Of the 27 repeat biopsies a positive histological diagnosis was obtained in 22 patients. The remaining 5 were again non-diagnostic giving a total of 98 patients being treated definitively without a tissue diagnosis. Of these 98 cases, 39 (40%) were treated non-operatively, 37 (38%) had curettage and 22 (22%) underwent wide excision. In the curettage group 33 out of 37 patients had a benign tumour on final histology. Four patients turned out to have intermediate/high grade tumours and subsequently underwent wide excision. In the wide excision group, 17 out of 22 patients had an intermediate/high grade tumour on final histology. Five patients underwent an unnecessarily wide excision of a benign lesion. None of the patients treated non-operatively turned out to have a tumour.
To determine the diagnostic performance of image-guided percutaneous core needle biopsy (CNB) in patients presenting with pathologic fractures of the appendicular skeleton. To determine factors associated with non-diagnostic biopsy and identify cases which should be considered for primary open biopsy. A retrospective audit identified 129 consecutive patients presenting with pathological fractures to a specialist orthopaedic oncology unit over a 9 year period. All patients underwent percutaneous CNB using CT (n=98), fluoroscopy (n=15) or US (n=16) guidance. In all cases MRI or CT was available prior to biopsy to assess the presence and degree of extra-osseous tumour mass. The resulting sample was classified as diagnostic (Group 1) or non-diagnostic (Group 2) on histopathological study. Diagnostic performance was evaluated on the basis of the diagnostic yield and accuracy; these were related to the site of the lesion and presence/absence of extra-osseous mass. Of 129 biopsies, 99 (77%) were classified as Group 1 and 30 (23%) as Group 2. The commonest sites of pathological fracture without associated soft tissue component and resulting in a non-diagnostic biopsy were the proximal femur and proximal humerus. The average cross-sectional diameter of lesions in Group 1 was 5.7 x 5.9cm. Of the 30 lesions comprising Group 2, no soft tissue component was identified on pre-biopsy cross-sectional imaging in 27 lesions (90%) whereas the remaining 3 (10%) showed a smaller extra-osseous soft tissue component compared to the lesions in Group 1. Image-guided percutaneous CNB is a reliable method for obtaining a tissue diagnosis in patients presenting with a pathologic fracture of the appendicular skeleton with high accuracy rate. However, those lesions which are purely intra-osseous or have only very small extra-osseous components are likely to be associated with a non-diagnostic biopsy, and should be considered for a primary open procedure.
Disarticulation of the hip in patients with high-grade tumours in the upper thigh results in significant morbidity. In patients with no disease of the proximal soft tissue a femoral stump may be preserved, leaving a fulcrum for movement and weight-bearing. We reviewed nine patients in whom the oncological decision would normally be to disarticulate, but who were treated by implantation of an endoprosthesis in order to create a functioning femoral stump. The surgery was undertaken for chondrosarcoma in four patients, pleomorphic sarcoma in three, osteosarcoma in one and fibrous dysplasia in one. At follow-up at a mean of 80 months (34 to 132), seven patients were alive and free from disease, one had died from lung metastases and another from a myocardial infarction. The mean functional outcome assessment was 50 (musculoskeletal tumor society), 50 and 60 (physical and mental Short-form 36 scores). Implantation of an endoprosthesis into the stump in carefully selected patients allows fitting of an above-knee prosthesis and improves wellbeing and the functional outcome.
Elastofibroma dorsi is a rare, benign, slow-growing ‘pseudotumour’ classically presenting as an ill defined mass at the inferior pole of the scapula. Typical symptoms include mass, pain, scapular snapping and impingement like features. There is a predilection for females after the fifth decade of life. The aetiology is unclear. We identified 15 patients (21 tumours) with a diagnosis of elastofibroma. Seven lesions were found on the left side and fourteen on the right; bilateral lesions were found in six patients. The male:female ratio was eight:seven and mean age at presentation was 60.9 years (range 40 – 71). The mean duration of symptoms (most commonly pain, mass and scapular snapping) prior to presentation was 25.8 months. Eighteen tumours were excised with a mean follow-up of 4.2 years (0.25–16). Four lesions were diagnosed by combined MRI and CT guided biopsy, the remainder identified using MRI alone. All patients were asked specifically about symptoms, occupation, family history and employment history (including hobbies). Pain was assessed using the Visual Analogue Score (VAS) and functional outcome using the Stanmore Percentage of Normal Shoulder Assessment (SPONSA) Score. Range of forward flexion of the shoulder joint was also assessed. In the operative group, the mean VAS score improved from 4.6 (0–10) pre-operatively to 2.5 (0–8) post-operatively. Mean SPONSA scores improved from 61.5% (20 – 100) to 81.8% (30 – 100). Mean pre-operative forward flexion was 135 degrees (70 – 180), this improved to 166 degrees (100 – 180) post-operatively. A high number of patients had been involved in occupations involving heavy lifting. MRI had a 100% sensitivity in identifying elastofibroma when correlated with histopathological evaluation. This series demonstrates that elastofibroma may be reliably diagnosed using MRI alone and, in the symptomatic patient, pain and function may be improved through operative excision.
Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue neoplasm most commonly presenting in young to middle-aged adults. LGFMS is an indolent tumour with a deceptively benign histological appearance. Local recurrences are not uncommon and the tumours can metastasise. A particular gene translocation, FUS-CREB3L2, has been shown to occur commonly in cases of LGFMS. The literature suggests that the FUS-CREB3L2 fusion-gene is a specific marker for LGFMS. We report the cytogenetic analysis of 29 cases of LGFMS, and clinical outcomes of 21 patients treated surgically between 1998 and 2008 at our regional bone and soft-tissue tumour centre. The mean age was 45.4 years. The most common location of tumours in our series was the lower limb. The mean follow-up was 30.1 months (range 0 to 125 months). To date, there have been no cases of local recurrence or metastasis. Fifteen of our patients (52.2%) were FUS-CREB3L2 translocation-positive. This suggests either that the translocation incidence in our LGFMS series is lower than other studies, or that reverse-transcriptase polymerase chain reaction (PCR) is substantially less sensitive than the literature suggests. The patients in this series testing positive presented at a younger age (38.2 years, compared to 45.6 years), and had larger tumours than their negative counterparts (mean diameter 97.6mm, compared to 65.2mm), although there was no difference in clinical outcome. We conclude that PCR testing for the FUS-CREB3L2 translocation is a useful tool for confirming the diagnosis of LGFMS, but has no role in predicting short-term clinical outcome. In our experience it is not necessary to perform wide excision, and marginal margins are adequate. Longer-term follow-up is required to elucidate whether the previously reported recurrence and metastasis rates are a true reflection of the nature of this tumour, and may identify differences in the long-term clinical outcome between translocation-positive and negative patients.
Within a study group of 102 consecutive patients diagnosed at a supra-regional bone tumour unit with chondrosarcoma of the femur, tibia or humerus, an association with previously treated breast cancer was noted. There were 58 female patients and 44 male patients. The study group contained six females (10%, mean age 53 years) who had previously been treated for breast cancer, a higher proportion than would be expected. They were referred following identification of a solitary area of increased activity on routine screening with isotope bone scan, presumed to be a solitary bony metastasis. Most (86%) of this breast carcinoma sub-group had developed low-grade bone chondrosarcoma (Trojani grade 0.5-I) and only one case (14%) had developed high-grade chondrosarcoma (Trojani grade II-III). A suspicious long bone lesion on bone scan in a patient with a past medical history of breast cancer must, therefore, not be assumed to be a metastasis without further investigation; the possibility of a chondral lesion should be considered. It is important that patients receive a full multidisciplinary team investigation prior to treatment in order to obtain the correct tissue diagnosis, as the management of these conditions is often different. Our study suggests there may be a relationshipbetween patients previously treated for breast cancer and the development of subsequent chondrosarcoma.
Granular Cell Tumours are rare mesenchymal soft tissue tumours that arise throughout the body and are believed to be of neural origin. They often present as an asymptomatic slow-growing benign solitary lesion but may be multifocal. One to two percent of cases are malignant and can metastasise. Described series in the literature are sparse. We examined our database and identified eleven cases in ten patients treated surgically and followed-up for a period of over six years (May 2002 to January 2009) in our regional bone and soft tissue tumour centre. Five tumours were located in the lower limb, four in the upper limb and two in the axial skeleton. Mean patient age was 31.2 years (range 8 to 55 years). Excision was complete in one case, marginal in five cases and intra-lesional in five cases. No specimens showed evidence of malignancy. No patients required postoperative adjuvant treatment. Mean follow-up was 19.3 months (range 1 to 37 months), with no cases of local recurrence. One case was multi-focal. Histopathological examination revealed the classical features of granular cell tumour in all cases. Typically, tumour cells were diffusely and strongly positive for S100 protein by immunohistochemistry, whereas the other markers tested were negative. We believe this case series to be the largest of its type in patients presenting to an orthopaedic soft tissue tumour unit. We present our findings and correlate it with findings of other series in the literature.
Chondrosarcoma is the second most common primary malignant bone tumour. Distinguishing between grades is not necessarily straightforward and may alter the management of the disease. We evaluated the correlation between the pre-operative needle biopsy and excision biopsy histological grading of chondrosarcoma of the femur, tibia and humerus. A consecutive retrospective series of 100 patients with a histological diagnosis of chondrosarcoma was reviewed. Twenty-one patients were excluded because 20 had only excision biopsy and one had only the pre-operative biopsy on record, thus this series included 79 available cases. In 11 instances, there was a discrepancy in histological grade. Therefore, there was an 86% (68 out of 79) accuracy rate for pre-operative histological grading of chondrosarcoma, based on needle biopsy. However, the accuracy of the diagnostic biopsy to distinguish low-grade from high-grade was 90% (71 out of 79).
Medical records of children <
/=5 years, treated by the London sarcoma service for malignant primary bone tumours (average new cases osteosarcoma (OS)/Ewings sarcoma (ES), all ages: 125/year) between 1999 and 2009, were reviewed.
Complications developed in seven patients: two implants failed requiring revision, one peri-prosthetic fracture occurred, one developed a flexion deformity of 25 degrees at the knee joint, which was subsequently overcome and three died of disseminated disease.
The five most common histopathological diagnoses were chondrosarcoma (9%), osteosarcoma (9%), meta-static renal carcinoma (8%), giant-cell tumour (6%), lymphoma (5%). 77% of biopsies yielded a tissue diagnosis. The remaining 23% underwent open biopsy, repeat image-guided needle biopsy or were not further investigated. In the 30 cases (23%) of non-diagnostic biopsies 80% of these lesions had no extra-osseous component to them and the remaining 20% had a very small extra-osseous component.
This paper describes the preliminary results of a proximal tibial endoprosthesis which spares the knee joint and enables retention of the natural articulation by replacing part of the tibial metaphysis and diaphysis. In eight patients who had a primary malignant bone tumour of the proximal tibia, the distal stem, which had a hydroxyapatite-coated collar to improve fixation, was cemented into the medullary canal. The proximal end had hydroxyapatite-coated extracortical plates which were secured to the remaining proximal tibial metaphysis using cortical screws. The mean age of the patients at operation was 28.9 years (8 to 43) and the mean follow-up was for 35 months (4 to 48). The mean Musculoskeletal Tumour Society score was 79% (57% to 90%), the mean Oxford Knee score was 40 points of 48 (36 to 46) and the mean knee flexion was 112° (100° to 120°). In one patient, revision to a below-knee amputation through the prosthesis was required because of recurrence of the tumour. Another patient sustained a periprosthetic fracture which healed with a painful malunion. This was revised to a further endoprosthesis which replaced the knee. In the remaining six patients the prosthesis allowed preservation of the knee joint with good function and no early evidence of loosening. Further follow-up is required to assess the longevity of these prostheses.
We retrospectively studied 67 patients who underwent proximal humeral replacement with the Bayley-Walker prosthesis, for tumour of the proximal humerus between 1997 and 2007. Of the 67 patients 10 were lost to follow up. Of the 41 surviving patients, function was assessed using the Musculoskeletal Tumour Society (MSTS) Score and the Toronto Extremity Salvage Score (TESS) questionnaire. 4 of the 41 patients received the new Bayley-Walker ‘captured’ proximal humeral replacement. The mean age was 46 years (7–87). The mean MSTS score at follow-up was 72.0 % and the mean TESS score was 77.2 %. The sub-group of 4 pts who received the new captured prosthesis had a mean MSTS score of 77.7 %. There was no mechanical failure of any prostheses in the follow up period. Endoprosthetic replacement for tumour of the proximal humerus with the Bayley-Walker prosthesis, is a reliable operation yielding reasonable functional results and good long-term prosthesis survivorship. The performance of this prosthesis is expected to improve further with the new ‘captured’ prosthesis.
We aimed to determine whether proximal femoral bone remodelling, as measured by DEXA scan for each Gruen Zone, differed for 3 different femoral stem designs. These were:
Group 1 – Triple taper polished (C-stem J&
J DePuy) Group 2 – Double taper polished (TPS J&
J DePuy) Group 3 – Collared satin finish stem (Stanmore Biomet) Ethical Committee approval was obtained and all patients gave informed consent. All operations were performed by 3 surgeons using a standard lateral approach, third generation cementing technique and a standard post-operative regime. Seventy-five patients were randomised prospectively using a stratified randomisation programme for known risk factors in bone density. The randomisation was stratified by age, sex, Charnley Group (A or B), BMI and femoral neck bone mineral density. There were 25 patients in each Group, 5 patients did not have complete data sets and were therefore withdrawn from evaluation. The Groups were similar with regard to age, sex, BMI, BMD and Charnley Group. Patients were followed up at 3, 6, 12 and 24 months by Nurse Practitioners for clinical and radiological evaluation and DEXA scan at each time of follow-up. There were no catastrophic failures of sepsis or dislocation. All patients functioned well at 24 months by Harris Hip Score. The bone remodelling pattern will be described for each stem design. Findings are summarised thus. In the lateral Gruen Zones the triple taper and collared stem design faired better and both behaved differently to the double taper design. In Gruen Zone 7, all stems demonstrated reduced bone density as compared to pre-operative scans but this was less with the triple taper design.