Aims. Tenosynovial giant cell tumour (TGCT) is one of the most common soft-tissue tumours of the foot and ankle and can behave in a locally aggressive manner. Tumour control can be difficult, despite the various methods of treatment available. Since treatment guidelines are lacking, the aim of this study was to review the multidisciplinary management by presenting the largest series of TGCT of the foot and ankle to date from two specialized sarcoma centres. Methods. The Oxford Tumour Registry and the Leiden University Medical Centre Sarcoma Registry were retrospectively reviewed for patients with histologically proven foot and ankle TGCT diagnosed between January 2002 and August 2019. Results. A total of 84 patients were included. There were 39 men and 45 women with a mean age at primary treatment of 38.3 years (9 to 72). The median follow-up was 46.5 months (interquartile range (IQR) 21.3 to 82.3). Localized-type TGCT (n = 15) predominantly affected forefoot, whereas diffuse-type TGCT (Dt-TGCT) (n = 9) tended to panarticular involvement. TGCT was not included in the radiological differential diagnosis in 20% (n = 15/75). Most patients had open rather than arthroscopic surgery (76 vs 17). The highest recurrence rates were seen with Dt-TGCT (61%; n = 23/38), panarticular involvement (83%; n = 5/8), and after arthroscopy (47%; n = 8/17). Three (4%) fusions were carried out for osteochondral destruction by Dt-TGCT. There were 14 (16%) patients with Dt-TGCT who underwent systemic treatment, mostly in refractory cases (79%; n = 11). TGCT initially decreased or stabilized in 12 patients (86%), but progressed in five (36%) during follow-up; all five underwent subsequent surgery. Side effects were reported in 12 patients (86%). Conclusion. We recommend open surgical excision as the primary treatment for TGCT of the foot and ankle, particularly in patients with Dt-TGCT with extra-articular involvement. Severe osteochondral destruction may justify salvage procedures, although these are not often undertaken. Systemic treatment is indicated for unresectable or refractory cases. However, side effects are commonly experienced, and relapses may occur once treatment has ceased. Cite this article: Bone Joint J 2021;103-B(4):788–794

Aims. Although chondrosarcomas (CSs) display true malignant features, including local recurrence (LR) and metastases, their behaviour in the hands and feet is thought to differ from that in other parts of the axial and appendicular skeleton by having a lower metastatic potential. The purpose of this study was to investigate the disease-specific and surgical factors that affect the local and systemic prognosis of CS of the hands and feet. Methods. A multicentre retrospective study was carried out at two tertiary sarcoma centres. A database search identified all patients with a CS treated between January 1995 and January 2018. There were 810 CSs of which 76 (9.4%) were located in the fingers, toes, metacarpals, and metatarsal bones. Results. The median age of the study population was 55 years (36 to 68) with a median follow-up of 52 months (22 to 87) months. Overall, 70% of the tumours were in the hand (n = 54) and 30% in the foot (n = 22). Predictors for LR were margin (p = 0.011), anatomical location (p = 0.017), and method of surgical management (p = 0.003). Anatomical location (p = 0.026), histological grade between 1 and 3 (p = 0.004) or 2 and 3 (p = 0.016), and surgical management (p = 0.001) were significant factors for LR-free survival. Disease-specific survival was affected by histological grade (p < 0.001), but not by LR (p = 0.397). Conclusion. Intralesional curettage of a low-grade CS is associated with an increased risk of LR, but LR does not affect disease-specific survival. Therefore, for low-grade CSs of the hands and feet, surgical management should aim to preserve function. In grade 2 CS, our study did not show any decreased disease-specific survival after recurrence; however, we suggest a more aggressive surgical approach to these tumours to prevent local recurrence, especially in the metacarpal and metatarsal bones. In high-grade tumours, the incidence of progressive disease is high and, therefore, the treatment of the primary tumour should be aggressive where possible, and patients observed closely for the development of metastatic disease. Cite this article: Bone Joint J 2021;103-B(3):562–568

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

Giant-cell tumour of the tendon sheath (GCT-TS) is a benign solitary tumour which usually arises in the limbs. It occurs most often in the hand where local recurrence after excision has been reported in up to 45% of cases. It is less common in the foot where the biological behaviour and risk of local recurrence have not been defined. We have studied 17 cases of GCT-TS of the foot and ankle in which treatment was by excision. Fifteen presented as a solitary, painless, slow-growing soft-tissue swelling. One lesion was associated with sensory deficit of a digital nerve and one with pain on walking. Thirteen cases originated from the periarticular tendon-sheath complex of the small joints of the toes and four from the capsule or long tendons of the ankle. A correct preoperative diagnosis was made in only three cases. MRI proved to be the most useful preoperative investigation as GCT-TS has a characteristic appearance which allows planned local excision to be carried out. None of the patients with histologically confirmed GCT-TS required further surgery. There was no local recurrence in 15 patients who were available for follow-up at a mean of 85 months

Giant cell tumours (GCTs) of the small bones of the hands and feet are rare. Small case series have been published but there is no consensus about ideal treatment. We performed a systematic review, initially screening 775 titles, and included 12 papers comprising 91 patients with GCT of the small bones of the hands and feet. The rate of recurrence across these publications was found to be 72% (18 of 25) in those treated with isolated curettage, 13% (2 of 15) in those treated with curettage plus adjuvants, 15% (6 of 41) in those treated by resection and 10% (1 of 10) in those treated by amputation.

We then retrospectively analysed 30 patients treated for GCT of the small bones of the hands and feet between 1987 and 2010 in five specialised centres. The primary treatment was curettage in six, curettage with adjuvants (phenol or liquid nitrogen with or without polymethylmethacrylate (PMMA)) in 18 and resection in six. We evaluated the rate of complications and recurrence as well as the factors that influenced their functional outcome.

At a mean follow-up of 7.9 years (2 to 26) the rate of recurrence was 50% (n = 3) in those patients treated with isolated curettage, 22% (n = 4) in those treated with curettage plus adjuvants and 17% (n = 1) in those treated with resection (p = 0.404). The only complication was pain in one patient, which resolved after surgical removal of remnants of PMMA. We could not identify any individual factors associated with a higher rate of complications or recurrence. The mean post-operative Musculoskeletal Tumor Society scores were slightly higher after intra-lesional treatment including isolated curettage and curettage plus adjuvants (29 (20 to 30)) compared with resection (25 (15 to 30)) (p = 0.091). Repeated curettage with adjuvants eventually resulted in the cure for all patients and is therefore a reasonable treatment for both primary and recurrent GCT of the small bones of the hands and feet.

Cite this article: Bone Joint J 2013;95-B:838–45.

Aims. The aim of this study was to report the results of three forms of reconstruction for patients with a ditsl tibial bone tumour: an intercalary resection and reconstruction, an osteoarticular reconstruction, and arthrodesis of the ankle. Methods. A total of 73 patients with a median age of 19 years (interquartile range (IQR) 14 to 36) were included in this retrospective, multicentre study. Results. Reconstructions included intercalary resection in 17 patients, osteoarticular reconstruction in 11, and ankle arthrodesis in 45. The median follow-up was 77 months (IQR 35 to 130). Local recurrence occurred in eight patients after a median of 14 months (IQR 9 to 36), without a correlation with adequacy of margins or reconstructive technique. Major complications included fracture of the graft in ten patients, nonunion of the proximal osteotomy in seven, and infection in five. In the osteoarticular group, three of 11 patients developed radiological evidence of severe osteoarthritis, but only one was symptomatic and required conversion to ankle arthrodesis. Functional evaluation showed higher values of the Musculoskeletal Tumour Society (MSTS) and American Orthopaedic Foot and Ankle Society (AOFAS) scores in the intercalary group compared with the others. Conclusion. Preservation of the epiphysis in patients with a distal tibial bone tumour is a safe and effective form of limb-sparing treatment. It requires rigorous preoperative planning after accurate analysis of the imaging. When joint-sparing resection is not indicated, ankle arthrodesis, either isolated tibiotalar or combined tibiotalar and subtalar arthrodesis, should be preferred over osteoarticular reconstruction. Cite this article: Bone Joint J 2020;102-B(11):1567–1573

Aims. The aim of this study was to evaluate the surgical management and outcome of patients with an acral soft-tissue sarcoma of the hand or foot. Patients and Methods. We identified 63 patients with an acral soft-tissue sarcoma who presented to our tertiary referral sarcoma service between 2000 and 2016. There were 35 men and 28 women with a mean age of 49 years (. sd. 21). Of the 63 sarcomas, 27 were in the hands and 36 in the feet. The commonest subtypes were epithelioid sarcoma in the hand (n = 8) and synovial sarcoma in the foot (n = 11). Results. In 41 patients (65%), the tumour measured less than 5 cm in its largest dimension (median size 3 cm (٢ to ٦)); 27 patients (43%) were diagnosed after inadvertent excision prior to their referral to the specialist sarcoma unit. After biopsy and staging, primary surgical intervention at the sarcoma unit was excision and limb salvage in 43 (68%), partial (digit or ray) amputation in 14 (22%), and more proximal amputation in six (10%). At final follow up, local recurrence had been treated by one partial amputation and six amputations, resulting in a partial amputation rate of 24% and a proximal amputation rate of 19%. The five-year survival rate was 82%. Patients who underwent inadvertent excision showed no statistically significant difference in survival or local recurrence, but were more likely to undergo amputation (p = 0.008). Large tumour size (> 5 cm) was associated with lower survival (p = 0.04) and a higher risk of local recurrence (p = ٠.٠٠٩). Conclusion. Most acral soft-tissue sarcomas measure less than 5 cm at presentation, indicating that while size can be a useful prognostic factor, it should not be used as a diagnostic threshold for referral. Increased tumour size is associated with a higher rate of local recurrence and reduced survival. Sarcoma excision with limb preservation does not result in an increased risk of local recurrence. Cite this article: Bone Joint J 2018;100-B:1518–23

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The aim of this study was to report the patterns of symptoms and insufficiency fractures in patients with tumour-induced osteomalacia (TIO) to allow the early diagnosis of this rare condition.

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Most patients with advanced malignancy suffer bone metastases, which pose a significant challenge to orthopaedic services and burden to the health economy. This study aimed to assess adherence to the British Orthopaedic Oncology Society (BOOS)/British Orthopaedic Association (BOA) guidelines on patients with metastatic bone disease (MBD) in the UK.

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. Methods. Between January 2010 and December 2016, 56 patients were treated primarily with serial intralesional sclerosant injections. Their mean age was 20 years (1 to 54). The sites involved were clavicle (n = 3), humeri (n = 11), radius (n = 1), ulna (n = 3), hand (n = 2), pelvis (n = 12), femur (n = 7), tibia (n = 13), fibula (n = 3), and foot (n = 1). After histopathological confirmation of the diagnosis, 3% polidocanol (hydroxypolyaethoxydodecan) was injected into the lesion under image intensifier guidance. Patients were evaluated clinically and radiologically every six to eight weeks. In the absence of clinical and/or radiological response, a repeat sclerosant injection was given after eight to 12 weeks and repeated at similar intervals if necessary. Results. There were no complications of treatment. One patient was lost to follow-up. Overall, 46/55 (84%) of lesions healed after one or more injections of polidocanol: 24/55 (44%) patients healed with a single injection, and 43/55 (78%) within two injections. Of these 46, four (9%) patients developed local recurrence, two of whom healed with a repeat sclerosant injection. Thus, 44/55 (80%) patients of primary ABC healed with sclerotherapy. The mean follow-up was 62 months (20 to 111). The local recurrence free survival (LRFS) with percutaneous sclerosant therapy with polidocanol was 100%, 98% (95% confidence interval (CI) 85 to 100) and 93% (95% CI 78 to 98) at two, three, and five years, respectively. Conclusion. Percutaneous sclerotherapy using polidocanol is a safe, effective, minimally invasive and inexpensive method of treating a primary ABC of the limbs or pelvis. Cite this article: Bone Joint J 2020;102-B(2):186–190

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Current literature suggests that survival outcomes and local recurrence rates of primary soft-tissue sarcoma diagnosed in the very elderly age range, (over 90 years), are comparable with those in patients diagnosed under the age of 75 years. Our aim is to quantify these outcomes with a view to rationalizing management and follow-up for very elderly patients.

Aims. Due to the complex anatomy of the pelvis, limb-sparing resections of pelvic tumours achieving adequate surgical margins, can often be difficult. The advent of computer navigation has improved the precision of resection of these lesions, though there is little evidence comparing resection with or without the assistance of navigation. Our aim was to evaluate the efficacy of navigation-assisted surgery for the resection of pelvic bone tumours involving the posterior ilium and sacrum. . Patients and Methods. Using our prospectively updated institutional database, we conducted a retrospective case control study of 21 patients who underwent resection of the posterior ilium and sacrum, for the treatment of a primary sarcoma of bone, between 1987 and 2015. The resection was performed with the assistance of navigation in nine patients and without navigation in 12. We assessed the accuracy of navigation-assisted surgery, as defined by the surgical margin and how this affects the rate of local recurrence, the disease-free survival and the effects on peri-and post-operative morbidity. . Results. The mean age of the patients was 36.4 years (15 to 66). The mean size of the tumour was 10.9 cm. In the navigation-assisted group, the margin was wide in two patients (16.7%), marginal in six (66.7%) and wide-contaminated in one (11.1%) with no intralesional margin. In the non-navigated-assisted group; the margin was wide in two patients (16.7%), marginal in five (41.7%), intralesional in three (25.0%) and wide-contaminated in two (16.7%). Local recurrence occurred in two patients in the navigation-assisted group (22.2%) and six in the non-navigation-assisted group (50.0%). The disease-free survival was significantly better when operated with navigation-assistance (p = 0.048). The blood loss and operating time were less in the navigated-assisted group, as was the risk of a foot drop post-operatively. Conclusion . The introduction of navigation-assisted surgery for the resection of tumours of the posterior ilium and sacrum has increased the safety for the patients and allows for a better oncological outcome. . Cite this article: Bone Joint J 2017;99-B:261–6

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The aims of the study were to analyze differences in surgical and oncological outcomes, as well as quality of life (QoL) and function in patients with ankle sarcomas undergoing three forms of surgical treatment, minor or major limb salvage surgery (LSS), or amputation.

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Chondrosarcoma is the second most common primary sarcoma of bone: conventional chondrosarcoma accounts for 85% of all cases. Conventional chondrosarcoma may be central or peripheral. Most studies group central and peripheral chondrosarcomas together, although there is growing evidence that their clinical behaviour and prognosis differ. The aims of this study were to analyze any differences in characteristics between central and peripheral chondrosarcomas and to investigate the incidence and role of different syndromes.

Patients who have limb amputation for musculoskeletal tumours are a rare group of cancer survivors. This was a prospective cross-sectional survey of patients from five specialist centres for sarcoma surgery in England. Physical function, pain and quality of life (QOL) outcomes were collected after lower extremity amputation for bone or soft-tissue tumours to evaluate the survivorship experience and inform service provision. Of 250 patients, 105 (42%) responded between September 2012 and June 2013. From these, completed questionnaires were received from 100 patients with a mean age of 53.6 years (19 to 91). In total 60 (62%) were male and 37 (38%) were female (three not specified). The diagnosis was primary bone sarcoma in 63 and soft-tissue tumour in 37. A total of 20 tumours were located in the hip or pelvis, 31 above the knee, 32 between the knee and ankle and 17 in the ankle or foot. In total 22 had hemipelvectomy, nine hip disarticulation, 35 transfemoral amputation, one knee disarticulation, 30 transtibial amputation, two toe amputations and one rotationplasty. The Toronto Extremity Salvage Score (TESS) differed by amputation level, with poorer scores at higher levels (p < 0.001). Many reported significant pain. In addition, TESS was negatively associated with increasing age, and pain interference scores. QOL for Cancer Survivors was significantly correlated with TESS (p < 0.001). This relationship appeared driven by pain interference scores. . This unprecedented national survey confirms amputation level is linked to physical function, but not QOL or pain measures. Pain and physical function significantly impact on QOL. These results are helpful in managing the expectations of patients about treatment and addressing their complex needs. Cite this article: Bone Joint J 2015;97-B:1284–90

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Our aim was to develop and validate nomograms that would predict the cumulative incidence of sarcoma-specific death (CISSD) and disease progression (CIDP) in patients with localized high-grade primary central and dedifferentiated chondrosarcoma.

Schwannomas are the most common tumours of the sheath of peripheral nerves. The clinical diagnosis is usually straightforward, but may be delayed for many years in a schwannoma of the posterior tibial nerve. The symptoms are often attributed to entrapment neuropathy or to lumbosacral radiculopathy. We describe 25 patients with a schwannoma of the posterior tibial nerve. Only three were diagnosed within a year of presentation. The mean time to diagnosis was 86.5 months with a median of 48 months (2 to 360). All the patients complained of pain, which was felt specifically in the sole of the foot in 18. A Tinel sign was detected in all 25 patients. MRI confirmed the diagnosis in all the cases in which it had been undertaken. Surgical resection of the lesion abolished the neuropathic pain. In patients with a long history of neuropathic pain in the lower limb in whom lumbar and pelvic lesions have been excluded, a benign tumour of the sheath of a peripheral nerve may explain the symptoms. Surgical resection of the tumour is safe and effective

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

Resection of a primary sarcoma of the diaphysis of a long bone creates a large defect. The biological options for reconstruction include the use of a vascularised and non-vascularised fibular autograft. The purpose of the present study was to compare these methods of reconstruction. Between 1985 and 2007, 53 patients (26 male and 27 female) underwent biological reconstruction of a diaphyseal defect after resection of a primary sarcoma. Their mean age was 20.7 years (3.6 to 62.4). Of these, 26 (49 %) had a vascularised and 27 (51 %) a non-vascularised fibular autograft. Either method could have been used for any patient in the study. The mean follow-up was 52 months (12 to 259). Oncological, surgical and functional outcome were evaluated. Kaplan–Meier analysis was performed for graft survival with major complication as the end point. At final follow-up, eight patients had died of disease. Primary union was achieved in 40 patients (75%); 22 (42%) with a vascularised fibular autograft and 18 (34%) a non-vascularised (p = 0.167). A total of 32 patients (60%) required revision surgery. Kaplan–Meier analysis revealed a mean survival without complication of 36 months (0.06 to 107.3, . sd. 9) for the vascularised group and 88 months (0.33 to 163.9, . sd. 16) for the non-vascularised group (p = 0.035). . Both groups seem to be reliable biological methods of reconstructing a diaphyseal bone defect. Vascularised autografts require more revisions mainly due to problems with wound healing in distal sites of tumour, such as the foot. Cite this article: Bone Joint J 2014;96-B:1258–63

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The aim of this study was to report the results of custom-made endoprostheses with extracortical plates plus or minus a short, intramedullary stem aimed at preserving the physis after resection of bone sarcomas in children.