Objective

Sarcomas are a rare group of tumours, which pose numerous problems regarding correct diagnosis and appropriate management. This study aimed to examine whether symptom duration and tumour size at diagnosis have changed over time, using size and symptom duration as methods of comparison.

We performed a retrospective review of 98 patients with malignant tumours of the periacetabular region, treated by resection and endoprosthetic reconstruction between 1971 and 2005 at the Royal National Orthopaedic Hospital, Stanmore and The Royal Orthopaedic Hospital, Birmingham.

The mean age of the patients was 43.6 years (10 to 76). 53 patients (54%) were male. The age distribution demonstrated peaks in the 2nd and 6th decades. The mean follow up was 65 months (2 to 405. The overall 10-year survival rate was 56% (determined by the Kaplan-Meier method).

54 patients (58.1%) had one or more complications. Infection was the most common (30%) with the majority occurring in the first 2 years. Dislocation occurred in 19 (20%) of our patients all in the first year following surgery. All were recurrent with a mean of 2.9 (2 to 6) episodes. Operations performed before 1994 were associated with a dislocation rate of 40.5% and after 1996 the rate was only 3.9% (p < 0.001).

Function was assessed according the Toronto Extremity Salvage Sore (TESS). The overall TESS was 59.4. In patients that had a major complication the TESS was 37.1 but in the group that did not the TESS was 70.3 (p < 0.001).

Men experienced higher rates of death, infection and revision than women (p < 0.05). The local recurrence rate was 31% with high grade tumours associated with higher recurrence rates (p < 0.05). Tumour resection in the ilium is associated with higher rates of infection than tumours located in the periacetabular region alone (p < 0.05).

This method of reconstruction is still associated with high complication rates. Function is good if a major complication can be avoided. The lower dislocation rate more recently is probably the result of improved surgical technique and the use of larger femoral heads.

We have investigated whether improvements in design have altered the outcome for patients undergoing endoprosthetic replacement of the distal femur following tumour resection.

Survival of the implant and ‘servicing’ procedures have been documented using a prospective database and review of the implant design records and case records. A total of 335 patients underwent a distal femoral replacement with 162 having a fixed hinge design and 173 a rotating hinge with most of the latter group having a hydroxyapatite collar at the bone prosthesis junction. The median age of the patients was 24 years (range 13-82 yrs). With a minimum follow up of 5 years and a maximum of 30 years, 192 patients remain alive with a median follow-up of 11 years. The risk of revision for any reason was 17% at 5 years, 34% at 10 years and 58% at 20 years. One in ten patients developed an infection and 42% of these patients eventually required an amputation. Aseptic loosening was the most common reason for revision in the fixed hinge knees whilst infection and stem fracture were the most common reason in the rotating hinges. The risk of revision for aseptic loosening in the fixed hinges was 32% at ten years compared with nil for the rotating hinge knees with a hydroxyapatite collar. The overall risk of revision for any reason was halved by use of the rotating hinge, and for patients older than 40 years at time of implant.

EPRs are the treatment of choice following resection of tumours. These have been used for 39 years in our institution. There has been concern regarding the long term survival of endoprosthesis; this study investigates the fate of the reconstruction.

Current guidelines suggest that most lumps less than 5cm in size will not be malignant. We reviewed our experiences of small lumps at the Royal Orthopaedic Hospital, Birmingham, in order to try and identify factors associated with malignancy. All referrals to our clinic were included and data on size, symptoms, depth and diagnosis were analysed. We identified 325 patients, with 57 (17.5%) malignant and 268 (82.5%) benign lumps. Size >3cm was the most accurate predictor of malignancy (35.8% malignant vs 9.9% <3cm). 23.6% of lumps reported as increasing size were found to be malignant, compared to 8.6% of those that were not. Pain was found to be a poor predictor of malignancy, with only 15.5% of painful lumps found to be malignant and 18.9% of painless lumps found to be malignant. ‘Size >3cm’ has a sensitivity of 73.6% and a specificity of 56.3%, ‘Depth’ has a sensitivity of 73.6% and a specificity of 30.2%, ‘Increasing in Size’ a sensitivity of 71.4% and a specificity of 56.7% and ‘Pain’ a sensitivity of 33.3% and a specificity of 57.1%. If a lump has none of the above features then one can be 89.5% certain that it is not malignant. If it has 1 feature there is a 16.1% chance of its becoming malignant, 2 features a 58% chance, 3 features an 87% chance and if a lump has all 4 features there is a 93.5% chance of its being found to be malignant.

The main limitation with this study is that the data set is skewed because all lumps referred to the Oncology Department are by their nature suspicious. This study identifies those factors which suggest malignancy in small lumps and provides the basis for carrying out a large, community-based study on all lumps, to identify features associated with malignancy.

Chondroblastomas arise in the epiphyseal area of bones. In the femoral head this can cause considerable difficulty in obtaining access as the epiphysis is entirely intra-articular.

We have reviewed management and outcome of 10 patients with chondroblastoma of the femoral head to identify outcome and complications. The mean age was 14 years and all presented with pain (frequently in the knee) and a limp. All were diagnosed on plain Xray and MRI. Five younger children were treated by curettage by a lateral approach up the femoral neck (to try and minimise damage to the epiphysis) and five by a direct approach through the joint.

Two of the five patients with a lateral approach developed local recurrence whilst none of the direct approaches did. Both local recurrences were cured with a direct curettage. One patient developed overlengthening of the leg by 1cm but there was no case of growth arrest or osteoarthritis.

We recommend a direct approach to the lesion whenever possible to give the best chance of cure with a low risk of complications.

Introduction

Despite the advances in adjuvant chemotherapy and surgical techniques, the diagnosis of a bone tumour still carries with it a significant risk of mortality. This study investigates factors affecting survival, in patients treated for malignant tumours of bone using Endoprosthetic replacement (EPR).

Propionibacteria are organisms of low virulence, although they do cause deep periprosthetic infections. The aim of this study was to show that Propionibacteria do not always cause a significant rise in ESR and CRP.

Between May 2001 and May 2004, we identified 77 patients with prosthetic joint replacements colonised with Propionibacteria, 47 males and 30 females. There were 47 hip joint replacements, 27 knee joint replacements, 2 endoprosthetic replacements of the femur and 1 shoulder joint replacement. We retrieved successfully the medical records of 66 patients in order to identify the number of patients treated for an infected prosthetic joint arthroplasty. The pre-operative values of ESR and CRP were recorded. For the purposes of this study, an ESR rate of 30mm/hr or higher and a CRP level of 10mg/lt or higher were considered to be suggestive of infection and were deemed a positive result.

All of the 77 patients had both ESR and CRP measured pre-operatively. In only 16 (21%) both ESR and CRP were higher than 30mm/hr and 10mg/l respectively. In 33 patients (43%) with prosthetic joint replacements colonised with Propionibacteria, the pre-operative values of ESR and CRP were normal. 23 patients were treated for an infected prosthetic joint arthroplasty. In 7 (30%) of those patients both ESR and CRP were normal.

This suggests that normal pre-operative values of ESR and CRP in suspected failed prosthetic joint replacements might not exclude infection, if the causative organism is of low virulence such as Propionibacteria.

Soft tissue sarcomas (STS) arising in the adductor compartment of the thigh are frequently large before clinical detection, posing particular challenges with surgical resection and associated with a high risk of wound complications. This study compares oncological and functional outcomes and complications following treatment of adductor compartment soft tissue sarcomas from three international centres with different treatment philosophies.

184 patients with new primary, non-metastatic, deep STS in the adductor compartment diagnosed between 1990 and 2001 were identified from the centres' local databases. The Toronto Extremity Salvage Score (TESS) was used to assess function in patients.

There were 94 male and 90 female patients, with ages ranging from 13 to 88 years (median age 57 years). The period of follow-up ranged from 1 to 162 months. The overall survival was 65% at 5 years and related to grade and size of the tumour. There was no difference in overall survival or systemic relapse between the three centres. There was however a significant difference in local control (28% LR in centre 1 compared to 10% in centre 2 and 5% in centre 3, which appeared to be principally related to the use of radiotherapy and surgical margins.)

66 patients (36%) from the three centres developed wound complications post-operatively and it was shown to be associated with high grade and large tumours (>10cm).

Functional scores averaged 78% but were significantly worse for patients with wound complications or high-grade tumours; however, they were not affected by timing of radiotherapy or use of prophylactic free muscle flaps.

Fifty-six patients with stage II-B osteosarcoma around the knee were followed-up for a minimum of 92 months. The percentage of tumour cells expressing VEGF/MMP-9 was assessed using immunohistochemistry. The relationship between VEGF/MMP-9 expression and survival was assessed using Kaplan-Meier and Cox regression models. Patients with tumours expressing VEGF in >25% of their cells had shorter overall (p=0.019) and disease-free survival (p=0.009). Patients with tumours expressing MMP-9 had shorter overall (p=0.0042) and disease-free survival (p=0.0004). There was an association between VEGF and MMP-9 expression (p=0.021). The negative effects of VEGF/MMP-9 expression on survival were independent of traditional prognostic factors.

Aim

To identify patient, tumour or treatment factors that influence outcome in patients with radiation induced sarcoma of bone.

Dedifferentiated chondrosarcoma is a rare but highly malignant manifestation that can occasionally arise in patients with cartilage tumours. There remains uncertainty as to the best treatment for this condition and in particular whether chemotherapy may have a role in improving prognosis.

Members of EMSOS were invited to contribute data on patients, tumours, treatment and outcomes of patients with dedifferentiated chondrosarcoma.

Eight centres contributed data on 317 patients from 7 countries. The mean age was 59 (range 15 to 89) and the most common site was the femur (46%) followed by the pelvis (28%). 25% of patients presented with a pathological fracture and the most common high grade component identified was MFH. 23% had metastases at diagnosis and these patients had a median survival of 5 months. 30% of patients received chemotherapy, with 47% under 60 having chemotherapy compared with 10% over 60. One third of this group had neoadjuvant chemotherapy and the rest had adjuvant reatment. 88% had surgery with limb salvage in 80% of this group. The overall survival was 38% at 2 years and 24% at 5 years but in patients without metastases at diagnosis these figures were 44% and 28% respectively. Poor prognostic factors for survival were: metastases at diagnosis, amputation or no operation, local recurrence, age over 60 and pathological fracture at presentation. We were unable to identify any group in whom chemotherapy appeared to have a survival benefit.

Dedifferentiated chondrosarcoma carries a dismal prognosis. Although 30% of patients received chemotherapy in this study we were not able to prove that it improved survival. Early diagnosis and complete surgical excision still offer the best prognosis for this condition.

Primary malignant bone tumours frequently arise in children close to the knee, hip or shoulder. Resection of the tumour will often require excision of the epiphysis and frequently one side of the involved joint. In these children an extendable endoprosthesis is usually required to allow for maintenance of limb length equality.

We have used 180 extendable endoprostheses in 176 children since 1975. The indication for use of an extendable prosthesis was if there was more than 30mm of growth remaining in the resected bone. The age of the patients ranged from 2 to 15 and 99 were boys. The sites of the endoprostheses used were: distal femur in 91, proximal tibia in 42, proximal femur in 11, total femur in 6 and proximal or total humerus in 26. 131 of the operations were for osteosarcoma and 34 for Ewing's.

Five types of lengthening mechanism have been used. Two designs used a worm screw gear, one type used a C collar, one type a ball bearing mechanism and the latest uses a non invasive lengthening system whereby a motor inside the prosthesis is activated by an electromagnetic field.

Of the 176 patients, 59 have died and of the remainder, 89 have reached skeletal maturity. 19 patients had an amputation, 11 due to local recurrence and 8 due to infection. The risk of infection was 19% in surviving patients. Most of the skeletally mature had equal leg lengths. The average number of operations was 11 but ranged between 2 and 29. Most operations were for lengthening but younger children always needed revisions of the prosthesis. Functional scores were 77%.

Extendable endoprostheses are demanding both for the patient and the surgeon. The high complication rate should be decreased by non invasive lengthening prostheses.

All patients referred to our unit with previously untreated metastatic renal cancer were included in this review. We investigated likely prognostic factors including age, sex, site, synchronous or metachronous metastasis, stage of the disease and the type of treatment received.

From 1976 until 2004, a total of 198 patients were treated by our unit for renal metastases. 15 patients were excluded because they were referred after failure of previous treatment or only had advice. 96 patients were already known to have renal metastasis with their diagnosis having been made between 0.2 and 17 years from the diagnosis of primary cancer (mean 4 years). 33 patients presented to us with a pathological fracture and were found to have renal cancer. A total of 54 patients had multiple metastases and 129 had a solitary metastasis. The cumulative survival from the time of diagnosis of the bone metastasis is 70 percent at 1 year, 40% at 3 years and 18% at 5 years. In patients with a solitary metastasis, the overall survival was 74% at 1 year and 45% at 3 years, whereas in patients with multiple metastases it was 55% at 1 year and 22% at 3 years. (p=0.02) In patients with a solitary metastasis treated by excision of the metastasis, the survival at 1 year was 86% as compared to 38% for those that were treated with just a local procedure. Cox multivariate analysis shows that survival was better in those with solitary metachronus metastasis who underwent a radical procedure.

Introduction: Soft tissue tumours are common in the foot however few are malignant. Synovial Sarcoma of the foot is the most common soft tissue sarcoma to present in the foot (~50%). Despite this diagnosis is often delayed and treatment may be difficult. The aim of this paper is to review the presenting features, management and outcome of synovial sarcoma of the foot and to try and identify areas for improvement.

Method: The clinical presentation, radiological features and treatment of 33 patients with histologically proven synovial sarcoma of the foot were reviewed along with outcomes in terms of local control, metastases and survival.

Results: 33 patients with synovial sarcoma were treated at out unit over a 25 year period, 22 in the last 10 years. The average duration of symptoms was 125 weeks. The age range at presentation was between 11 to 80 years (mean 44). The mean size of the tumour at diagnosis was 5cm in diameter (range 1–10cm). 75% of the tumours were deep at the time of diagnosis. Treatment was by amputation in 21 patients and limb salvage in 11 with 1 patient receiving palliative chemotherapy. Only one patient had local recurrence and presented with lung metastases. Four patients had metastases at diagnosis and nine developed them subsequently. Overall survival at 10 years was 53%.

Discussion: These results have shown that late diagnosis is common for synovial sarcoma of the foot and that by the time of diagnosis 75 % have invaded extra-compartmentally leading to a high risk for amputation. 20 patients had an inadvertent excision of the tumour before referral to our unit. Local control was best achieved with amputation. Overall survival was surprisingly poor despite successful local control.

Conclusion: Earlier aggressive investigation of patients with foot swelling or pain may influence their long term prognosis.

Background: There is currently no standard follow up protocol for patients who have been diagnosed with and treated for high-grade osteosarcoma. We therefore investigated the possibility of creating a risk based follow-up protocol for patients with primary osteosarcomas.

Methods: 313 patients diagnosed with primary osteosar-comas were studied. The identified risk factors for local recurrence included poor necrosis, inadequate margins and high risk tumour site in the bone. The risk factors for metastases were poor necrosis, inadequate margins, extracompartmental stage and tumour size ≥5cm.

Results: The risk of local recurrence and/or metastases within three years of diagnosis increases as the number of risk factors increase. Patients were grouped according to their number of risk factors. The cumulative risk of metastases for patients with 0, 1, 2, 3 and 4 risk factors is 0%, 12%, 21%, 54% and 60% respectively (p=< 0.0001). Risk of local recurrence for patients with 0, 1, 2 and 3 risk factors is 5%, 14%, 25% and 20% respectively (p=0.0025).

Conclusion: Our investigation shows that by grouping patients together according to their number of identified risk factors, it is possible to identify groups of patients that are most at risk. This information can be used to design an evidence based follow up protocol which would have important implications for clinical practice.

Background: Malignant tumours of the radius compose only 3% of all upper limb tumours. Owing to their rarity they are often difficult to manage satisfactorily. Of the options for fixation available, endo-prosthetic replacements have been scarcely utilized despite their success in limb preservation with malignant tumours in other parts of the body. At our centre we have used these when biological solutions (eg fibula graft) were not indicated due to extensive disease or the need for radiotherapy.

Patients: We performed four endoprosthetic replacements of the distal radius in three males and one female with ages ranging from 19–66 years (average= 42.25 years of age). Two were performed for varieties of osteosarcoma (parosteal and osteoblastic osteosarcomas), one for a large destructive giant cell tumour (GCT) and one for destructive renal metastases. Three were right sided (75%) and one left sided (25%).

Methods: Medical records were evaluated for information on local recurrence, metastases, complications and functional outcome using the Toronto Extremity Salvage Score (TESS).

Results: Follow up ranged from 22 to 205 months (average= 116.5 months). The average TESS score was 58.1% (range= 44.6–74.5%). Neither case of osteosarcoma recurred. The GCT recurred twice and the patient with renal metastases had nodules removed from his affected wrist on two further occasions. There were no cases of infection, but one of the earlier cases had problems with metacarpal stems cutting out and joint subluxation. The two earlier cases have since died at 205 (parosteal osteosarcoma) and 189 months (GCT) respectively of other disease.

Conclusions: We conclude that although this is a very small series of endoprosthetic replacement of the distal radius, the technique is a useful addition to the surgical options, with acceptable post-operative functional results and complication rates when a biological solution or preservation of the wrist joint is not indicated.

Background & Methods: We reviewed the treatment of 311 patients with primary osteosarcoma treated at a specialist centre between 1995 and 2004. Of this number we identified 8 patients (2.5%) who had undergone inappropriate surgical treatment otherwise known as a ‘whoops’ procedure. These patients were studied to determine the impact of the whoops operations on their subsequent treatment and prognosis.

Results: The whoops procedure consisted of arthroscopic surgery in 3 patients, open reduction and internal fixation of pathological fractures in 2 patients, excision in 2 patients and curettage in 1 patient. The patients who had a whoops procedure were three times more likely to be male, older (median age 25 versus 16 years), had tumours in the lower extremities and were more likely to have amputation as primary local treatment (40% versus 25%). the rates of local recurrence and survival were similar in those with whoops procedures and without.

Conclusion: Whoops procedure is associated with increased risk of amputation and more extensive procedures but does not appear to affect the prognosis of the patient.

Non-invasive expandable prostheses for limb salvage tumour surgery were first used in 2002. These implants allow ongoing lengthening of the operated limb to maintain limb-length equality and function while avoiding unnecessary repeat surgeries and the phenomenon of anniversary operations.

A large series of skeletally immature patients have been treated with these implants at the two leading orthopaedic oncology centres in England (Royal National Orthopaedic Hospital, Stanmore, and Royal Orthopaedic Hospital, Birmingham).

An up to date review of these patients has been made, documenting the relevant diagnoses, sites of tumour and types of implant used. 74 patients were assessed, with an age range of 7 – 16 years and follow up range of 4 – 88 months.

We identified five problems with lengthening. One was due to soft tissue restriction which resolved following excision of the hindering tissue. Another was due to autoclaving of the prosthesis prior to insertion and this patient, along with two others, all had successful further surgery to replace the gearbox. Another six patients required mechanism revision when the prosthesis had reached its maximal length. Complications included one fracture of the prosthesis that was revised successfully and six cases of metalwork infection (two of which were present prior to insertion of the implant and three of which were treated successfully with silver-coated implants). There were no cases of aseptic loosening.

Overall satisfaction was high with the patients avoiding operative lengthening and tolerating the non-invasive lengthenings well. Combined with satisfactory survivorship and functional outcome, we commend its use in the immature population of long bone tumour cases.

Introduction: Giant cell tumor (GCT) is a benign but locally aggressive tumor that primarily affects the epiphyses of long bones of young adults. Pulmonary metastases in giant cell tumor are rare. We report our experience of treating pulmonary metastatic GCT of bone over the last 24 years between 1984–2007.

Methods: A retrospective review of patients’ records and oncology database of patients with metastatic GCT

Results: We had 471 patients with GCT of bone out of which 7 patients developed pulmonary metastases (1.48%). Six patients following diagnosis and initial treatment and one with pulmonary metastases present at the diagnosis. There were 4 males and 3 females aged between 23 to 40 years (median, 27 years). All patients had GCT around the knee (distal femur/proximal tibia). All patients eventually required Endoprosthetic Replacement apart from one who was treated with curettage only. The time of pulmonary metastases from initial diagnosis was 16–92 months (median, 44.6 months). All patients who developed metastases in the postoperative period had thoracotomy for excision of the pulmonary metastases. Two patients received chemotherapy for control of the local recurrence. At an average follow up of 151 months (27–304 months), all patients were alive

Discussion: Pulmonary metastases have been reported as 1% to 9% in GCT. Because of its rarity, very little is known about the long-term outcome and the best treatment for the pulmonary lesions. The mortality rates recorded for patients with pulmonary metastatic GCT range from 0% to 37%. In our series the mortality rate was 0% and metastases 1.48%. It seems that surgical resection of pulmonary metastases gave excellent rate of survival.