Aims

Solitary plasmacytoma of bone is a locally aggressive lesion seen by sarcoma teams. Some patients progress to myeloma. Local therapy decisions can be complex. This study reviews the experience of this condition in a single centre with a view to informing future treatment.

Introduction

Aseptic loosening is the most common mode of failure of massive endoprostheses. Introduction of Hydroxyapatite coated collars have reduced the incidence of aseptic loosening. However bone growth is not always seen on these collars.

Introduction

We aimed to determine the extent of osseous integration of the hydroxyapatite collars of tumour endoprostheses implanted in our unit.

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.

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.

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.

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.

Introduction: The ‘Two Week Wait’ (2ww) process has been in force since the year 2000, with the subsequent implementation of 32-day diagnosis and 62-day treatment ‘rules’ in 2005. The aims of this study were to compile a definitive diagnostic profile of 2ww referrals, establish whether a histological biopsy was required for diagnosis and consider the current 2ww impact on services in our centre.

Materials and Methods: Two hundred and nine patients were referred under 2ww to the North of England Bone and Soft Tissue Tumour service and prospectively recorded on a computerised multidisciplinary tumour database from 2006–8. The data was reviewed and verified using pathology, radiology reports and patient records.

Results: Malignancy was diagnosed in 41(20%) patients. This comprised 21 soft tissue sarcomas (10%), 11 primary bone tumours (5%), and 9 metastatic bone tumours (4%). 63 (30%) benign bone or soft tissue neoplasia and 80 (38%) non-neoplastic conditions were diagnosed. No mass lesion was identifiable in 25 patients (12%). A diagnostic or therapeutic biopsy was required in 108 (52%) patients.

Discussion and Conclusion: 15% of 2ww referrals to our centre have a primary bone or soft tissue sarcoma but over half of all 2ww patients require biopsy for diagnosis creating additional strains on resources under the 32- and 62-day rule. Emphasis is placed on obtaining a rapid diagnosis, to ease pressure on time to treatment, utilising a ‘one-stop clinic’ approach for biopsies of accessible tumours where applicable. The availability of timely radiological resources, facilitated by an MDT involving a designated coordinator (‘patient-tracker’), is key to ensure treatment is not delayed for any cancer patient regardless of referral route. Our centre is 100% compliant for waiting times for sarcoma according to the Department of Health 2008 data.

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.

Introduction: The ‘Two Week Wait’ (2ww) process has been in force since the year 2000, with the subsequent implementation of 32-day diagnosis and 62-day treatment ‘rules’, as part of reforms to NHS cancer services. The aims of this study were to compile a definitive diagnostic profile of 2ww referrals, establish whether a histological diagnosis was required and consider the current 2ww impact on services in our centre.

Methods: Two hundred and nine patients were referred to the North of England Bone and Soft Tissue Tumour service and prospectively recorded on a computerised multidisciplinary tumour database from 2006–8. The data was reviewed and verified using pathology, radiology reports and patient records.

Results: Malignancy was diagnosed in 41(20%) patients (n=209). This comprised 21 soft tissue sarcomas (10%), 11 primary bone tumours (5%), and 9 metastatic bone tumours (4%).

63 (30%) benign bone or soft tissue neoplasia and 80 (38%) non-neoplastic conditions were diagnosed. No mass lesion was identifiable in 25 patients (12%). A diagnostic or therapeutic biopsy was undertaken in 108 (52%) patients.

Discussion: Fifteen percent of 2ww referrals to our centre have a primary bone or soft tissue malignancy. The 2ww caseload has increased significantly in recent years and non-malignant conditions (80%) must still be diagnosed within the 31 day rule. We utilise a ‘one-stop clinic’ approach, with access to ultrasound guided biopsy, and a weekly multidisciplinary meeting to facilitate timely investigation and treatment of all patients regardless of referral route.

Introduction: The Literature is divisive in regards to the superiority of Core versus Incision biopsy in the diagnosis of Soft Tissue Tumour. The Aim of the study is to compare the accuracy of Trucut biopsy and open Incision Biopsy.

Methods: This was a retrospective review of case notes and pathology records. Between January 2006 and June 2007, 34 Trucut biopsies were performed without imaging guidance in an outpatient setting and 57 incision biopsies were performed as an inpatient on patients referred with a soft tissue mass to our service. In each case the accuracy of biopsy in providing a diagnostic sample, in determining the tumour type and the histological grade of tumour were calculated. For each biopsy method we compared the diagnosis after biopsy with the final diagnosis after excision. The proportion of diagnostic biopsies was calculated, as were the sensitivity and specificity of each technique in providing a diagnosis. Fisher’s exact test was used to test for differences in the techniques.

Results: In this series there were 41 soft tissue sarcomas, 8 metastatic adenocarcinoma soft tissue deposits, 7 lymphomas, 1 non soft tissue sarcoma, 32 benign soft tissue tumours and 1 infection. 33/34 Trucut biopsies and 55/57 open biopsies provided the final histological diagnosis (p=1). There was no statistical difference between the techniques in the accuracy of identifying the type and grade of soft tissue sarcoma

Discussion: Trucut biopsy is equivalent to incision biopsy in its accuracy of diagnosing soft tissue tumours. Biopsy in an outpatient setting for appropriate tumours is cost effective and likely shortens the time to diagnosis. Our results are comparable to published data from other centres.

Introduction: The Two Week Waiting Time (2wwt) 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 as “two week waiters” 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

This was compared with the total number of referrals to the unit and their tumour detection rates.

A total of 40 patients were referred as “two week waiters” in the given time period. They were seen on an average of 8 days following the referral. Of the 40 patients, four patients had soft tissue metastasis from a primary tumour elsewhere, and six had primary malignant soft tissue tumours. 12 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 515 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 2wwt standard 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.

Case Report: Metastatic deposits in the proximal femur commonly result in pathological fracture. Conventionally these fractures are treated surgically, by internal fixation or arthroplasty. The emphasis in treating these fractures is on restoring stability to the proximal femur and relieving pain. We present two cases in which pathological fractures of the proximal femur secondary to metastatic renal carcinoma were treated conservatively with excellent functional outcomes. In both cases, the medical condition of the patient precluded surgery. A 68 year old male with a subcapital fracture of the proximal femur was treated with bedrest and mobilisation. At 6 months he was able to mobilise with crutches, swim, and had returned to almost all normal activities despite non-union of the fracture.

A 63 year old male had a pathological fracture of the proximal femur treated by DCS fixation. The fracture failed to unite and the plate fractured. Despite this the patient was able to walk with crutches, pain free. Discussion: After a pathological fracture of the proximal femur conservative management can lead to satisfactory analgesia, function and therefore quality of life.

Introduction and aims: Despite advances in local therapy, there is an ongoing risk of local recurrence after treatment for soft tissue sarcoma. Early detection of local recurrence with MRI scanning may improve outcomes for patients. The purpose of this retrospective study was to evaluate the usefulness of routine postoperative MRI scans in diagnosing clinically occult local recurrence after surgery for trunk and extremity soft tissue sarcomas.

Material and Methods: We reviewed the clinical and radiology records of all patients who underwent surgery for trunk or extremity soft tissue sarcoma in our service with the potential for 3 years of follow up. We looked at the number of postoperative MRI scans performed, the indications for the scans (routine or clinical suspicion of recurrence) and the scan results.

Results: Between 1998 and 2003, 151 patients met the inclusion criteria. The mean age was 59 (17 – 94) years. The diagnosis was liposarcoma in 37%, malignant fibrous histiocytoma in 17%, and leiomyosarcoma in 15%. Reflecting differences in practice between consultants, 79 patients had routine postoperative MRI scans, 8 patients had MRI scans following clinical suspicion of a local recurrence, and 64 patients did not have a postoperative MRI scan. Of 79 patients undergoing a total of 354 routine postoperative scans, 2 had detection of a local recurrence not suspected clinically. This represents a cost of £55,224 per recurrence detected. Of the 8 patients who underwent MRI scanning for a clinical suspicion of local recurrence, 4 had a local recurrence confirmed on scanning.

Conclusions: Most local recurrences are detected clinically. The cost of detecting local recurrence of a trunk or extremity soft tissue sarcoma by MRI scanning is high. The benefit of earlier detection over clinical examination is not known.

Aims: Bone and soft tissue tumours not infrequently arise from the chest wall. Resection may require removal of ribs and reconstruction using mesh, biological materials such as lyophylised pig skin and muscle flaps. The purpose of this study was to review the experience of our multidisciplinary team in the management of chest wall resections for bone and soft tissue tumours. Patients and methods: This was a retrospective review of patient records. Between 2001 and 2005, 20 patients of mean age 50.3 years (13 to 92) underwent resections involving the chest wall. Ten were male.

Results: The diagnosis was chondrosarcoma in 8, osteosarcoma in 3, PNET/Ewings in 2, MPNST in 2, sarcoma NOS in 2, and one each of leiomyosarcoma, pleomorphic MFH, and metastatic renal carcinoma. 15 patients underwent rib resection, four sternal resections and one tumour of the clavicle was removed with the underlying rib. In 3 cases a latissimus dorsi flap was used as part of the chest wall reconstruction. The surgical margins were intralesional in 5, marginal in 11 and wide in 4 cases. Two patients died following a complication of treatment. Four patients died at a mean of 6 months (4 to 8 months) from metastatic disease. Two patients had local recurrence. At a mean follow up of 26 months (4 to 58) twelve patients were alive without evidence of disease, and two were alive with metastatic disease.

Conclusion: Chest wall resection for malignant bone or soft tissue tumours is feasible and can be achieved safely. However, there is a significant mortality rate associated with this procedure. This procedure demonstrates par excellence the value of multidisciplinary team working. Local anatomical constraints may mean that achieving a wide surgical margin is not always possible.

Aim: To describe referral pathways and assess delays in order to inform targeting of educational initiatives.

Methods: Anonymised data on all patients with non-gynaecological sarcoma over 2 years (1999–2000), was obtained from the Northern & Yorkshire Cancer Registry.

Results: 362 cases were registered (29 per million). Patients were referred to a maximum of three hospitals. Of 86 managed solely at the first hospital, 13 were treated at a specialist centre. 225 (59.8%) eventually reached a sarcoma specialist centre. Those referred for further treatment were younger compared to those managed at the initial hospital (median age 55–59 vs 65–69 years) p< 0.01, and were symptomatic for a shorter period (292 vs 419 days, NS). Average time between attendance at first and second hospital was 52 days (median 34, range 0–678 days) and between second and third hospitals was 77 days (median 35, range 0–414 days). Onward average referral time by specialty varied from 5 to 93 days.

Conclusion: Evidence suggests that sarcoma treatment is best undertaken by specialist multi-disciplinary teams. However, less than 60% of patients regionally access specialist management, and many experience considerably delay in the referral pathway. Patients referred on from the initial treating hospital tend to be younger and may have had shorter duration of symptoms. Further work is needed to quantify referral delays in primary care. Future guidelines may therefore be usefully targeted both at primary and secondary care.

Objective: To report a rare case of lymphomatous transformation in a Pagetic bone

Methods: A 61yr old lady with an 8yr history of monostotic Paget’s disease affecting her right proximal humerus presented with increasing right arm pain. Initial investigations including plain films, a radioisotope bone scan and MRI scan showed evidence of malignant transformation.

Results: The patient was admitted for an incision biopsy. Initial pathological examination suggested a high grade Paget’s sarcoma. However, further stains and immunohistochemical markers showed bone involvement by a malignant B-cell lymphoma.

Conclusion: Although sarcoma is by far the commonest malignant transformation of Pagetic bone, rare cases of lymphoma must also be considered, especially since the management and prognosis are radically different.

The soft tissue sarcomas (STS) are a diverse collection of malignant tumours of the connective tissues arising from the primitive mesoderm and ectoderm. While the primary treatment of most is surgery, chemotherapy can be offered to patients presenting with locally advanced or metastatic disease although sarcomas are resistant to the majority of anticancer drugs. The reasons for this are not fully understood but it is thought that p53 abnormalities and mdm2 overexpression may be involved. Samples from twenty eight adult patients with soft tissue sarcomas have been analysed for p53 mutations in exons 4 to 9 both by denaturing high performance liquid chromatography (dHPLC) and by direct automated sequencing. By sequencing we found mutations in 7/28 patients, giving a mutation rate of 25%. 4/6 were point mutations in exons 5, 7 and 8 and the remaining three were deletions in exons 4, 7 and 8. Six of these samples gave abnormalities in dHPLC analysis with a concordance rate of 97.5% between the sequencing and dHPLC data. Thirty nine and forty samples have been assessed by immunohistochemistry for p53 and mdm2 expression respectively. Do7 antibody which recognises the N terminus of p53 and F4-14 which recognises the carboxy-terminus of mdm2 were used. Immunohistochemistry was scored semiquantitatively by two independent observers and the results scored accordingly: low (< 20%), intermediate (20–80%) and high (> 80%). The initial results showed that 23/40 (58%) of patients were high staining for mdm2 in contrast to only 15/39 (38%) of patients for p53. All patients with deletions in p53 had intermediate staining for mdm2. 2/3 of these had intermediate staining for p53 and 1/3 had high staining for p53. One patient with a point mutation had high staining for both p53 and mdm2 but the other two have yet to be analysed by immunohistochemistry. These results confirm the overexpression of mdm2 in STS. Future experiments are planned using fluorescent in situ hydridisation (FISH) to determine whether MDM2 amplification is one of the mechanisms involved in mdm2 overexpression.

To assess the performance of calcium sulphate pellets as a bone graft substitute in an Orthopaedic Oncology practice using clinical and radiological outcomes.

Between 1998 and 2001, calcium sulphate pellets were used in cavitary defects in 38 procedures in 34 patients with bone tumours. In 29 calcium sulphate pellets were used alone, in 8 allograft and in 1 autograft bone was added. The diagnosis was unicameral bone cyst in 13, giant cell tumour in 11, non-ossifying fibroma in 2, chondroblastoma in 2, benign fibrous histiocytoma in 2 and another pathology in 8 procedures. The femur was involved in 12 procedures, the humerus in 8, the radius in 5, the tibia in 4, the fibula in 3, the calcaneus in 2, and one procedure each in the tarsal cuboid, a metatarsal, the talus, and the middle phalanx of a finger.

Median follow up was 14 months (3 to 48). Seven patients had wound complications. Pellets had absorbed completely in 26/28 (93%) evaluable procedures by 3 months. Healing of the defect occurred in 24/28 (86%) evaluable procedures by 6 months. In 6 cases, the healed defect contained cystic areas simulating local recurrence. In 3 cases, there was collapse of the defect.

In cavitary defects, calcium sulphate pellets reliably absorb. Some patients have wound complications, especially where the cavity is relatively superficial. The pellets do not provide mechanical stability where there is attenuated cortical bone. Cysts within the healed defect may simulate recurrence.