Purpose

Ewings sarcoma (EWS) is the second most common primary malignancy of bone. Five-year survival ranges between 50 and 70% for patients who present with localized disease. EWS occurs in both axial and appendicular locations. The prognostic importance of anatomic location (axial vs. appendicular) is unclear. The purpose of this study is to report a single institution experience in the management of patients with EWS, and to investigate anatomic location as a prognostic factor.

It is generally accepted that there is a high rate of local recurrence following surgical excision of chordoma of the sacrum, even if the margins of excision appear clear. There is uncertainty as to whether the addition of postoperative radiotherapy may decrease the risk of local recurrence, particularly if there are close or involved margins. We aimed to determine the effect of conventional radiotherapy, in the post-operative setting, on the effect of local recurrence, metastases and patient survival in a multi-centre study.

Osteosarcomas represent a heterogeneous group of primary bone tumours that affect predominantly the long bones of patients in the first two decades of life. We aim to describe the secondary effects of a poor response (⋋90% necrosis) to chemotherapy on the effectivity of other treatment outcomes, local recurrence and survival rates.

182 cases of osteosarcoma with necrosis of less than 90% and no metastases at diagnosis have been seen at our institution over 24 years. There were 60 amputations. 122 patients underwent limb salvage, with 105 marginal margins and 17 contaminated. There was no difference in size or location between the two groups. In the 122 patients with LSS, 21 had adjuvant radiotherapy and 101 did not. In the entirety of patients with ⋋90% necrosis, survival was 64% at 2 years and 37% at 5 years. When LSS Marginal resections were compared with amputation there was a significant (P=0.006) difference in survival. LSS with a marginal margin had a 25% risk of LR. In these patients there was 25% survival, whereas the absence of a local recurrence, conferred a benefit of a 40% survival XRT was used in 21 of the 122 who underwent limb salvage. The decision to use XRT was made by the local oncologist at the treating unit. There was a 24% rate of recurrence in the XRT group and 25% with no XRT.

These data demonstrated that patients who had a poor response to chemotherapy and underwent an amputation faired poorly when compared to patients with LSS. There is a selection bias in patients selected to undergo amputation. Additionally, patients who underwent amputation had a lower rate of local recurrence, but still had a poorer survival when compared to LSS.

Introduction

The role of adjuvants in curettage for giant cell tumours (GCT) is still controversial. Our aim was to determine if adjuvant cementation lowers local recurrence (LR) rates for GCTs treated with curettage.

The aim was to identify when primary amputation was used as primary treatment and to describe outcomes in patients managed with modern chemotherapy. A detailed review of the electronic patient records was undertaken. Statistical analysis was performed with univariate analysis using Kaplan-Meier curves and Chi² testing, whilst multivariate analysis was performed using Cox regression analysis.

There were 354 osteosarcomas. 93 patients presented with metastases and 192 subsequently developed metastases at a mean of 46 months. Amputation was performed as the primary surgical treatment in 101 patients. Endoprosthetic reconstruction was used in 253 patients. Amputation was performed as a secondary procedure on 15 patients.

The 5 and 10 year survival data for all patients, including those with metastatic disease were 60% and 60% for amputation with good chemotherapy response (>89% necrosis), 65% and 63% for limb salvage and good response, 21% and 21% for amputation and poor response (⋋90% necrosis) and 51% and 30% for limb salvage with poor response.

Local recurrence occurred both with amputation (10.8%) or limb salvage (9%), with no significant differences between the two.

Univariate analysis demonstrated that the extent of response to chemotherapy induced necrosis significantly affected survival, whether the patient had an amputation or not.

Whether or not amputation or LSS was used in the surgical management of patients, local recurrence rates where similar between the two groups. Further assessment of chemotherapy-induced necrosis is a key factor in determining subsequent limb salvage or amputation management strategies.

Introduction

Fibromatosis is a disorder characterised by a spectrum of biological behaviour from relative indolence to aggressive local infiltration. With aimed to describe the pre and post-operative functional status of these patients managed with surgery and analyse the effect of radiotherapy on functional outcome.

Fibromatosis represent a highly heterogeneous group of tumours in growth pattern, location and management. Our aim was to describe the demographics of the patient population who had undergone surgical resection and to identify predictors of local recurrence. Any lesion that was infiltrating the chest or abdominal cavity was excluded. Patients were also not included if they had a plantar or palmar lesions or had received hormonal or chemotherapy.

67 men and 88 women aged from 16 to 77 with a median age of 39 were analyzed. 121 patients had no prior resective operative intervention. 34 patients had undergone an attempted resection procedure at another unit of which 30 had locally recurred. 3 were located in the abdominal wall, 5 chest wall, 15 paraspinal, 56 lower and 76 upper limb. 40 patients did not receive XRT, 18 in the post-operative period and 97 in the pre-operative period. 67 operations produced margin negative resection, 85 were positive and 3 in which the margin status was unknown. Follow-up ranged from 1 day post op to 23.3 years. 23 patients had a local recurrence. Following subsequent re-resections, the total number of patients who were alive with evidence of disease was 16.

6 pts had deceased. 149 were alive with no evidence of disease. No factors were found to be statistically significant for predicting local recurrence, including the use of radiation (0.06) and margin status (0.81). Although radiation, given either pre or post-operatively did trend towards preventing local recurrence (HR 0.40; 95% CI 0.15 to 1.06; p = 0.06).

We retrospectively compared the outcome after the treatment of giant cell tumours of bone either with curettage alone or with adjuvant cementation. Between 1975 and 2008, 330 patients with a giant cell tumour were treated primarily by intralesional curettage, with 84 (25%) receiving adjuvant bone cement in the cavity. The local recurrence rate for curettage alone was 29.7% (73 of 246) compared with 14.3% (12 of 84) for curettage and cementation (p = 0.001). On multivariate analysis both the stage of disease and use of cement were independent significant factors associated with local recurrence. The use of cement was associated with a higher risk of the subsequent need for joint replacement. In patients without local recurrence, 18.1% (13 of 72) of those with cement needed a subsequent joint replacement compared to 2.3% (4 of 173) of those without cement (p = 0.001). In patients who developed local recurrence, 75.0% (9 of 12) of those with previous cementation required a joint replacement, compared with 45.2% (33 of 73) of those without cement (p = 0.044).

Purpose: Impending and pathologic fractures of the humerus, usually due to metastatic disease, are associated with significant pain, morbidity, loss of function, and diminished quality of life. Several methods of stabilization have been described. Here we report the outcome of fixation using intramedullary poly methyl methacrylate (bone cement) and non-locking plates.

Method: A retrospective review was undertaken which included all patients treated at a tertiary musculoskeletal oncology referral center from February, 1989 to October, 2009. Patients who underwent surgical management of an impending or pathologic fracture of the humerus were included. All patients were treated using the following technique: Vascular tumors were embolized pre-operatively. Following gross tumor removal through curettage, antibiotic bone cement was placed into the humeral canal and bone defect. If there was a fracture, the bone ends were held in place as the cement cured. The humerus was stabilized using non-locking plates fixed with screws inserted through the bone and hardened bone/cement composite. Ideally, plates spanned the osseous defect by at least 2 cortical diameters and often the entire length of the bone.

Results: Clinical records were available for 67 patients who underwent the above procedure. There were 44 males and 23 females with an average age of 62.2 years. In 76% of patients there was a pathologic fracture at presentation, while in 24% it was impending. The most common histology was myeloma (21%), followed by renal (20%) and lung adenocarcinoma (20%). Forty-nine patients (73%) had one plate, 16 (24%) had two plates, one patient had three plates, and one had four plates. Complications occurred in 14 (21%) cases, and eight (12%) required reoperation of the humerus. The most common cause for reoperation was disease progression (six of eight). There were two nerve palsies, one deep infection, and one hardware failure. Interestingly, the single hardware failure occurred in a patient whose pain relief and functional status improved to the point that he fractured his construct while hammering with the affected arm in a home improvement project.

Conclusion: Intralesional tumor resection and stabilization of impending and pathologic fractures of the humerus with the described technique has several attributes. Most importantly, it provides immediate, absolute rigidity of the upper extremity and enables early pain relief and return of function without the need for osseous union. Radiation has no negative effects on the construct. The patient’s local disease burden is reduced, thus helping to alleviate tumor-related pain and slow local disease progression. Finally, this technique is user-friendly and cost-effective as it does not require any special equipment or devices that are not available to community orthopaedic surgeons. This technique provides a durable option for the treatment of impending and pathologic humerus fractures.

Purpose: Massive endoprostheses have become the mainstay of treatment for reconstruction after resection of primary bone tumours. The Kotz Modular Femoral Tibial Replacement (KMFTR, Kotz prosthesis, Stryker Inc.) system has been one of the most widely utilized uncemented modular systems. Although this prosthesis has excellent bone ingrowth characteristics and a low aseptic loosening rate, we have identified a significant incidence of mechanical failure and breakage of the prosthesis. The purpose of this investigation is to review the outcomes after prosthetic revision for a broken Kotz prosthesis.

Method: A retrospective review was undertaken of our institutional database from the years 1989, when we first utilized the Kotz prosthesis, until present. We identified all patients who had undergone a revision of the prosthesis for mechanical failure or prosthetic breakage. Periprosthetic fractures and revisions for polyethylene bushing wear were excluded.

Results: 119 distal femoral, 55 proximal tibial and 47 proximal femoral Kotz endoprostheses (221 in total) have been implanted in our center since 1989. There were 21 revisions (9.5% of total prostheses) for mechanical failure. Of these, 16 were in the distal femur, four in the proximal tibia and one in the proximal femur. Mechanical failures occurred at a mean of 77 months (range 24–170). Of the 21 metal failures, 8 stems broke at the junction of the stem and body, 8 fractured through screw holes in the stem, 3 fractured the derotation lug, one fractured the tibial housing and one lateral side-plate failed. Of these failures only three implants had associated definite loosening and two of these three were cemented. Broken stems initially required extraction whilst preserving as much of the longitudinal and transverse bone stock as possible in order to facilitate osseo-mechanical integration of the revision prosthesis. This was accomplished using trephines to core the ingrown broken stem out of the bone. Over the last 20 years, the 16 broken stems have been revised in 5 patients to larger Kotz uncemented stems, 2 to cemented GMRS stems with an adaptor to the KMFTR system, 3 to Restoration uncemented revision hip stems with a custom adaptor to the KMFTR system, 2 to custom GMRS uncemented stems with an adaptor to the KMFTR system, and 4 to total femurs. All except one patient was alive with no evidence of disease. Post-revision, 14 patients had TESS, MSTS87, MSTS93 scores of 80.5, 25.5 and 70 respectively.

Conclusion: Despite very low aseptic loosening rates, mechanical failure of the Kotz prosthesis continues to be a significant clinical problem even several years after implantation. Fatigue failure often leads to the difficult scenario of removing a well-ingrown uncemented stem. Our data illustrates that these prostheses can often be successfully revised by trephining out the broken stem and inserting new uncemented stems. Functional outcome continues to be good and is comparable to pre-revision levels.

Pathological fractures of the humerus are associated with pain, morbidity, loss of function and a diminished quality of life. We report our experience of stabilising these fractures using polymethylmethacrylate and non-locking plates. We undertook a retrospective review over 20 years of patients treated at a tertiary musculoskeletal oncology centre. Those who had undergone surgery for an impending or completed pathological humeral fracture with a diagnosis of metastatic disease or myeloma were identified from our database. There were 63 patients (43 men, 20 women) in the series with a mean age of 63 years (39 to 87).

All had undergone intralesional curettage of the tumour followed by fixation with intramedullary polymethylmethacrylate and plating. Complications occurred in 14 patients (22.2%) and seven (11.1%) required re-operation. At the latest follow-up, 47 patients (74.6%) were deceased and 16 (25.4%) were living with a mean follow-up of 75 months (1 to 184). A total of 54 (86%) patients had no or mild pain and 50 (80%) required no or minimal assistance with activities of daily living. Of the 16 living patients none had pain and all could perform activities of daily living without assistance.

Intralesional resection of the tumour, filling of the cavity with cement, and plate stabilisation of the pathological fracture gives immediate rigidity and allows an early return of function without the need for bony union. The patient’s local disease burden is reduced, which may alleviate tumour-related pain and slow the progression of the disease. The cemented-plate technique provides a reliable option for the treatment of pathological fractures of the humerus.

Survival of patients with high-grade osteosarcoma has significantly improved with combined multi-agent chemotherapy and aggressive local surgical control. However, despite modern therapy, approximately one-third recur and those that do recur are difficult to treat successfully. The recurrence of osteosarcoma is rare. Local recurrence occurs in 4–10% of patients following effective treatment.

This report details a lady with local recurrence of osteosarcoma seventeen years following initial presentation. She was diagnosed with an osteosarcoma with both chondroblastic and osteoblastic differentiation of the right ilium in November 1989 (aged 41). There were no distant metastases. She received one cycle of neo-adjuvant chemotherapy (PIA) prior to a right hemipelvectomy in April 1990. Six weeks post excision, she underwent a hemipelvic and proximal femoral replacement. She received 5 cycles of adjuvant (PIA) chemotherapy. Post-operative recovery was complicated by infection leading to formation of a discharging sinus. Despite exploration and an external oblique rotation graft, the sinus continued to discharge and the femoral and pelvic prostheses were removed in March 1994.

She mobilised with the use of two crutches and functioned extremely well. She was not keen for reinsertion of a prosthesis and remained on yearly follow-up until 2000.

In June 2007, she presented to her general practitioner with dull right iliac fossa pain. She was referred back to our service and examination revealed a mass in the right iliac fossa. This was biopsied and demonstrated locally recurrent osteosarcoma. Staging investigations revealed no metastatic disease.

She had excision of the osteosarcoma in September 2007 followed by re-excision local re-recurrence within psoas in April 2008. To our knowledge, this is the first time that locally recurrent intramedullary osteosarcoma, 17 years from initial diagnosis and treatment, is described in the literature. This case serves as a useful clinical reminder.