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The Bone & Joint Journal
Vol. 103-B, Issue 4 | Pages 788 - 794
1 Apr 2021
Spierenburg G Lancaster ST van der Heijden L Mastboom MJL Gelderblom H Pratap S van de Sande MAJ Gibbons CLMH

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


The Bone & Joint Journal
Vol. 103-B, Issue 3 | Pages 553 - 561
1 Mar 2021
Smolle MA Leithner A Kapper M Demmer G Trost C Bergovec M Windhager R Hobusch GM

Aims. 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. Methods. A total of 69 patients with ankle sarcomas, treated between 1981 and 2017 at two tumour centres, were retrospectively reviewed (mean age at surgery: 46.3 years (SD 22.0); 31 females (45%)). Among these 69 patients 25 were analyzed prospectively (mean age at latest follow-up: 61.2 years (SD 20.7); 11 females (44%)), and assessed for mobility using the Prosthetic Limb Users Survey of Mobility (PLUS-M; for amputees only), the Toronto Extremity Salvage Score (TESS), and the University of California, Los Angeles (UCLA) Activity Score. Individual QoL was evaluated in these 25 patients using the five-level EuroQol five-dimension (EQ-5D-5L) and Fragebogen zur Lebenszufriedenheit/Questions on Life Satisfaction (FLZ). Results. Of the total number of patients in the study, 22 (32%) underwent minor LSS and 22 (32%) underwent major LSS; 25 underwent primary amputation (36%). Complications developed in 26 (38%) patients, and were more common in those with major or minor LSS in comparison to amputation (59% vs 36% vs 20%; p = 0.022). A time-dependent trend towards higher complication risk following any LSS was present (relative risk: 0.204; 95% confidence interval (CI) 0.026 to 1.614; p = 0.095). In the prospective cohort, mean TESS was higher following minor LSS in comparison to amputation (91.0 vs 67.3; p = 0.006), while there was no statistically significant difference between major LSS and amputation (81.6 vs 67.3; p = 0.099). There was no difference in mean UCLA (p = 0.334) between the three groups (p = 0.334). None of the items in FLZ or EQ-5D-5L were different between the three groups (all p > 0.05), except for FLZ item “self-relation”, being lower in amputees. Conclusion. Complications are common following LSS for ankle sarcomas. QoL is comparable between patients with LSS or amputation, despite better mobility scores for patients following minor LSS. We conclude that these results allow a decision for amputation to be made more easily in patients particularly where the principles of oncological surgery would otherwise be at risk. Cite this article: Bone Joint J 2021;103-B(3):553–561


The Journal of Bone & Joint Surgery British Volume
Vol. 92-B, Issue 3 | Pages 424 - 429
1 Mar 2010
Cribb GL Loo SCS Dickinson I

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


The Journal of Bone & Joint Surgery British Volume
Vol. 91-B, Issue 10 | Pages 1378 - 1382
1 Oct 2009
Shekkeris AS Hanna SA Sewell MD Spiegelberg BGI Aston WJS Blunn GW Cannon SR Briggs TWR

Endoprosthetic replacement of the distal tibia and ankle joint for a primary bone tumour is a rarely attempted and technically challenging procedure. We report the outcome of six patients treated between 1981 and 2007. There were four males and two females, with a mean age of 43.5 years (15 to 75), and a mean follow-up of 9.6 years (1 to 27). No patient developed a local recurrence or metastasis. Two of the six went on to have a below-knee amputation for persistent infection after a mean 16 months (1 to 31). The four patients who retained their endoprosthesis had a mean musculoskeletal tumour society score of 70% and a mean Toronto extremity salvage score of 71%. All were pain free and able to perform most activities of daily living in comfort. A custom-made endoprosthetic replacement of the distal tibia and ankle joint is a viable treatment option for carefully selected patients with a primary bone tumour. Patients should, however, be informed of the risk of infection and the potential need for amputation if this cannot be controlled


The Journal of Bone & Joint Surgery British Volume
Vol. 84-B, Issue 7 | Pages 1000 - 1003
1 Sep 2002
Gibbons CLMH Khwaja HA Cole AS Cooke PH Athanasou NA

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


The Bone & Joint Journal
Vol. 102-B, Issue 11 | Pages 1567 - 1573
7 Nov 2020
Sambri A Dalla Rosa M Scorianz M Guido D Donati DM Campanacci DA De Paolis M

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


Bone & Joint Open
Vol. 4, Issue 11 | Pages 817 - 824
1 Nov 2023
Filis P Varvarousis D Ntritsos G Dimopoulos D Filis N Giannakeas N Korompilias A Ploumis A

Aims

The standard of surgical treatment for lower limb neoplasms had been characterized by highly interventional techniques, leading to severe kinetic impairment of the patients and incidences of phantom pain. Rotationplasty had arisen as a potent limb salvage treatment option for young cancer patients with lower limb bone tumours, but its impact on the gait through comparative studies still remains unclear several years after the introduction of the procedure. The aim of this study is to assess the effect of rotationplasty on gait parameters measured by gait analysis compared to healthy individuals.

Methods

The MEDLINE, Scopus, and Cochrane databases were systematically searched without time restriction until 10 January 2022 for eligible studies. Gait parameters measured by gait analysis were the outcomes of interest.


The Bone & Joint Journal
Vol. 105-B, Issue 5 | Pages 568 - 574
1 May 2023
Kobayashi H Ito N Nakai Y Katoh H Okajima K Zhang L Tsuda Y Tanaka S

Aims

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.

Methods

The study included 33 patients with TIO who were treated between January 2000 and June 2022. The causative tumour was detected in all patients. We investigated the symptoms and evaluated the radiological patterns of insufficiency fractures of the rib, spine, and limbs.


Aims

To evaluate mid-to long-term patient-reported outcome measures (PROMs) of endoprosthetic reconstruction after resection of malignant tumours arising around the knee, and to investigate the risk factors for unfavourable PROMs.

Methods

The medical records of 75 patients who underwent surgery between 2000 and 2020 were retrospectively reviewed, and 44 patients who were alive and available for follow-up (at a mean of 9.7 years postoperatively) were included in the study. Leg length discrepancy was measured on whole-leg radiographs, and functional assessment was performed with PROMs (Toronto Extremity Salvage Score (TESS) and Comprehensive Outcome Measure for Musculoskeletal Oncology Lower Extremity (COMMON-LE)) with two different aspects. The thresholds for unfavourable PROMs were determined using anchor questions regarding satisfaction, and the risk factors for unfavourable PROMs were investigated.


The Bone & Joint Journal
Vol. 104-B, Issue 12 | Pages 1352 - 1361
1 Dec 2022
Trovarelli G Pala E Angelini A Ruggieri P

Aims

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

Methods

The search terms used in combination were “multicentric”, “giant cell tumour”, and “bone”. Exclusion criteria were: reports lacking data, with only an abstract; papers not reporting data on multicentric GCT; and papers on multicentric GCT associated with other diseases. Additionally, we report three patients treated under our care.


Bone & Joint Open
Vol. 4, Issue 11 | Pages 846 - 852
8 Nov 2023
Kim RG Maher AW Karunaratne S Stalley PD Boyle RA

Aims

Tenosynovial giant cell tumour (TGCT) is a rare benign tumour of the musculoskeletal system. Surgical management is fraught with challenges due to high recurrence rates. The aim of this study was to describe surgical treatment and evaluate surgical outcomes of TGCT at an Australian tertiary referral centre for musculoskeletal tumours and to identify factors affecting recurrence rates.

Methods

A prospective database of all patients with TGCT surgically managed by two orthopaedic oncology surgeons was reviewed. All cases irrespective of previous treatment were included and patients without follow-up were excluded. Pertinent tumour characteristics and surgical outcomes were collected for analysis.


The Bone & Joint Journal
Vol. 104-B, Issue 10 | Pages 1180 - 1188
1 Oct 2022
Qu H Mou H Wang K Tao H Huang X Yan X Lin N Ye Z

Aims

Dislocation of the hip remains a major complication after periacetabular tumour resection and endoprosthetic reconstruction. The position of the acetabular component is an important modifiable factor for surgeons in determining the risk of postoperative dislocation. We investigated the significance of horizontal, vertical, and sagittal displacement of the hip centre of rotation (COR) on postoperative dislocation using a CT-based 3D model, as well as other potential risk factors for dislocation.

Methods

A total of 122 patients who underwent reconstruction following resection of periacetabular tumour between January 2011 and January 2020 were studied. The risk factors for dislocation were investigated with univariate and multivariate logistic regression analysis on patient-specific, resection-specific, and reconstruction-specific variables.


The Journal of Bone & Joint Surgery British Volume
Vol. 89-B, Issue 10 | Pages 1356 - 1362
1 Oct 2007
Lampasi M Magnani M Donzelli O

We report the results of the treatment of nine children with an aneurysmal bone cyst of the distal fibula (seven cysts were juxtaphyseal, and two metaphyseal). The mean age of the children was 10 years and 3 months (7 years and 4 months to 12 years and 9 months). All had open physes. All cysts were active and in seven cases substituted and expanded the entire width of the bone (type-2 lesions). The mean longitudinal extension was 5.7 cm (3 to 10). The presenting symptoms were pain, swelling and pathological fracture. Moderate fibular shortening was evident in one patient. In six patients curettage was performed, using phenol as adjuvant in three. Three with juxtaphyseal lesions underwent resection. A graft from the contralateral fibula (one case) and allografts (two cases) were positioned at the edge of the physis for reconstruction. The mean follow-up was 11.6 years (3.1 to 27.5). There was no recurrence. At the final follow-up there was no significant difference in the American Orthopaedic Foot and Ankle Society scores (excellent/good in all cases) and in growth disturbance, alignment, stability and bone reconstitution, but in the resection group the number of operations, including removal of hardware, complications (two minor) and time of immobilisation/orthosis, were increased. Movement of the ankle was restricted in one patient. The potential risks in the management of these lesions include recurrence, physeal injury, instability of the ankle and hardware and graft complications. Although resection is effective it should be reserved for aggressive or recurrent juxtaphyseal lesions


The Bone & Joint Journal
Vol. 97-B, Issue 9 | Pages 1284 - 1290
1 Sep 2015
Furtado S Grimer RJ Cool P Murray SA Briggs T Fulton J Grant K Gerrand CH

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


The Journal of Bone & Joint Surgery British Volume
Vol. 91-B, Issue 9 | Pages 1227 - 1231
1 Sep 2009
Abuhassan FO Shannak AO

We describe the treatment by subperiosteal resection of an aneurysmal bone cyst in the distal fibula in eight patients and highlight the role of the periosteum in the regeneration of bone defects. The mean age of the patients was 13.5 years (12 to 17). Seven had an open growth plate. The mean size of the resected specimen was 5.12 cm (3.5 to 8.0). None of the patients received instillation of bone marrow, autogenous bone graft, allograft or any synthetic bone substitutes. All had complete regeneration of the bone defect within three to nine months, with no joint instability or recurrence. The mean length of follow-up was 11.5 years (2 to 18). At the final follow-up there was no difference in the range of movement, alignment or stability of the ankle when compared with the opposite side. The periosteum played a major role in the complete filling of the bone defects and avoided the morbidity of other techniques


The Bone & Joint Journal
Vol. 100-B, Issue 12 | Pages 1633 - 1639
1 Dec 2018
Zhao Z Yan T Guo W Yang R Tang X Yang Y

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.

Patients and Methods

There were 19 male and 11 female patients. The mean age of the patients was 19 years (6 to 59) and the mean follow-up was 5.1 years (1.25 to 12.58). Massive allograft was used in 11 patients, and autograft was used in 14 patients. The time to union, the survival time of the reconstruction, complication rate, and functional outcomes following the different surgical techniques were compared. The overall patient survival was also recorded.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XIV | Pages 48 - 48
1 Apr 2012
Dieckmann R Gebert C Streitbürger A Henrichs M Dirksen U Budny T Ahrens H Gosheger G Hardes J
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Aim. We present the greatest study of patients with proximal fibula resection. Moreover we describe a new classification system for tumour resection of the proximal fibula independent of the tumour dignity. Method. In 57 patients the functional and clinical outcome was evaluated. The follow up ranged between 6 months and 22.2 years (median 7.2 years). Indicationfor surgery was in 10 cases benign tumours and in 47 cases malignant tumours. In 32 patients a resection of the peroneal with resulting peroneal palsy was necessary. Results. In 13 of 45 patients, where a resection of the lateral ligament complex was done, knee instability occurred. Patients with peroneal resection had a significant worse functional outcome than patients without peroneal resection. An ankle foot orthosis was tolerated well by these patients. 3 of 4 patients with pathologic tibia fracture had a local radiation therapy. There was no higher risk of tibia fracture in patients with partial tibia resection. There was a significant better outcome of patients with benign than of patients with malignant tumours. Conclusion. Resection of tumours in the proximal fibula can cause knee instability, peroneal palsy and in case of local radiation therapy a higher risk of delayed wound healing and fracture. Despite the risks of proximal fibula resection good functional results can be achieved


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXX | Pages 32 - 32
1 Jul 2012
Cosker T White HB James A Athanisou N Whitwell D Gibbons M
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Introduction. Primary soft tissue sarcomas of the extremities are uncommon. Many such lesions will present to specialists in other clinics such as specialist Hand or Foot and Ankle clinics. Many are of a small size at presentation and may appear to be alternate, more common pathology. Methods. We collected data from all those patients with acral soft tissues sarcomas and referred to the Oxford Sarcoma Service, Nuffield Orthopaedic Centre, Oxford over a thirteen year period from 1997 – 2010. Data were collected regarding the primary suspected diagnosis, the final diagnosis, the referral route and whether patients had undergone previous inadvertent excision. Results. 38 patients were treated (24 foot, 14 hand tumours). The commonest histological subtype was synovial sarcoma in the foot group, and leiomyosarcoma in the hand group. 25 of the 38 patients had a lesion of less than 5cm at presentation. For those patients in whom a wide excision was performed 50% underwent complex plastic surgical reconstruction. Discussion. This study shows that 30% of patients with acral tumours required further surgery due to previous inadequate/inadvertent excision. 5cm as a benchmark for specialist referral should not be applied to lesions in the hand or feet and we recommend the use of the “marble rule” i.e. any lesion the size of a marble should be referred urgently and no patient should undergo surgery without imaging


The Bone & Joint Journal
Vol. 101-B, Issue 3 | Pages 272 - 280
1 Mar 2019
Verspoor FGM Mastboom MJL Hannink G van der Graaf WTA van de Sande MAJ Schreuder HWB

Aims

The aim of this study was to evaluate health-related quality of life (HRQoL) and joint function in tenosynovial giant cell tumour (TGCT) patients before and after surgical treatment.

Patients and Methods

This prospective cohort study run in two Dutch referral centres assessed patient-reported outcome measures (PROMs; 36-Item Short-Form Health Survey (SF-36), visual analogue scale (VAS) for pain, and Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC)) in 359 consecutive patients with localized- and diffuse-type TGCT of large joints. Patients with recurrent disease (n = 121) and a wait-and-see policy (n = 32) were excluded. Collected data were analyzed at specified time intervals preoperatively (baseline) and/or postoperatively up to five years.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXX | Pages 34 - 34
1 Jul 2012
Young P Bell S Jane M Mahendra A
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Introduction. Bony tumours of foot account for approximately 3% of osseous tumours. However, literature regarding calcaneal tumours comprises individual case reports, short case series or literature reviews, with last large case series in 1973. Literature on bony tumours of the talus is limited to case reports only. Methods. We retrospectively reviewed medical notes and imaging for all patients with calcaneal or talar tumours recorded in the Scottish Bone Tumour Registry since the 1940's. Demographics, presentation, investigation, histology, management and outcome were reviewed. Results. 38 calcaneal tumours and 24 talar tumours were identified. Calcaneal tumours. Mean age at presentation 30 with heel pain and swelling, average length of symptoms 9 months. 4 cases present with pathological fracture. 24 tumours benign including 6 unicameral bone cysts, 3 chondroblastoma, 3 PVNS with calcaneal erosion, and wide variety of individual lesions. 13 malignant tumours comprising 6 osteosarcoma, 5 chondrosarcoma and 2 Ewing's sarcoma. 1 metastatic carcinoma. Talar Tumours. Mean age at presentation 28 with ankle pain and occasionally swelling, average length of symptoms 5 months. 20 benign cases including 7 osteoid osteoma, 4 chondroblastoma, and variety of individual lesions. 3 malignant lesions comprising 2 chondrosarcoma, one osteosarcoma. Discussion. Tumours of hindfoot frequently are delayed in diagnosis due to rarity and lack of clinician familiarity. They are more common in men especially talar tumours which are most commonly benign osteoid osteoma or chondroblastoma. Calcaneal tumours have 1 in 3 risk of malignancy. Diagnosis is often made on plain radiograph but MRI is the gold standard. Conclusion. We present largest case series of calcaneal tumours and first case series of talar tumours from the Scottish Bone Tumour Registry. Despite their rarity clinicians should maintain a high index of suspicion as accurate and timely diagnosis is important to management and outcome