Ewing’s sarcoma principally arises in bone but can also present as a soft tissue tumour. Very few studies have assessed the outcomes of extra-skeletal Ewing’s sarcomas. This study compares the oncological outcomes of the two forms of Ewing’s sarcomas to see if there is any difference in prognostic factors. 198 patients with primary, non metastatic Ewing’s sarcoma diagnosed between 1980 and 2005 were identified from our database. There were 118 males and 80 females with a median age of 15 years. The three most common sites of diagnosis were the femur (24%), pelvis (15%) and tibia (13%). There were 169(85%) bony Ewing’s and 29 (15%) extra-skeletal Ewing’s sarcomas. All patients received chemotherapy. 86% of the patients had surgery for local control but 28(14%) patients had radiotherapy. The overall survival at five years was 89% and was related to the age of patient (92% <
16years p=0.005), size (p=0.03) and site of tumour (p=0.004) as well as the response to chemotherapy. There was no difference in the overall survival of patients with bony Ewing’s (90%) and extra-skeletal Ewing’s (85%) (p=0.85). There was a 10% risk of local recurrence at 5 years with site of tumour (p=0.01) and surgical excision (p=0.05) being significant prognostic factors. The risk of local recurrence was also not related to the type of Ewing’s sarcoma. This large series has shown that the oncological outcomes of Ewing’s sarcoma is related to tumour characteristics, patient age and treatment factors and not determined by the tissue component.
Disappearing bone disease is also known as vanishing bone disease, phantom bone disease, massive osteolysis, Gorham’s disease or Gorham-Stout disease. Basically, it is characterised by osteolysis in (contiguous) bone segments, due to localised proliferation of thin-walled vascular channels in the bone and surrounding soft tissues. The etiology and pathophysiology of this condition remain poorly understood and largely unclear, but there is increasing evidence that disordered lymphangiogenesis plays a role. It is an extremely rare cause of osteolysis, so all other differential diagnoses should be considered and ruled out before retaining the diagnosis of disappearing bone disease. Treatment is fairly disappointing and no single treatment modality has proven effective in actually arresting the disease. Conservative treatment includes ant-resorptive agents (bisphosphonates), immunomodulating substances and radiation therapy, whereas surgical treatment options include resection and reconstruction with bone grafts and/or prostheses versus amputation. We report on the only two cases that were identified in our database between 1984 and 2008, both affecting the lower limb (one tibia, one femur). In an attempt to limb salvage, these patients initially underwent endoprosthetic replacement of the affected bone segment, but due to disease progression both eventually ended up with a hip disarticulation.
Given that the incidence of bone sarcomas is 9/million population per year, our 3000 patients represent 333 million population years. When the incidence of a condition is known in the population this allows an estimation of the risk of malignancy compared with the normal population. Retinoblastoma for instance is known to arise in 1 in 16000 births. The 7 malignancies we saw thus represents a risk to individuals with retinoblastoma of 336/million/yr – a figure 37 times the risk of the normal population. Approximate figures of risk have been calculated for other entities.
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, endoprosthetic 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. 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%). Medical records were evaluated for information on local recurrence, metastases, complications and functional outcome using the Toronto Extremity Salvage Score (TESS). 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 the two earlier cases had problems with metacarpal stems cutting out and jointsubluxatinos. The two earlier cases have since died at 205 (parosteal osteosarcoma) and 189 months (GCT) respectively of other disease. 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 postoperative functional results and complication rates when a biological solution or preservation of the wrist joint is not indicated.
The 38 procedures were identified from September 1995 to June 2007 and included 17 distal femoral replacements, 12 proximal femoral replacements, 4 proximal humeral replacements, 2 distal humeral replacements, 2 hemi-pelvic replacements and 1 total femoral replacement. EPR survivorship was calculated using a Kaplan-Meier survival curve. The quality of patients’ mobility and performance of activities of daily living was used to assess functional outcome.
87.4% of patients who underwent a lower limb EPR achieved a satisfactory or very satisfactory functional outcome. 100% of patients achieved a satisfactory or very satisfactory functional outcome in the upper limb EPR group. 3 implants failed, 2 as a result of infection and required staged revisions, 1 eventually requiring amputation, and 1 failed as a result of aseptic loosening. 2 patients dislocated their proximal femoral replacements, both were treated successfully by closed reduction. Despite the salvage surgery subsequent amputation was only required in one patient.
Overall cumulative patient survival was 58% at 5 years and 44% at 10 years. Locally recurrent disease occurred in 350 patients (14%), 204 patients (8%) presented with and 720 patients (30%) subsequently developed metastatic disease. Prognostic factors for locally recurrent disease were arm tumours (p=0.003, HR=0.3), hip tumours (p=0.01, HR=0.31), thigh tumours (p=0.002, HR=0.52), intralesional margins (p<
0.0001, HR=3.7), high grade tumours (p=0.03, HR=1.8), tumour size 3–6cm (p=0.04, HR=0.54) and tumour size 6–10cm (p=0.03, HR=0.63). Prognostic factors for patient survival were deep location (p=0.02, HR=1.6), high grade tumours (p<
0.0001, HR=4.7), intermediate grade tumours (p<
0.0001, HR=3.4), surgical margins (p=0.04), age at diagnosis (p<
0.0001, HR=1.02), size of tumour <
3cms (p=0.04, HR=0.29), 3–6cms (p<
0.0001, HR=0.41), 6–10cms (p=0.007, HR=0.63), no locally recurrent disease (p=0.0001, HR=0.59).
Of the 7242 patients with soft tissue lumps, 476 had a past history of malignancy. Of these patients, only 12% actually had a soft tissue metastasis while 28% had a benign diagnosis, 55% a soft tissue sarcoma and 5% other malignancy.
In our database of 7935 patients referred for investigation of a soft-tissue mass, only 100 were found to have a soft-tissue metastasis (1.3%). Our aim was to define the clinical features of such patients and to identify the site of their primary tumour. The most common presentation was a painful lump, deep to the fascia, ranging between 2 cm and 35 cm (mean 8.3 cm) with 78% of the lumps located deep to the fascia. The mean age of the patients at presentation was 64 years (22 to 84) and there were almost equal numbers of men and women. Of 53 patients with a history of malignancy, 52 had metastases from the same primary (lung in 12, melanoma in ten, kidney in nine, gastrointestinal track in four, breast in five, bladder in four, and others in eight). The other 47 had no history of malignancy and the metastasis was the first presentation. The primary sites in these cases were the lung in 19, gastro-intestinal track in four, kidney in two, melanoma in nine, other in three, and unknown (despite investigations) in ten. There was no correlation between the site of the metastases and the primary tumour. Of the 7935 patients, 516 had a history of malignancy. Of these, only 10% had a soft-tissue metastasis, 29% had a benign diagnosis, 55% a soft-tissue sarcoma and 6% another malignancy. Patients with soft-tissue metastases have similar clinical features to those with soft-tissue sarcomas and should be considered for assessment at appropriate diagnostic centres for patients with suspicious soft-tissue lumps.
We reviewed the treatment and clinical outcome of 32 consecutive patients with Ewing’s sarcoma who presented with or developed pathological fracture after biopsy between 1984 and 2004. The minimum follow-up was 18 months. The mean age at diagnosis was 20 years (5 – 51). There were 18 males and 14 females. All patients were newly diagnosed and had localized disease at the time of diagnosis. 21 patients presented with pathological fracture while 11 patients developed fracture during the course of chemotherapy. The femur was the most common location in 15 patients. All the patients had chemotherapy according to the protocol current at the time of treatment. 6 patients had radiotherapy alone while 26 patients underwent surgical excision and reconstruction. Of the patients who had surgery, 7 patients had adjuvant radiotherapy. Fracture healing was the norm after pre-operative chemotherapy. Surgical margins were wide in 17 patients, marginal in 4 and intralesional in 3 patients. Local recurrence developed in one patient (3%). Metastases occurred in 12 patients (37%). At the time of review 16 patients were free of disease, 3 were alive with disease and 13 patients had died of disease. The cumulative 5 year metastases free and overall survival in all the patients was 58% and 61 % respectively and similar to patients with Ewing’s sarcoma without fracture treated at our centre. The prognosis of patients who presented with fracture was exactly similar to those who developed fracture in the course of treatment. We conclude that limb preserving surgery is perfectly safe in patients with Ewing’s sarcoma who have associated pathological fracture and survival is not in any way compromised. Survival of patients who present with fracture is similar to those who develop fracture in the course of treatment. The exact role of adjuvant radiotherapy in these patients needs to be clarified.
We undertook a cemental unipolar proximal femoral endoprosthetic replacement in 131 patients with a mean age of 50 years (2 to 84). Primary malignant tumours were present in 54 patients and 67 had metastatic disease. In addition, eight patients had either lymphoma or myeloma and two had non-oncological disorders. The mean follow-up was 27 months (0 to 180). An acetabular revision was required later in 14 patients, 12 of whom had been under the age of 21 years at the time of insertion of their original prosthesis. The risk of acetabular revision in patients over 21 years of age was 8% at five years compared with 36% in those aged under 21 years. All the unipolar hips in this younger age group required revision within 11 years of the initial operation. We conclude that unipolar replacement should not be used in younger patients and should be avoided in patients with a life expectancy of more than five years.
Endoprosthetic replacement of the proximal femur may be required to treat primary bone tumours or destructive metastases either with impending or established pathological fracture. Modular prostheses are available off the shelf and can be adapted to most reconstructive situations for this purpose. We have assessed the clinical and functional outcome of using the METS (Stanmore Implants Worldwide) modular tumour prosthesis to reconstruct the proximal femur in 100 consecutive patients between 2001 and 2006. We compared the results with the published series for patients managed with modular and custom-made endoprosthetic replacements for the same conditions. There were 52 males and 48 females with a mean age of 56.3 years (16 to 84) and a mean follow-up of 24.6 months (0 to 60). In 65 patients the procedure was undertaken for metastases, in 25 for a primary bone tumour, and in ten for other malignant conditions. A total of 46 patients presented with a pathological fracture, and 19 presented with failed fixation of a previous pathological fracture. The overall patient survival was 63.6% at one year and 23.1% at five years, and was significantly better for patients with a primary bone tumour than for those with metastatic tumour (82.3% vs 53.3%, respectively at one year (p = 0.003)). There were six early dislocations of which five could be treated by closed reduction. No patient needed revision surgery for dislocation. Revision surgery was required by six (6%) patients, five for pain caused by acetabular wear and one for tumour progression. Amputation was needed in four patients for local recurrence or infection. The estimated five-year implant survival with revision as the endpoint was 90.7%. The mean Toronto Extremity Salvage score was 61% (51% to 95%). The implant survival and complications resulting from the use of the modular system were comparable to the published series of both custom-made and other modular proximal femoral implants. We conclude that at intermediate follow-up the modular tumour prosthesis for proximal femur replacement provides versatility, a low incidence of implant-related complications and acceptable function for patients with metastatic tumours, pathological fractures and failed fixation of the proximal femur. It also functions as well as a custom-made endoprosthetic replacement.
We treated 98 patients with peri-acetabular tumours by resection and reconstruction with a custom-made pelvic endoprosthesis. The overall survival of the patients was 67% at five years, 54% at ten years and 51% at 30 years. One or more complications occurred in 58.1% of patients (54), of which infection was the most common, affecting 30% (28 patients). The rate of local recurrence was 31% (29 patients) after a mean follow-up of 71 months (11 to 147). Dislocation occurred in 20% of patients (19). Before 1996 the rate was 40.5% (17 patients) but this was reduced to 3.9% (two patients) with the introduction of a larger femoral head. There were six cases of palsy of the femoral nerve with recovery in only two. Revision or excision arthroplasty was performed in 23.7% of patients (22), principally for uncontrolled infection or aseptic loosening. Higher rates of death, infection and revision occurred in men. This method of treatment is still associated with high morbidity. Patients should be carefully selected and informed of this pre-operatively.
We reviewed the treatment and clinical outcome of 32 consecutive patients with Ewing’s sarcoma who presented with or developed pathological fracture after biopsy between 1984 and 2004. The minimum follow-up was 18 months. The mean age at diagnosis was 20 years (5 – 51). There were 18 males and 14 females. All patients were newly diagnosed and had localized disease at the time of diagnosis. 21 patients presented with pathological fracture while 11 patients developed fracture during the course of chemotherapy. The femur was the most common location in 15 patients. All the patients had chemotherapy according to the protocol current at the time of treatment. 7 patients had radiotherapy alone while 25 patients underwent surgical excision and reconstruction. Of the patients who had surgery, 7 patients had adjuvant radiotherapy. Fracture healing was the norm after pre-operative chemotherapy. Surgical margins were wide in 17 patients, marginal in 4 and intralesional in 3 patients. Local recurrence developed in one patient (3%). Metastases occurred in 12 patients (37%). At the time of review 16 patients were free of disease, 3 were alive with disease and 13 patients had died of disease. The cumulative 5 year metastases free and overall survival in all the patients was 58% and 61 % respectively and similar to patients with Ewing’s sarcoma without fracture treated at our centre. The prognosis of patients who presented with fracture was exactly similar to those who developed fracture in the course of treatment. We conclude that limb preserving surgery is perfectly safe in patients with Ewing’s sarcoma who have associated pathological fracture and survival is not in any way compromised. Survival of patients who present with fracture is similar to those who develop fracture in the course of treatment. The exact role of adjuvant radiotherapy in these patients needs to be clarified.
We report the results of contained bone defects after curettage of benign bone tumours of the distal radius treated without bone grafting or the use of bone substitute. 11 consecutive patients treated with follow-up of 3 to 11 years (mean 5.7 years) were studied. The mean age at diagnosis was 27 (range 11 to 55). There were7 males and 4 females. Histological diagnosis was giant cell tumour in 8 and aneurismal bone cyst in 3 patients. The mean bone defect at diagnosis was 23.7cm3 (9.2 – 68cm3). Pathological fracture was present in 5 patients prior to surgery. We observed full radiological consolidation of the defects in all the patients within 12 months of surgery. Radiologically detectable osteoarthritis was noted in 5 patients (grade 1 in two patients, grade 2 in one and grade 4 in two patients). Development of osteoarthritis was significantly related to size of the defect and involvement of the joint by the original tumour. No patient without joint involvement developed osteoarthritis. There was no relationship between pathological fracture and development of osteoarthritis. We conclude that contained bone defects in the distal radius do rapidly consolidate without the use of bone grafting or bone substitute. The bone remodels nicely over time. Development of osteoarthritis is related to the damage to the articular defect caused by the tumour.
Synovial sarcoma is a morphologically well-defined neoplasm that most commonly occurs in soft tissue accounting for 5% to 10 % of all soft tissue sarcomas. We reviewed 156 patients with synovial sarcoma of soft tissues treated at a supra-regional centre to determine survival and prognostic factors. There were 77men and 79 women with mean age at presentation of 38 years (3 to 84). Follow-up periods ranged from 3 to 494 months (median 43 months). Tumor was located in lower extremities in 111patients, upper extremities in 34 patients, and trunk and pelvis in 11 patients. Overall survival was 66% at 5 years and 48% at 10 years. The 5 and 10 year survival for the 23 patients who had metastases at the time of diagnosis was 13% and 0% respectively compared to 75% and 54% for those without metastases at diagnosis. Local recurrence occurred in 18 patients (13%). The significant prognostic factors for survival included presence of metastases at diagnosis and development of local recurrence. Tumour size and depth, age of patients and use of chemotherapy did not significantly influence survival. We conclude that the clinical factors which influence survival of patients with synovial sarcoma are different from those of soft tissue sarcomas in general. Biological factors may better predict prognostic survival than the usual clinical factors.