Introduction: The workload of a bone and soft tissue tumour (BSTT) multidisciplinary team (MDT) is varied. Only a proportion of the workload attracts specific funding from the National Commissioning Group (NCG) but many patients who do not have primary malignant bone tumours are also seen and treated. We analysed the workload of our supra-regional BSTT MDT to determine the variety of conditions seen, the proportion that does not attract specific funding and the expertise required to run the service. Methods: A prospective database was used to identify all new patients discussed at our weekly BSTT MDT meetings between 2004 and 2008 inclusively. Patients were divided by diagnosis into eight categories and further identified as to whether or not they attracted funding under NCG regulations. Results: 1743 new patients were identified of which 83 were excluded. Of the remaining 1660, 65% were non-sarcoma and 50% were benign. 31% of the malignant workload was non-sarcoma. Only 9% of treated patients were eligible for NCG funding. Of those requiring surgery, the orthopaedic team managed 93% of benign and 77% of malignant cases; general, plastic, or thoracic surgical teams managed the remainder. Discussion: NCG funds the management of all malignant primary bone tumours and the investigation and/or treatment of other selected conditions; the majority of our workload does not qualify. Despite fluctuations in the total workload, the ratio of benign to malignant cases remains relatively constant. An effective MDT requires expertise across many specialties

Objective: Appendicular soft tissue tumours are rare. They represent less than 1% of all malignancy. Failure to appropriately investigate a malignant mass can result in unnecessary loss of limb or life. New Zealand is a sparsely populated country and has four orthopaedic tumour surgeons which is in keeping with the recommended ratio of 1/1,000,000. Consequently some patients find themselves long distances from Tertiary tumour centres. We looked at the investigation and referrals of patients to the Bone and Soft tissue tumour service at our institution. We reviewed the charts of all patients (126) with a soft tissue tumour referred to the senior authors in 2006 and 2007. The information was stored in a prospective Tumour Registry Database. Where information was not available in records kept at our institution, the referring institution was contacted. There were 92 tertiary referrals and 34 GP referrals. The majority of the tertiary referrals came from orthopaedic surgeons (55), and general surgeons (28). The mean duration between referral and review was 16 days (0–215 days). There was a of 13 days wait for tertiary referral review. Tissue samples and histology reports accompanied 33 patients which had resulted from 35 invasive Pre Referral Procedures (PRP). This group suffered 21 complications affecting 18 patients. The remainder (91) were Pre Biopsy Referrals (PBR). Biospy was deemed necessary in 47 cases. The PBR group suffered 4 complications. Only one complication occurred to a patient with benign histology in each group. There was an extremely significant relationship between Pre Referral Procedures and suffering a complication (P< 0.0001). The relative risk of complication was 6.2 (C.I. 2.0–18.4) if an invasive procedure was performed prior to referral. There were 3 amputations (plus one recommended but refused by patient) in the PRP group and 1 amputation in the PBR group. This was not statistically significant. The median interval between referral and senior author review was 8 days for the PRP group and 10 days for the PBR group (P=0.2574). Sixty six percent of tertiary referrals were PBR (74% when including GP referrals). Biopsy of suspected appendicular Soft Tissue Sarcoma should be done by a tumour specialist or in prior consultation with one. There is virtually no delay to see an orthopaedic tumour specialist in New Zealand and achieving a tissue diagnosis does not expedite this

Introduction: In 2004 The Bone and Soft Tissue Tumour Service based at the Freeman Hospital gained recognition as a specialist sarcoma service. As a result, the service formed standards with auditable outcomes based on the NICE guidelines for specialist cancer services. This study aimed to explore whether the Bone and Soft Tissue Tumour Service meets the standards set. It also compares the results of the current study to the results of the last service evaluation carried out in 2005. Methods: Four key domains identified from the current standards were explored including; face-to-face contact with healthcare professionals, access to a specialist nurse, knowledge of support services and hospital stay. Design – retrospective questionnaire. Setting – Bone and Soft Tissue Tumour clinics, Musculoskeletal outpatients, Freeman Hospital. Subjects – 45 follow-up patients with a diagnosis of sarcoma. Results: Of the 45 patients who participated 100% were satisfied with face-to-face consultations with healthcare professionals. This included consultations regarding the diagnosis, investigations and results. 98% were ‘satisfied’ or ‘very satisfied’ with their hospital stay. In comparison to the previous study, access to the specialist nurse had improved by 22%. Although, not all patients currently access a specialist nurse. Patients’ knowledge of support services (sarcoma support group and helpline) has fallen since the previous survey. 80% (36/45) of patients had no complaints about the service. Discussion: The results of this study suggest a high rate of satisfaction with the service currently delivered. Of the key areas explored, face-to-face consultations and satisfaction with hospital stay scored highly. Improvement in patient access to a specialist nurse may subsequently improve knowledge of support services. This means patients are better emotionally supported thus, further improving overall quality of service delivered

Introduction: Seeding of bone or soft tissue tumour along the biopsy tract is a known complication of percutaneous biopsies. Correct surgical management requires preoperative Identification and excision of the biopsy tract at time of surgery. We aim to audit how well biopsy tract sites can be identified preoperatively and investigate factors influencing their Identification. Method: Prospective audit of patients who had tissue biopsies for bone and soft tissue tumours at the RNOH Stanmore and presented for surgery between February and April 2008. Case note analysis, patient history and examination at the time of surgery used to collect data. Results: 13/23 patients had their biopsy tract site accurately identified preoperatively, with a mean time gap of 43 days (6–118) between biopsy and excision. In 10/23 patients the biopsy site could not be accurately identified preoperatively. In these patients the mean time between biopsy and excision was 106 days (55–158) (p=< 0.05). 7 patients had neoadjuvant chemotherapy with a mean time gap of 110 days; in 5/7 the tract site was unidentifiable. One patient had preoperative radiotherapy and the biopsy site was unidentifiable. Discussion: This audit has shown that Identification of the biopsy site is more difficult after 40 days. In order to ensure accurate Identification of the biopsy site an Indian ink tattoo should be considered at time of biopsy. It may be particularly advisable for patients who are likely to require neoadjuvant chemotherapy or preoperative radiotherapy. On this basis we would recommend that all patients have the biopsy site marked at the time of biopsy and a further audit will be carried out to evaluate this change in practice

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

Limb-sparing surgery has become the preferred surgical treatment of malignant bone tumours of the knee. In patients with intra-articular extension of their tumour, extra-articular limb sparing surgery can prevent the knee from amputation. In a retrospective study between January 1985 and December 2007, we performed 34 extra-articular tumour resections of the knee-joint for a bone- or soft tissue tumour in the distal femur or proximal tibia with (suspect) intra-articular tumour extension into the knee on MRI. Contra-indications were extension of the tumour into the extensor mechanism and/or tumour involvement of the neurovascular bundle. Osteosarcoma (23/34) was the most common primary malignancy. Mean age was 36 years (17–70) and the mean follow up was 9 years (1–19). Patient survival rates at 5 years and 10 years are 78% and 58% respectively, mean patient survival was 47 months (8–211). In 12 (35%) patients, the primary implanted prosthesis failed during follow up. Prosthetic survival rates including minor revision surgery were 63% at 5 years and 36% at 10 years. Six (18%) patients had local recurrence of their malignancy, 5 of them in the popliteal fossa. Local recurrence was significantly correlated with marginal margins (P< 0.05). Fifteen patients had major complications (44%) mainly deep infection in proximal tibia resections and aseptic loosening in distal femur resections. Aseptic loosening was significantly correlated with non HA-coated stems (P< 0.05). Functional outcome scores according to MSTS (mean 81, (65–93)) and TESS (mean 85, (56–98)) of survivors are good. Our results suggest that extra-articular tumour resections of the knee-joint can provide a functional endoprosthesis and can be an alternative for primary amputation. However it is a technical demanding procedure with acceptable local recurrence and high complication rates in patients with, in general, poor survival

Objective: Modular tumour prostheses are often chosen for the reconstruction of osseous or joint defects following wide tumour resection in limb salvage procedures. In this retrospective trial we were looking for the clinical use in accordance to long-term-follow up especially on aseptic loosening of stem, wear of polyethylene, implant related complications and clinical and functional results. Methods: From 1996 to 2008 we performed in our clinic in 69 cases a modular distal femur replacement (MUTARS) after wide bone or soft tissue tumour resection. In our outpatient clinic we have assessed the clinical follow-up as clinical examination (Enneking-score) and standardized radiological follow-up for 5 years, then once per year. In the focus of interest were aseptic loosening of the stems, wear of polyethylene, and mechanical problems as implant failure. Results: In long-term-follow-up 6 polyethylene locks had to be changed into PEEK locks (8,6%9). PEEK-lock complications were not seen in this series. In 5 cases late infection of the prosthesis occured. In another 5 cases aseptic loosening of the prosthesis was diagnosed, fractures of the stems were not seen. We conclude that in tumour patients with major osseous reconstruction after wide resection a certain loss ob function cannot be avoided, but the rate of complications in the long-term-follow-up after implantation of modular tumour prosthesis is acceptable

Introduction: The workload of a bone and soft tissue tumour (BSTT) multidisciplinary team (MDT) is varied. Only a proportion of the workload attracts specific funding from the National Commissioning Group (NCG) but many patients who do not have primary malignant bone tumours are also seen and treated. We analysed the workload of our supra-regional BSTT MDT to determine the variety of conditions seen, the proportion that does not attract specific funding and the expertise required to run the service. Methods: A prospective database was used to identify all new patients discussed at our weekly BSTT MDT meetings between 2004 and 2008 inclusively. Patients were divided by diagnosis into eight categories and further identified as to whether or not they attracted funding under NCG regulations. Results: 1743 new patients were identified of which 83 were excluded. Of the remaining 1660, 65% were non-sarcoma and 50% were benign. 31% of the malignant workload was non-sarcoma. Only 11% of patients were eligible for NCG funding. Of those requiring surgery, the orthopaedic team managed 93% of benign and 77% of malignant cases; general, plastic, or thoracic surgical teams managed the remainder. Discussion: NCG funds the management of all malignant primary bone tumours and the investigation and/or treatment of other selected conditions; the majority of our workload does not qualify. Despite fluctuations in the total workload, the ratio of benign to malignant cases remains relatively constant. Considerable expertise across many different specialties is essential for an effective and efficient MDT

Aims

The aim of this study was to report the long-term prognosis of patients with multiple Langerhans cell histiocytosis (LCH) involving the spine, and to analyze the risk factors for progression-free survival (PFS).

Introduction: Well-differentiated liposarcomas have a tendency to recur locally but do not metastasise unless dedifferentiation occurs. In this study, a tumour superficial to the deep fascia of the trunk or limb is termed an atypical lipoma (AL) and one deep to fascia, a lipoma-like liposarcoma (LL) reflecting increased difficulty in wide local excision.

Methods: We prospectively collected data for 87 well-differentiated liposarcomas excised at our institution from 1998–2008. Data was recorded on a multidisciplinary team database and verification was undertaken using patient records. Any radiological investigation performed was determined retrospectively. Primary excisions performed elsewhere were excluded. The aim was to produce recommendations on the clinical and radiographical post-operative management of these common tumours.

Results: LL was seen in 74 patients and AL in 13 (mean age 58 years, mean follow up 5 years). The mean size of LL excisional biopsy was 148mm and 54mm for AL (p< 0.05). There were no AL recurrences. Five LL (7%) locally recurred within a mean of 5 years (range 2–10 years). All were deeply related to neurovascular structures (4 thigh-marginal/complete excisions and 1 upper arm-piecemeal excision). One recurrence was detected by MRI from 26 LL patients (35%), the other four being clinically suspected prior to re-scanning. During follow up, a chest radiograph was performed in 21 LL patients (27%) and no metastases were detected.

Discussion: Patients with a completely excised superficial AL need no routine follow up. Follow up of LL is determined by the patient, the tumour size and the location. The routine use of interval MRI to detect local recurrence of uncomplicated LL is not necessary. MRI provides ‘base-line’ post-operative information where a neurovascular bundle was closely related to the tumour or excision was incomplete. Chest radiographs are not indicated in screening for metastases in these tumours unless locally recurrent.

Primary malignant bone and soft tissue tumours often occur in the lower extremities of active individuals including children, teenagers and young adults. Survivors routinely face long-term physical disability. Participation in sports is particularly important for active young people but the impact of sarcoma treatment is not widely recognised and clinicians may be unable to provide objective advice about returning to sports. We aimed to identify and summarise the current evidence for involvement in sports following treatment of lower limb primary malignant bone and soft tissue tumours. A comprehensive search strategy was used to identify relevant studies combining the main concepts of interest: (1) Bone/Soft Tissue Tumour, (2) Lower Limb, (3) Surgical Interventions and (4) Sports. Studies were selected according to eligibility criteria with the consensus of three authors. Customised data extraction and quality assessment tools were used. 22 studies were selected, published between 1985 – 2020, and comprising 1005 patients. Fifteen studies with data on return to sports including 705 participants of which 412 (58.4%) returned to some form of sport at a mean follow-up period of 7.6 years. Four studies directly compared limb sparing and amputation; none of these were able to identify a difference in sports participation or ability. Return to sports is important for patients treated for musculoskeletal tumours, however, there is insufficient published research to provide good information and support for patients. Future prospective studies are needed to collect better pre and post-treatment data at multiple time intervals and validated clinical and patient sports participation outcomes such as type of sports participation, level and frequency and a validated sports specific outcome score, such as UCLA assessment. In particular, more comparison between limb sparing and amputation would be welcome

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

Diffuse-type Tenosynovial Giant-Cell Tumour (d-TGCT) of large joints is a rare, locally aggressive, soft tissue tumour affecting predominantly the knee. Previously classified as Pigmented Villonodular Synovitis (PVNS), this monoarticular disease arises from the synovial lining and is more common in younger adults. Given the diffuse and aggressive nature of this tumour, local control is often difficult and recurrence rates are high. Current literature is comprised primarily of small, and a few larger but heterogeneous, observational studies. Both arthroscopic and open synovectomy techniques, or combinations thereof, have been described for the treatment of d-TGCT of the knee. There is, however, no consensus on the best approach to minimize recurrence of d-TGCT of the knee. Some limited evidence would suggest that a staged, open anterior and posterior synovectomy might be of benefit in reducing recurrence. To our knowledge, no case series has specifically looked at the recurrence rate of d-TGCT of the knee following a staged, open, posterior and anterior approach. We hypothesized that this approach may provide better recurrence rates as suggested by larger more heterogeneous series. A retrospective review of the local pathology database was performed to identify all cases of d-TGCT or PVNS of the knee treated surgically at our institution over the past 15 years. All cases were treated by a single fellowship-trained orthopaedic oncology surgeon, using a consistent, staged, open, posterior and anterior approach for synovectomy. All cases were confirmed by histopathology and followed-up with regular repeat MRI to monitor for recurrence. Medical records of these patients were reviewed to extract demographic information, as well as outcomes data, specifically recurrence rate and complications. Any adjuvant treatments or subsequent surgical interventions were noted. Twenty-three patients with a minimum follow-up of two years were identified. Mean age was 36.3 at the time of treatment. There were 10 females and 13 males. Mean follow-up was seven and a half years. Fourteen of 23 (60.9%) had no previous treatment. Five of 23 had a previous arthroscopic synovectomy, one of 23 had a previous combined anterior arthroscopic and posterior open synovectomy, and three of 23 had a previous open synovectomy. Mean time between stages was 87 days (2.9 months). Seven of 23 (30.4%) patients had a recurrence. Of these, three of seven (42.9%) were treated with Imatinib, and four of seven (57.1%) were treated with repeat surgery (three of four arthroscopic and one of four open). Recurrence rates of d-TGCT in the literature vary widely but tend to be high. In our retrospective study, a staged, open, anterior and posterior synovectomy provides recurrence rates that are lower than rates previously reported in the literature. These findings support prior data suggesting this approach may result in better rates of recurrence for this highly recurrent difficult to treat tumour

Diffuse-type Tenosynovial Giant-Cell Tumour (d-TGCT) of large joints is a rare, locally aggressive, soft tissue tumour affecting predominantly the knee. Previously classified as Pigmented Villonodular Synovitis (PVNS), this monoarticular disease arises from the synovial lining and is more common in younger adults. Given the diffuse and aggressive nature of this tumour, local control is often difficult and recurrence rates are high. Current literature is comprised primarily of small, and a few larger but heterogeneous, observational studies. Both arthroscopic and open synovectomy techniques, or combinations thereof, have been described for the treatment of d-TGCT of the knee. There is, however, no consensus on the best approach to minimize recurrence of d-TGCT of the knee. Some limited evidence would suggest that a staged, open anterior and posterior synovectomy might be of benefit in reducing recurrence. To our knowledge, no case series has specifically looked at the recurrence rate of d-TGCT of the knee following a staged, open, posterior and anterior approach. We hypothesized that this approach may provide better recurrence rates as suggested by larger more heterogeneous series. A retrospective review of the local pathology database was performed to identify all cases of d-TGCT or PVNS of the knee treated surgically at our institution over the past 15 years. All cases were treated by a single fellowship-trained orthopaedic oncology surgeon, using a consistent, staged, open, posterior and anterior approach for synovectomy. All cases were confirmed by histopathology and followed-up with regular repeat MRI to monitor for recurrence. Medical records of these patients were reviewed to extract demographic information, as well as outcomes data, specifically recurrence rate and complications. Any adjuvant treatments or subsequent surgical interventions were noted. Twenty-three patients with a minimum follow-up of two years were identified. Mean age was 36.3 at the time of treatment. There were 10 females and 13 males. Mean follow-up was seven and a half years. Fourteen of 23 (60.9%) had no previous treatment. Five of 23 had a previous arthroscopic synovectomy, one of 23 had a previous combined anterior arthroscopic and posterior open synovectomy, and three of 23 had a previous open synovectomy. Mean time between stages was 87 days (2.9 months). Seven of 23 (30.4%) patients had a recurrence. Of these, three of seven (42.9%) were treated with Imatinib, and four of seven (57.1%) were treated with repeat surgery (three of four arthroscopic and one of four open). Recurrence rates of d-TGCT in the literature vary widely but tend to be high. In our retrospective study, a staged, open, anterior and posterior synovectomy provides recurrence rates that are lower than rates previously reported in the literature. These findings support prior data suggesting this approach may result in better rates of recurrence for this highly recurrent difficult to treat tumour

In this paper, a retrospective review was undertaken of a large musculoskeletal tumour database to identify patients who presented with tumours of the foot and ankle. Soft tissue tumours occurred more frequently than bone tumours, and were also more frequently malignant than bone tumours. In contrast to the more recent trend towards limb-preserving surgery in other anatomic areas, malignant tumours of the foot and ankle were frequently unresectable and were treated with amputation. Although the majority of extremity tumours that present to the orthopaedic surgeon are found in the proximal limbs or around the knee, tumours of the ankle and foot are also relatively common. The purpose of this study is to identify the frequency with which benign and malignant bone and soft tissue tumours occur in the foot and ankle and the oncologic and surgical outcomes of these patients. A retrospective review of a large musculoskeletal tumor database in a tertiary referral center from the years 1986–2002 was undertaken. For oncologic outcomes, a minimum two-year follow up was considered. A total of one hundred and sixteen bone and one hundred and seventy-one soft tissue tumours were identified. Seventy-seven bone tumours were benign and thirty-nine were malignant. Sixty-six soft tissue tumours were benign and one hundred and five were malignant. The most common benign bone tumour was giant cell tumour and osteosarcoma was the most common malignancy. Malignant fibrous histiocytoma was common in the distal leg but synovial sarcoma and clear cell sarcoma were more common in the foot. Twenty patients with bone malignancies (51%) and twenty-four with soft tissue sarcomas (23%) had amputation as definitive surgical management. Death from metastases occurred in 25% of patients with bone malignancies and 10% of soft tissue sarcomas. At this center, the majority of bone tumours treated are benign but the majority of soft tissue tumours are malignant. Limb salvage is often not possible and amputation for local tumour control is necessary far more often than in other anatomic sites

Introduction. The Two Week Waiting Time Standard, which requires that patients with suspected cancer referred by general practitioners should be seen within 2 weeks, was introduced in 2000. We reviewed the performance of this standard with regards to proportion of patients seen and tumour detection rates. Methods and results. We reviewed all the referrals sent under the ‘two week’ rule from January 2004 to December 2005, to our bone and soft tissue sarcoma service. These referrals were evaluated for:. Whether or not the referral met established referral guidelines for bone and soft tissue tumours. The proportion of patients seen within two weeks. The proportion of patients referred under the guidelines that had malignant tumours. This was compared with the total number of referrals to the unit and their tumour detection rates. A total of 40 patients were referred under the ‘two week’ rule. 95% of these were seen within two weeks of referral. Of the 40 patients, three patients had soft tissue metastasis from a primary tumour elsewhere, and six had primary malignant soft tissue tumours. 13 had a benign bone/ soft tissue tumour. 18 (45%) patients had a non neoplastic pathology (6 Muscle tear/ herniation; 4 ganglion/bursa; 2 lumps that disappeared) During the same period a total of 507 patients were referred by other routes. Conclusion. Only 10 of 40 patients referred under the 2-week rule had malignant tumours. The majority of referrals to our service do not fall under this rule. Significant numbers of referral under the rule are not in line with the referral guidelines. It is our impression that the 2-week rule, whilst highlighting the need of these patients to be seen urgently, may distort clinical priorities and disadvantage patients referred from other sources

Granular Cell Tumours are rare mesenchymal soft tissue tumours that arise throughout the body and are believed to be of neural origin. They often present as an asymptomatic slow-growing benign solitary lesion but may be multifocal. One to two percent of cases are malignant and can metastasise. Described series in the literature are sparse. We examined our database and identified eleven cases in ten patients treated surgically and followed-up for a period of over six years (May 2002 to January 2009) in our regional bone and soft tissue tumour centre. Five tumours were located in the lower limb, four in the upper limb and two in the axial skeleton. Mean patient age was 31.2 years (range 8 to 55 years). Excision was complete in one case, marginal in five cases and intra-lesional in five cases. No specimens showed evidence of malignancy. No patients required postoperative adjuvant treatment. Mean follow-up was 19.3 months (range 1 to 37 months), with no cases of local recurrence. One case was multi-focal. Histopathological examination revealed the classical features of granular cell tumour in all cases. Typically, tumour cells were diffusely and strongly positive for S100 protein by immunohistochemistry, whereas the other markers tested were negative. We believe this case series to be the largest of its type in patients presenting to an orthopaedic soft tissue tumour unit. We present our findings and correlate it with findings of other series in the literature

Aims. Malignant tumours of the foot and ankle are rare, but easily missed. NICE guidelines for bone and soft tissue tumours may be less appropriate for the foot and ankle than elsewhere. The purpose of this study was to identify the clinical features and treatment of malignant tumours arising in the foot and ankle to see if guidelines should be modified. Patient and Methods. This was a retrospective review of patients presenting to the Bone and Soft Tissue Tumour Service with a suspected tumour of the foot or ankle. Between March 1998 and July 2009, 132 patients were identified from a prospectively collected database of patients reviewed at a weekly multidisciplinary meeting. Results. Of 132 patients, 43 had benign tumours, 26 malignant tumours and 65 tumour like conditions (eg. ganglions, epidermal cysts, osteophytosis). In the malignant tumour group, the median duration of symptoms prior to presentation was 24 months, with a painful, small but enlarging mass being the most common clinical presentation. In 4 of the 26 cases (12%) unplanned excision had been undertaken prior to referral. Of the 26 malignant tumours, 4 were primary bone tumours (1 Ewing's sarcoma, 1 osteosarcoma and 2 chondrosarcomas) and 22 were soft tissue tumours of which 9 (41%) were synovial sarcomas. In 15 of 26 (58%) of cases the malignant tumour was high grade. In 10 of 26 (39%), amputation was required in order to achieve curative margins and 7 (25%) cases required soft tissue reconstructive surgery following tumour resection. Conclusions. The majority of malignant tumours in the foot and ankle are soft tissue in origin and high grade. Their clinical presentation can make early detection challenging and a high index of suspicion is required. In this review most malignant tumours presented as longstanding, small but enlarging, painful masses. Specific guidelines for investigation and referral may be warranted in addition to the current NICE recommendations