of a sarcoma of the pelvis compare poorly to those of the limbs and have a high incidence of complications. The outcome for patients who need a hindquarter amputation (HQA) to treat a pelvic sarcoma is poor. Our aim was to evaluate the patient, tumour, and reconstructive factors that affect the survival of the patients who undergo HQA for primary or recurrent pelvic sarcoma.

Aims

We retrospectively report our experience of managing 30 patients with a primary malignant tumour of the distal tibia; 25 were treated by limb salvage surgery and five by amputation. We compared the clinical outcomes of following the use of different methods of reconstruction.

Objectives

To assess the accuracy of patient-specific instruments (PSIs) versus standard manual technique and the precision of computer-assisted planning and PSI-guided osteotomies in pelvic tumour resection.

In our database of 7935 patients referred for investigation of a soft-tissue mass, only 100 were found to have a soft-tissue metastasis (1.3%). Our aim was to define the clinical features of such patients and to identify the site of their primary tumour.

The most common presentation was a painful lump, deep to the fascia, ranging between 2 cm and 35 cm (mean 8.3 cm) with 78% of the lumps located deep to the fascia. The mean age of the patients at presentation was 64 years (22 to 84) and there were almost equal numbers of men and women. Of 53 patients with a history of malignancy, 52 had metastases from the same primary (lung in 12, melanoma in ten, kidney in nine, gastrointestinal track in four, breast in five, bladder in four, and others in eight). The other 47 had no history of malignancy and the metastasis was the first presentation. The primary sites in these cases were the lung in 19, gastro-intestinal track in four, kidney in two, melanoma in nine, other in three, and unknown (despite investigations) in ten. There was no correlation between the site of the metastases and the primary tumour.

Of the 7935 patients, 516 had a history of malignancy. Of these, only 10% had a soft-tissue metastasis, 29% had a benign diagnosis, 55% a soft-tissue sarcoma and 6% another malignancy. Patients with soft-tissue metastases have similar clinical features to those with soft-tissue sarcomas and should be considered for assessment at appropriate diagnostic centres for patients with suspicious soft-tissue lumps.

We have analysed the pattern of symptoms in patients presenting with synovial sarcoma to identify factors which led to long delays in diagnosis. In 35 children, the early symptoms and the results of clinical and radiological investigation were reviewed, along with the presumed diagnoses. The duration of symptoms was separated into patient delay and doctor delay.

Only half of the patients had one or more of the four clinical findings suggestive of sarcoma according to the guidance of the National Institute for Clinical Excellence at the onset of symptoms. Of the 33 children for whom data were available, 16 (48.5%) presented with a painless mass and in ten (30.3%) no mass was identified. Seven (21.2%) had an unexplained joint contracture. Many had been extensively investigated unsuccessfully. The mean duration of symptoms was 98 weeks (2 to 364), the mean patient delay was 43 weeks (0 to 156) and the mean doctor delay was 50 weeks (0 to 362). The mean number of doctors seen before referral was three (1 to 6) and for 15 patients the diagnosis was obtained after unplanned excision. Tumours around the knee and elbow were associated with a longer duration of symptoms and longer doctor delay compared with those at other sites. Delays did not improve significantly over the period of our study of 21 years, and we were unable to show that delay in diagnosis led to a worse prognosis.

Our findings highlight the variety of symptoms associated with synovial sarcoma and encourage greater awareness of this tumour as a potential diagnosis in childhood.

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.