Low grade central osteosarcoma is a rare intramedullary bone producing tumour. It accounts for only 1–2% of all osteosarcomas. Due to the indolent nature of low grade central osteosarcoma, achieving a correct and prompt diagnosis is the real challenge both from imaging and histology, particularly as it may resemble a benign condition, i.e. Fibrous Dysplasia. We have reviewed 15 cases of low grade central osteosarcoma with long term follow-up (2 to 22 years) to identify problems in diagnosis and treatment and to assess outcome. There were 7 females and 8 males with a mean age of 37 yrs (range 11 to 72 years); 13 cases arose in the lower limb (8 femur, 4 tibia, 1 os calcis), 1 in the pelvis and 1 in the upper limb. The average duration of symptoms prior to presentation was over 2yrs. A primary diagnosis of low grade central osteosarcoma was achieved for only 6 cases (4 open and 2 needle biopsies), in the other 9 the primary diagnoses were GCT, cystic lesion or fibrous lesion (both benign and malignant) and all of them had undergone treatment (usually curettage with or without bone grafting for this). Definitive treatment was with surgery attempting to obtain wide margins. Marginal excision was associated with local recurrence in three cases but there were no local recurrences in patients who had a wide excision, even in those with prior treatment. Only one patient has died following the development of multiple metastases after 9 years. The survival rate is 90% at 15 years. We present this study to show the difficulties in diagnosing this rare type of osteosarcoma and to highlight the importance of wide surgical margins to obtain local control.
LR arose in 11 patients (17.5%) at a mean of 38 months and was related to surgical margins. There were no LR in the 26 wide resections, but the risk of LR was 20% in those with a marginal and 46% in those with an intralesional excision. LR appeared as a higher grade than the primary tumor in 3 patients (27%). 9 (14.3%) patients developed metastases at a median of 22 months (6 – 123). Five patients developed both LR and metastases. Seven patients died from the tumor, 2 patients are alive with metastatic disease. None of the patients with a low grade tumor and wide or marginal margins died of tumor, but 2 patients (18%) operated intralesional died of tumor.
Although bone and soft-tissue sarcomas are rare, early diagnosis and prompt referral to a specialised unit offers the best chance of a successful outcome both in terms of survival and surgical resection. This paper highlights the clinical and radiological features that might suggest the possibility of a bone or soft-tissue sarcoma and suggests a succinct management pathway for establishing whether a suspicious bone or soft-tissue lesion could be malignant.
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.
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.
We identified eight patients of 2900 with a primary malignant bone tumour who had coexisting neurofibromatosis type 1. This was a much higher incidence than would be expected by chance. The patients had a mean age of 22.4 years (9 to 54): five were male. Two patients subsequently developed a second bone sarcoma, one of which was radiation induced. Four of the primary tumours were osteosarcomas, four were spindle-cell sarcomas and one a Ewing’s sarcoma. All the patients were treated with chemotherapy and surgery: six of the eight appear to be cured. This study suggests a possible relationship between neurofibromatosis type 1 and the development of a bone sarcoma, the increased risk being estimated at eight times that of the normal population. We recommend that further research into this possible link should be considered.
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 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 have analysed the pattern of symptoms in patients presenting with synovial sarcoma to identify factors which led to long delays in diagnosis. In 35 children, the early symptoms and the results of clinical and radiological investigation were reviewed, along with the presumed diagnoses. The duration of symptoms was separated into patient delay and doctor delay. Only half of the patients had one or more of the four clinical findings suggestive of sarcoma according to the guidance of the National Institute for Clinical Excellence at the onset of symptoms. Of the 33 children for whom data were available, 16 (48.5%) presented with a painless mass and in ten (30.3%) no mass was identified. Seven (21.2%) had an unexplained joint contracture. Many had been extensively investigated unsuccessfully. The mean duration of symptoms was 98 weeks (2 to 364), the mean patient delay was 43 weeks (0 to 156) and the mean doctor delay was 50 weeks (0 to 362). The mean number of doctors seen before referral was three (1 to 6) and for 15 patients the diagnosis was obtained after unplanned excision. Tumours around the knee and elbow were associated with a longer duration of symptoms and longer doctor delay compared with those at other sites. Delays did not improve significantly over the period of our study of 21 years, and we were unable to show that delay in diagnosis led to a worse prognosis. Our findings highlight the variety of symptoms associated with synovial sarcoma and encourage greater awareness of this tumour as a potential diagnosis in childhood.
We report our experience of treating 17 patients with benign lesions of the proximal femur with non-vascularised, autologous fibular strut grafts, without osteosynthesis. The mean age of the patients at presentation was 16.5 years (5 to 33) and they were followed up for a mean of 2.9 years (0.4 to 19.5). Histological diagnoses included simple bone cyst, fibrous dysplasia, aneurysmal bone cysts and giant cell tumour. Local recurrence occurred in two patients (11.7%) and superficial wound infection, chronic hip pain and deep venous thrombosis occurred in three. Pathological fracture did not occur in any patient following the procedure. We conclude that non-vascularised fibular strut grafts are a safe and satisfactory method of treating benign lesions of the proximal femur.
We investigated whether our policy of routine re-excision of the tumour bed after an unplanned excision of a soft-tissue sarcoma was justified. Between April 1982 and December 2005, 2201 patients were referred to our hospital with the diagnosis of soft-tissue sarcoma, of whom 402 (18%) had undergone an unplanned excision elsewhere. A total of 363 (16.5%) were included in this study. Each patient was routinely restaged and the original histology was reviewed. Re-excision was undertaken in 316 (87%). We analysed the patient, tumour and treatment factors in relation to local control, metastasis and overall survival. Residual tumour was found in 188 patients (59%). There was thus no residual disease in 128 patients of whom 10% (13) went on to develop a local recurrence. In 149 patients (47%), the re-excision specimen contained residual tumour, but it had been widely excised. Local recurrence occurred in 30 of these patients (20%). In 39 patients (12%), residual tumour was present in a marginal resection specimen. Of these, 46% (18) developed a local recurrence. A final positive margin in a high-grade tumour had a 60% risk of local recurrence even with post-operative radiotherapy. Metastases developed in 24% (86). The overall survival was 77% at five years. Survival was related to the grade of the tumour and the finding of residual tumour at the time of re-excision. We concluded that our policy of routine re-excision after unplanned excision of soft-tissue sarcoma was justified in view of the high risk of finding residual tumour.
We have investigated the oncological outcome of 63 patients with soft-tissue sarcomas of the hand managed at three major centres in the United Kingdom. There were 44 males and 19 females with a mean age of 45 years (11 to 92). The three most common diagnoses were synovial sarcoma, clear cell sarcoma and epithelioid sarcoma. Local excision was carried out in 45 patients (71%) and amputation in 18 (29%). All those treated by amputation had a wide margin of excision but this was only achieved in 58% of those treated by local excision. The risk of local recurrence was 6% in those treated by amputation compared with 42% for those who underwent attempted limb salvage. An inadequate margin of excision resulted in a 12 times greater risk of local recurrence when compared with those in whom a wide margin of excision had been achieved. We were unable to demonstrate any role for radiotherapy in decreasing the risk of local recurrence when there was an inadequate margin of excision. Patients with an inadequate margin of excision had a much higher risk of both local recurrence and metastasis than those with wide margins. The overall survival rate at five years was 87% and was related to the grade and size of the tumour and to the surgical margin. We have shown that a clear margin of excision is essential to achieve local control of a soft-tissue sarcoma in the hand and that failure to achieve this results in a high risk of both local recurrence and metastastic disease.
Pathological fractures due to metastasis with destruction of the acetabulum and central dislocation of the hip present a difficult surgical challenge. We describe a series using a single technique in which a stable and long-lasting reconstruction was obtained using standard primary hip replacement implants augmented by strong, fully-threaded steel rods with cement and steel mesh, where required. Between 1997 and 2006, 19 patients with a mean age of 66 years (48 to 83) were treated using a modified Harrington technique. Acetabular destruction was graded as Harrington class II in six cases and class III in 13. Reconstruction was achieved using three 6.5 mm rods inserted through a separate incision in the iliac crest followed by augmentation with cement and a conventional cemented Charnley or Exeter primary hip replacement. There were no peri-operative deaths. At the final follow-up (mean 25 months (5 to 110)) one rod had fractured and one construct required revision. Of the 18 patients who did not require revision, 13 had died. The mean time to death was 16 months (5 to 55). The mean follow-up of the five survivors was 31 months (18 to 47). There were no cases of dislocation, deep infection or injury to a nerve, the blood vessels or the bladder.
We have investigated whether improvements in design have altered the outcome for patients undergoing endoprosthetic replacement of the proximal tibia following resection of a tumour. Survival of the implant and ‘servicing’ procedures have been documented using a prospective database. A total of 194 patients underwent a proximal tibial replacement, with 95 having a fixed-hinge design and 99 a rotating-hinge with a hydroxyapatite collar; their median age was 21.5 years (10 to 74). At a mean follow-up of 14.7 years (5 to 29), 115 patients remain alive. The risk of revision for any reason in the fixed-hinge group was 32% at five years, 61% at ten years and 75% at 15 and 20 years, and in the rotating-hinge group 12% at five years, 25% at ten years and 30% at 15 years. Aseptic loosening was the most common reason for revision in the fixed-hinge knees, fracture of the implant in the early design of rotating hinges and infection in the current version. The risk of revision for aseptic loosening in the fixed-hinge knees was 46% at ten years. This was reduced to 3% in the rotating-hinge knee with a hydroxyapatite collar. The cemented, rotating hinge design currently offers the best chance of long-term survival of the prosthesis.
Between 1966 and 2001, 1254 patients underwent excision of a bone tumour with endoprosthetic replacement. All patients who had radiotherapy were identified. Their clinical details were retrieved from their records. A total of 63 patients (5%) had received adjunctive radiotherapy, 29 pre-operatively and 34 post-operatively. The mean post-operative Musculoskeletal Tumor Society scores of irradiated patients were significantly lower (log-rank test, p = 0.009). The infection rate in the group who had not been irradiated was 9.8% (117 of 1191), compared with 20.7% (6 of 29) in those who had pre-operative radiotherapy and 35.3% (12 of 34) in those who radiotherapy post-operatively. The infection-free survival rate at ten years was 85.5% for patients without radiotherapy, 74.1% for those who had pre-operative radiotherapy and 44.8% for those who had post-operative radiotherapy (log-rank test, p <
0.001). The ten-year limb salvage rate was 89% for those who did not have radiotherapy and 76% for those who did (log-rank test, p = 0.02). Radiotherapy increased the risk of revision (log-rank test, p = 0.015). A total of ten amputations were necessary to control infection, of which nine were successful. Radiotherapy may be necessary for the treatment of a bone sarcoma but increases the risk of deep infection for which amputation may be the only solution.