Bone and Soft Tissue Sarcomas represent approximately 1% of all malignant tumours. Delays in diagnosis are frequent and the average size of Sarcomas at diagnosis has averaged 10cm for many years. In 1999 guidance was produced by NICE with the aim of leading to the earlier diagnosis of common cancers – including Sarcomas. We have attempted to analyze whether this guidance has had any impact on either the size of the tumours at diagnosis or the symptom duration prior to diagnosis experienced by the patients. Data for patients referred to the Royal Orthopaedic Hospital in Birmingham between 1992 and 2007 with Bone Sarcomas (n=1592) and Soft Tissue Sarcomas (n=2004) were analysed to determine the effect of the guidance. For Bone Sarcomas the mean size of the tumours decreased from 11.2cm prior to the guidance to 10.7cm after the guidance but the change was not statistically significant (p=0.09). The mean duration of symptoms increased from 18 to 21.2 weeks (p=0.01). For Soft Tissue Sarcomas, mean size fell from 10.8cm to 9.5cm (p<0.001), however the duration of symptoms actually increased from 27.3 to 32.1 weeks (p=0.01). Statistical modelling using restricted cubic splines confirmed these trends in the data. These results show that whilst there may have been a slight improvement in the size at diagnosis of Soft Tissue Sarcomas, overall most patients still experience a long delay between the onset of symptoms and diagnosis and commencement of treatment. It is difficult to conclude that the early diagnosis guidance produced in 1999 has had a significant effect on the basis of this study. Strategies to improve awareness of the symptoms and clinical features of Bone and Soft Tissue Sarcomas are still urgently required.
The percentage of adolescents and young adults with sarcoma enrolled in multicenter clinical trials is reportedly much lower than that of younger children. We intended to determine if this remained true despite the availability of a study open to patients up to the age of 40 years Review of interim recruitment and randomization rates in a large randomized multinational trial for resectable osteosarcoma, EURAMOS-1, jointly performed by COG, COSS, EOI, and SSG, within ECT-EUROCORES. Randomization scheduled after preoperative chemotherapy and definitive surgery of the primary tumour.Aim
Method
Recently a great deal of interest has emerged in new techniques for resection of bone tumours, such as the use of computer guided surgery, joint sparing prostheses and epiphysiolysis. However, all the techniques may require narrower margins at resection than the traditional Enneking wide margins. The aim of the study was to look at the effect of width and tissue at surgical margins, together with the use of adjuvant therapy on locally recurrent disease and disease free survival. The quality in terms of precise distance (mm) and tissue has been regularly reported by our pathologists for 5 years, prior to this it was reported as wide, marginal or intralesional. It is known from previous studies that 90% of locally recurrent disease for osteosarcoma and Ewings occurs within 2 years from surgery. Therefore all primary bone sarcomas of the pelvis and appendicular skeleton that underwent surgery between 1/1/2005 and 1/1/2008 were entered into the study group, giving at least 2 years follow up. The pathology records were reviewed and the quality of the margins and percentage tumour necrosis were recorded. Those patients who had suffered local or distal disease relapse or died were highlighted. Analysis was undertaken by Kaplan Meier survival curves with univariate and multivariate analysis.Introduction
Methods
Expandable prostheses were designed to allow progressive growth after tumour resection in children. The aim of this study was to report the late results of the non-invasive growing prostheses designed by A Soubeyran (Phenix prosthesis or Wright Repiphysis). From 1994 to January 2006, 27 children aged 4 to 12 (mean 8.5), underwent a resection of the knee for a bone tumour, with reconstruction by a non invasive expandable prosthesis. There were 16 boys and 11 girls. The tumours were 25 osteosarcomas and 2 Ewing tumours. All patients received pre and post-operative chemotherapy. There were 18 distal femur, 7 proximal tibia, and 2 femur + tibia resections. There were different successive designs based on the same electro-magnetic growing mechanism using a pre bent spring, released by eating in an induction coil. After, 7.2 years mean follow-up (4 months to 15 years), 20 patients had no evidence of disease and 7 were deceased. Two with a local recurrence were amputated. Mean lengthening was 5.1 centimeters (0 to 8), after 3 to 11 lengthening procedures. Mean limb-length discrepancy was 1.8 cm. Two patients had a secondary infection. Eleven had a revision for arthrofibrosis. All surviving patients were revised to a conventional hinged prosthesis. The mean MSTS functional score of the definitive prosthesis was 82% (63 to 96%). Theses prostheses showed many mechanical complications as loosening, fracture of the growing mechanism, and arthrofibrosis. The positive outcome was the possibility to perform a progressive lengthening, without surgery limiting the risk of infection. Theses prostheses should be considered as temporary until reconstruction with a conventional hinged prosthesis. Patients with multiple revisions had a tendency to show less favourable late functional results than with primary implanted hinged prosthesis.
The first osseointegrated transfemoral amputation prosthesis operation was performed in Gothenburg in 1990. The aim is improving quality of life for patients who cannot use conventional socket prosthesis. In 1999 the prospective OPRA-study (Osseointegrated Prosthesis for Rehabiliation of Amputees) was initiated with standardized surgery, equipment and rehabilitation program. The surgery consists of a two-stage procedure. At the primary surgery (S1), a titanium screw (the fixture) is inserted into the remaining diaphyseal bone. The fixture is 80 mm long with a diameter of 16–20 mm (+0.5 mm increments). The patient is hospitalized 5-7 days. At the secondary surgery (S2), six months later, an abutment is inserted into the fixture. The abutment has a hexagonal press-fitting into the fixture and is secured by an abutment screw. The patient remains hospitalized 10–12 days. Gradual increase of load and activity is initiated over a 6 month period.Aim
Method
To determine whether delayed diagnosis (lapse from initial symptoms to the beginning of treatment) has influence on the possibilities of crossing the physis by the tumour, and/or on the outcome in pediatric patients with high grade metaphyseal osteosarcoma. The clinical records, image methods and the histology reports of 157 metaphyseal paediatric osteosarcomas were reviewed. The mean follow-up time was 102 months. Location, histological subtype, time from initial symptoms to start of treatment, major diameter, % of necrosis, physis crossed by the tumour or not, and outcome (recurrence, metastases and status) were recorded in a SPSS v15.0 file.Aim
Patients and methods
Accurate and reliable patient information plays a crucial role in the multidisciplinary treatment of malignancies helping to ensure compliance of the patients and their relatives with often long-lasting and stressful treatment. The English version of the online encyclopaedia Wikipedia has been recently reported to be the prominent source of online health information. However, there is little information concerning the quality of information found in Wikipedia. We therefore created a questionnaire concerning of 20 questions asking for scope, completeness and accuracy of information found on osteosarcoma. Three independent observers tested the English version of Wikipedia as well as the patient version and the health professional version of the website of the National Cancer Institute. Answers (scores 0-3) were verified with authoritative resources and international guidelines.Aim
Method
Local recurrence after surgery of soft tissue sarcomas is dependent on surgical margins. Wide margins require large resections which may lead to impaired function or loss of limb. In some cases it may be technical impossible or even ethical unacceptable to achieve ideal surgical margins. Standard adjuvant treatment in such cases is ionising radiation, which may cause severe toxic side effects. PCI is a unique procedure for site-specific delivery of several types of membrane impermeable molecules. The technology is based on the photochemical induced cytosolic release of endocytosed macromolecules from endosomes and lysosomes into the cytosol. PCI has in this study been evaluated as an adjuvant to the surgical resection of sarcoma. Human fibrosarcom (HT1080) was transplanted to athymic mice. The photosensitizer aluminium phthalocyanine disulfonat (AlPcS2a), and bleomycin (BLM) was systemically administrated 48 hours and 30 minutes respectively prior to surgery and light exposure. After resection with intralesional margin the tumour bed was illuminated at 670 nm (15 J/cm2).Aim
Method
Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis because of late local recurrence and distant metastases. To our knowledge, no studies have minimum follow-up of 10 years that evaluate long-term outcomes for survivors. Data on 62 patients who had been treated for SS from 1968 to 1999 were studied retrospectively in a multicenter study. The following parameters were examined for their potential prognostic value: age at diagnosis, sex, tumour site and size, histology, histological grade, fusion type (SYT-SSX1 vs. SYT-SSX2), and surgical margin status. Mean follow-up of living patients was 17.2 years, and of dead patients 7.7 years.Aim
Method
Purpose of this study was to review a single Institution experience and results of management of extraskeletal osteosarcoma (OGS), with emphasis on the role of combined treatment consisting of surgery and adjuvant chemotherapy. Retrospective study of 48 patients observed 1966- 2007 was undertaken: 36 patients were managed at our Institution while 12 cases were consultations and not included in this study. Clinico-pathologic features and details of treatment of all 36 patients were correlated with outcome. Updated follow-up was available in all patients.Aim
Method
Chondrosarcomas are malignant hyaline cartilage tumours of bone. They are clinically resistant to conventional chemo- and radiotherapy and the underlying mechanism is poorly studied. Chemoresistance is a multifactorial process and the inaccessibility due to abundant hyaline cartilaginous matrix surrounding the cells, presence of multi-drug resistance pumps, and expression of anti-apoptotic proteins such as BCL2, have been suggested. Our aim was to study chemoresistance mechanisms in chondrosarcoma. We first studied the sensitivity of chondrosarcoma cell lines (SW1353, CH2879, JJ012, OUMS27) and 2 primary cultures for doxorubicin and cisplatin. We used a 3D pellet model of CH2879 to study doxorubicin incorporation. To investigate whether chondrosarcoma cells could be resensitised to chemotherapy we tested the BH3 mimetic ABT737 inhibiting anti-apoptotic BCL2 family proteins. Cell viability was assessed using a WST assay for mitochondrial activity. Dose response curves showed that chondrosarcoma cell lines and cultures are partially resistant to doxorubicin, while primary cultures were completely resistant to cisplatin. In 3D cell pellets, with morphology strongly resembling high grade chondrosarcoma, doxorubicin incorporation was confirmed. Chondrosarcoma cells responded to ABT737 with a >60% reduction in cell viability at high concentrations (25μM). Combination treatment allowing 2 days between ABT737 and chemotherapy addition led to a complete reduction of cell viability in all cell cultures. In conclusion, chondrosarcoma cell lines show a partial response to doxorubicin and less response to cisplatin. The incorporation of doxorubicin in the cells in a 3D pellet model indicates that resistance is not caused by inaccessibility of the cells for the drugs nor by multi-drug resistance pump activity. By combining BCL2 inhibition with Doxorubicin treatment, a complete reduction of cell viability was obtained. This suggests that BCL2 overexpression plays an important role in chemoresistance of chondrosarcoma, and turning on the apoptotic machinery by BCL 2 inhibition can render them chemosensitive.
Cryosurgery is a well established modality in the treatment of benign aggressive and low grade malignant tumours. In this setting it allows for intra-lesional resection and preservation of function without compromising oncological outcome. Here we present the outcome of 87 patients treated with cryosurgery for low-grade chondrosarcoma of bone. 87 patients were treated between 1988 and 2005. The mean age was 51 years (range, 8-77 years), and included 47 females and 40 males. Minimal follow-up was two years. Patients were treated for lesions of the distal femur (n = 30), proximal humerus (n = 33), proximal femur (n = 5), proximal tibia (n = 10), and the remaining sites included the iliac bone, distal tibia, forearm, carpal and tarsal bones (n=10). Patients were treated with intralesional curettage through a cortical window, adjuvant burr drilling, cryotherpay and reconstruction with cement or bone graft and hardware fixation when that was clinically indicated.Background
Materials and methods
Bumps and lumps of the hand are a common cause for consultation in general practice. However not all of these lesions are of true neoplastic nature and malignant tumours are a rarity in this location. The records of all tumours of the hand and wrist treated surgically at our institution in the period 1994 to 2009 were reviewed. Because of their non-neoplastic nature typical lesions of the hand such as ganglion cysts or palmar fibromatosis and the like were not included in this study. Histological entity, location, radiographic and clinical findings were analysed; malignant tumours were followed up by X-ray and MRI.Aim
Method
To present our experience and results of percutaneus sclerotherapy of aneurysmal bone cysts (ABC). All patients from January 2007 to September 2009, where radiology and cytology were consistent with ABC (n=20), were treated with repeated injections of 30 mg/ml polidocanol. The mean age was 16 (5-27) years. There were 13 lesions in the long bones, 2 in the pelvic bones, 2 in the sacrum, 2 in the foot, and 1 in a finger. The sclerotherapy was performed under fluoroscopic or CT guidance and under local or general anesthesia. Each injection consisted of 2 mg polidocanol per kg body weight. Three injections with an interval of 4 weeks were most commonly scheduled. Radiological assessment was performed regularly after the last injection. Injections were continued if the lesion had not healed.Aim
Method
The recent discovery of small non-coding RNAs, so-called micro RNAs (miRNAs), has provided new insights into cancer diagnosis. Several studies have shown that profiles of miRNA expression differ between normal tissue and tumour tissue and vary among tumour types. To exploit this difference, we evaluated the feasibility of using muscle-specific miRNAs (miR-1, 133a, 133b, 206) as biomarkers of rhabdomyosarcoma (RMS). Total RNA was extracted from 16 cell lines (7 RMS, 4 neuroblastoma, 3 Ewing sarcoma and 2 malignant rhabdoid tumour) and 21 tumour specimens (7 RMS, 1 Ewing sarcoma, 4 undifferentiated sarcoma, 1 osteosarcoma, 1 alveolar soft part sarcoma, 2 neuroblastoma, 2 Wilms tumour, 1 malignant rhabdoid tumour, 1 adrenal carcinoma and 1 retinoblastoma). miRNA was quantified by real-time RT-PCR. The expression levels of miRNAs were calculated utilizing the delta-delta Ct method, normalised to the level of miR-16, and compared using the Mann-Whitney U test.Aim
Method
Retroperitoneal sarcomas (RS) are rare tumours that may reach considerable size at diagnosis and should optimally be treated by a specialized multidisciplinary team. In Sweden, we have since 10 years enforced referral of RS to sarcoma centers, but have experienced a considerable delay, which provides the basis for this study on delays in RS diagnosis and treatment. We identified 33 patients treated for RS at the Southern Sweden Sarcoma Center (covering a population of 1.5 million), Lund University Hospital between 2003-2009. Data, including onset of symptoms, time to diagnosis and time to treatment were recorded from clinical files. Patient's delay was defined as the time from onset of symptoms to the first visit to a doctor, which could be a general practitioner or a specialist. Doctor's delay was defined as the time from the first visit to a doctor to the start of treatment, which was in most cases surgery. In total, 30 patients were referred to the sarcoma centre for treatment. Complete data are available from 25 patients (13 men) with a median age of 62 (20-86) years. Median patient's delay was 15 days (0-9 months) and median doctor's delay was 97 days (0-40 months). Median doctor's delay was indeed somewhat longer (52 days) at the sarcoma centre than at the local hospitals (38 days). Some of the longest delays were caused by primary erroneous diagnosis (16 and 40 months) and comorbidity (4, 8 and 19 months) that required other medical procedures before surgery. Though almost all patients with RS in Southern Sweden are referred to a sarcoma centre for treatment, delays are considerable for many patients with doctor's delays outnumbering patient's delays. Our findings demonstrate that centralisation per se is not sufficient to treat RS, but that optimized diagnostics and clinical management is needed also at sarcoma centers.
Grade is the most important predictor of the biological behaviour of soft tissue sarcomas. Assigning a pathologic grade is always a difficult task as discordance rate is 30-40% even among experienced sarcoma pathologists. Many of these tumours are heterogeneously large and only small fractions are sampled for biopsy. This emphasizes the need for an objective and accurate assessment of histology. Our aim is to evaluate the role of Choline as a tumour marker in (i) differentiating benign from malignant soft tissue tumour, (ii) to distinguish recurrent/residual tumours using in-vivo MR spectroscopy. PMRS Study was performed at 1.5Tesla MRI machine of the lesions in 25 patients. Single-voxel (SVS) study has been done in 10 cases and chemical shift imaging (CSI) study characterised the heterogeneity of the tumour in 15 cases by using point – resolved spectroscopic sequence (PRESS) with echo time TR=2000/TE = 30, 135 & 270 msec. The choline peak, identified at 3.2 ppm in spectra was considered significant. MRS results and histopathologic findings were correlated and P < 0.001, considered being significant.Aim
Methods
The aim of this study was to analyze our results using a modular endoprosthetic replacement system (MUTARS) for bone tumours of the proximal humerus. Thirty-nine patients were treated with a MUTARS endoprosthesis of the proximal humerus. Mean follow-up was 38 months (3-138 months). Most operations were necessitated by metastasis (n=30); surgery for a primary tumour (n=9) was less frequent. The Enneking score and the active ranges of motion for shoulder flexion, abduction, and external rotation were recorded. Complete refixation of the rotator cuff was possible in 23 cases. Radiographs also were obtained.Aim
Methods
The saddle prosthesis was originally developed for reconstruction of large acetabular defects in hip revision arthroplasty. Later on the saddle prosthesis was also used for hip reconstruction after resection of peri-acetabular tumours. In case of patient survival a long-term good hip function is required of the saddle prosthesis. The goal of this study is the measurement of long-term clinical results of saddle prosthesis after reconstruction of peri-acetabular tumours. Between 1987 and 2003 a total of 17 patients were treated in the Leiden University Medical Center with saddle prosthesis after resection of peri-acetabular tumours (12 chondrosarcoma, 3 osteosarcoma, 1 malignant fibrous histiocytoma, 1 metastasis). 11 of the 17 patients died, the mean survival was 37 months (range 2-59 months), and 6 patients were still alive (follow-up 12.1 year, range 8.3–16.8 year). The outcome was measured with the SF-36 questionnaire, the Toronto Extrimity Salvage Score (TESS) and the Musculo Skeletal Tumour Society (MSTS) score. In 1 patient the saddle prosthesis was removed already after 3 months because of luxation and infection.Aim
Method
To report late development of sarcomas on sites of previously curetted and grafted benign tumours. Rare cases of development of sarcomas in sites of previous benign lesions are documented, and the development is generally considered secondary to progression of benign lesions, even without radiotherapy. In our files, 12 cases curetted and grafted, without radiotherapy addition developed sarcomas from 6 to 28 years from curettage (median 18). Age at first diagnosis (9 GCT, 1 benign fibrous histiocytoma, ABC and solitary bone cyst) ranged from 13 to 55 (median 30). For all cases radiographic and clinic documentation was available. Histology was available for 7 of the benign lesions and for all malignant lesions. The type of bone used to fill cavities was autoplastic in 4 cases, homoplastic in 2 cases, homoplastic and tricalciumphosphate/hydrossiapatite in 1 case, autoplastic and homoplastic in 1 cases, heteroplastic in 1 case. In 3 cases the origin was not reported. Secondary sarcomas, all high grade, were 8 OS, 3 MFH, and 1 fibrosarcoma.Aim
Methods and Results
The pelvis has always been a difficult area for surgeons, with high complication rates from surgery and the perception of poor oncological outcomes. The aim of the study was to look at the surgical and oncological outcomes of pelvic tumours treated at our centre. From the 3100 primary bone tumours seen at the ROH. Information was retrieved on 539 patients seen with a primary bone tumour of the pelvis. The demographic details, oncological and surgical outcomes were reviewed.Introduction
Methods
We present the greatest study of patients with proximal fibula resection. Moreover we describe a new classification system for tumour resection of the proximal fibula independent of the tumour dignity. In 57 patients the functional and clinical outcome was evaluated. The follow up ranged between 6 months and 22.2 years (median 7.2 years). Indicationfor surgery was in 10 cases benign tumours and in 47 cases malignant tumours. In 32 patients a resection of the peroneal with resulting peroneal palsy was necessary.Aim
Method
Grade is the most important predictor of the biological behaviour of soft tissue sarcomas. Assigning a pathologic grade is always a difficult task as discordance rate is 30-40% even among experienced sarcoma pathologists. Many of these tumours are heterogeneously large and only small fractions are sampled for biopsy. This emphasizes the need for an objective and accurate assessment of histology. Our aim is to evaluate the role of Choline as a tumour marker in (i) differentiating benign from malignant soft tissue tumour, (ii) to distinguish recurrent/residual tumours using in-vivo MR spectroscopy. PMRS Study was performed at 1.5Tesla MRI machine of the lesions in 25 patients. Single-voxel (SVS) study has been done in 10 cases and chemical shift imaging (CSI) study characterised the heterogeneity of the tumour in 15 cases by using point – resolved spectroscopic sequence (PRESS) with echo time TR=2000/TE = 30, 135 & 270 msec. The choline peak, identified at 3.2 ppm in spectra was considered significant. MRS results and histopathologic findings were correlated and P < 0.001, considered being significant.Aim
Methods
It is common practice nowadays to treat patients with metastatic epidural spinal cord compression (MESCC) surgically. Extend and type of surgery should be in proper relation to the expected survival time of the patient. It is still difficult to predict patient's survival time and different scoring systems are used. Reliable prediction of survival is mandatory, in that way adjustable surgical treatment can be established. Evaluating potential prognostic factors for survival after surgery for MESCC.Background
Aim
Assess symptoms and diagnostic problems of chest wall chondrosarcoma and factors related to long doctor's delay. The material included all 106 consecutive patients with chondrosarcoma of the chest wall diagnosed in Sweden 1980-2002. Pathological specimens were re-evaluated and graded by the Scandinavian Sarcoma Group pathology board. Files from the very first medical visit for symptoms related to the chondrosarcoma were traced and used to characterise the initial symptoms, calculate patient's and doctor's delay and to identify factors contributing to the delaysAim
Methods
Bone lesions in Ewing's sarcoma (ES/PNET) have been traditionally diagnosed with bone Scan. PET-scan is emerging as a promising investigative modality for detection of metastatic lesions. In this prospective study, we compare the utility of both to detect the metastatic sites. One hundred and seventy five histologically proven cases of ESPNET from 2004-2009 were prospectively staged with bone scan and PET-scan with Breath- hold CT scan- thorax. The diagnostic value of PET-scan to pick up metastatic lesions was compared with bone scan.Background
Methods
Local treatment of Ewing sarcoma of the hip bones and sacrum remains one of the most difficult tasks in the treatment of bone sarcomas. We investigated the difference between size, local treatment and overall survival in Ewing sarcoma of the sacrum and hip bones. Patients with Ewing sarcoma of the hip bones or sacrum, diagnosed between 1986 and 2009, were identified through the Scandinavian Sarcoma Group registry. Data regarding tumour size, local treatment (radiation or surgery), local recurrence, surgical margin, metastatic disease, and overall survival were analyzed and compared between the two locations (hip bone or sacrum).Aim
Methods
To evaluate outcome and complications of knee arthrodesis with a modular prosthetic system (MUTARS(r) Implantcast), as primary and revision implants in musculoskeletal oncology. Between 1975 and 2009, 24 prostheses were used for knee arthrodesis. Nineteen in oncologic cases: 6 osteosarcomas, chondrosarcoma, synovial sarcoma and metastatic carcinoma 3 each, 2 pigmented villonodular synovitis (PVNS), malignant fibrous hystiocitoma and giant cell tumour 1 each. Patients were grouped into: A) primary implants, B) revision implants. Group A included 9 patients: 8 arthrodeses after extra-articular resection with major soft tissue removal, 1 after primary resection following multiple excisions of locally recurrent PVNS. Group B included 15 patients: 12 arthrodeses for infection (5 infected TKAs, 7 infected megaprostheses), 2 for failures of temporary arthodesis with Kuntscher nail and cement, 1 for recurrent chondrosarcoma in previous arthrodesis.Aim
Method
To compare the functional outcome of proximal femoral reconstruction using endoprosthetic replacement and hip arthrodesis using a vascularised fibular graft The study included thirty-five patients who had proximal femoral reconstruction following resection of a malignant bone tumour. Patients were divided into 2 groups according to the reconstructive modality used. Group 1 (15 patients) reconstructed by hip arthrodesis using a vascularised fibular graft. Group 2 (20 patients) reconstructed by endoprosthesis. The mean age of group I was 14.9 years (range, 7-25). 8 patients had Ewing's sarcoma, 5 osteogenic sarcoma, and 2 chondrosarcoma. In group 2, the mean age was 35 years (range, 14-61). Eight patients had osteogenic sarcoma, 2 chondrosarcoma, 2 Ewing's sarcoma, 1 lymphoma, 1 MFH, 1 synovial sarcoma, 1 parosteal osteosarcoma, and 4 metastatic carcinomas.Aim
Material and Methods
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. 313 patients diagnosed with primary osteosarcomas 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, extra-compartmental stage and tumour size ≥5cm. 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). 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.
To compare two consecutive treatment programs in metastatic osteosarcoma at presentation. Between 1987 and 1999 treatment of stage IIIB osteosarcoma included induction chemotherapy (CHT) which consisted in 3-5 cycles of intra-arterial doxorubicin (DOX) 75 mg/m2 or cis-platin (CDDP) 120 mg/m2, surgery or definitive local radiation therapy (RT) for primary and adjuvant CHT with DOX, CDDP or HDMTX. Thirty nine patients were included in this protocol. Since 2000 a more intensive induction CHT protocol was adopted. It consisted in 4 cycles of DOX 90 mg/m2 +CDDP 120 mg/m2 (24 patients) or DOX 60 mg/m2 +CDDP 120 mg/m2+Ifosfamide (IFO) 6 g/m2 (7 patients). Adjuvant chemotherapy included DOX, CDDP, IFO or VP-16. Residual lung deposits were removed at the end of adjuvant treatment.Aim
Methods
Ten years ago at the EMSOS 2000 meeting we have presented our experience concerning the non-surgical treatment of stage IIB osteosarcoma of extremities. The purpose of study was to evaluate long-term results and complications related to this non-standard and controversial treatment modality. Since 1988 definitive radiation gamma therapy (RT) in the median dose of 60 Gy was administrated to 36 patients who refused surgery after preoperative chemotherapy. Until 1999 induction consisted in 3-5 cycles of intra-arterial doxorubicin (DOX) 75 mg/m2 or cis-platin (CDDP) 120 mg/m2. Since 2000 induction comprised 4 cycles of DOX 90 mg/m2+CDDP 120 mg/m2 intra-arterially. Tumour response before local treatment was assessed non-invasively by means of X-ray, CT, MRI, angiography, 3-phase bone scans and alkaline phosphatase measurement. The end-points were overall (OS), metastases free (MFS) and local progression free (LPFS) survival at 10 years.Aim
Methods
Bone tumours rarely involve the joint surface as cartilage is thought to be a good barrier to tumour spread. When the tumour does cross the surface the surgeon is faced with the dilemma of whether to amputate the limb, resect it without reconstruction or reconstruct with an implant. This paper aims to investigate the oncological and functional outcomes of patients undergoing an extra-articular resection and reconstruction with an endoprosthesis. 3100 patients have been seen in ROH with primary bone tumours. Patients were identified who had an extra-articular resection considered pre-operatively and the notes and imaging was reviewed. This group was subdivided into a group who did have an extra-articular resection (EAR) and those who either had an amputation or traditional through joint resection. The outcomes of the three groups (group 1 = no joint involvement, group 2 = EAR considered but not done and group 3 = EAR) were then compared in terms of oncological outcome, surgical margins and complications.Introduction
Method
In skeletally immature patients, resection of
bone tumours and reconstruction of the lower limb often results
in leg-length discrepancy. The Stanmore non-invasive extendible
endoprosthesis, which uses electromagnetic induction, allows post-operative
lengthening without anaesthesia. Between 2002 and 2009, 55 children
with a mean age of 11.4 years (5 to 16) underwent reconstruction
with this prosthesis; ten patients (18.2%) died of disseminated
disease and one child underwent amputation due to infection. We
reviewed 44 patients after a mean follow-up of 41.2 months (22 to
104). The mean Musculoskeletal Tumor Society score was 24.7 (8 to
30) and the Toronto Extremity Salvage score was 92.3% (55.2% to
99.0%). There was no local recurrence of tumour. Complications developed
in 16 patients (29.1%) and ten (18.2%) underwent revision. The mean length gained per patient was 38.6 mm (3.5 to 161.5),
requiring a mean of 11.3 extensions (1 to 40), and ten component
exchanges were performed in nine patients (16.4%) after attaining
the maximum lengthening capacity of the implant. There were 11 patients
(20%) who were skeletally mature at follow-up, ten of whom had equal
leg lengths and nine had a full range of movement of the hip and
knee. This is the largest reported series using non-invasive extendible
endoprostheses after excision of primary bone tumours in skeletally
immature patients. The technique produces a good functional outcome,
with prevention of limb-length discrepancy at skeletal maturity.
There are eight reported cases in the literature
of osteosarcomas secreting β-hCG. Our primary aim was to investigate
the rate of β-hCG expression in osteosarcoma and attempt to understand
the characteristics of osteosarcomas that secrete β-hCG. We reviewed
37 histopathology slides (14 biopsies and 23 surgical specimens) from
32 patients with osteosarcoma. The slides were retrospectively stained
for β-hCG expression. Patient and tumour characteristics, including
age, gender, tumour location, subtype, proportion of necrosis, presence
of metastases and recurrence were recorded. A total of five of the
32 tumours were found to be positive for β-hCG expression (one strongly
and four weakly). This incidence of this expression was found in
tumours with poor histological response to neoadjuvant chemotherapy. The use of β-hCG expression as a diagnostic, prognostic or follow-up
marker is questionable and needs further investigation with a larger
sample size.
We reviewed our initial seven-year experience
with a non-invasive extendible prosthesis in 34 children with primary bone
tumours. The distal femur was replaced in 25 cases, total femur
in five, proximal femur in one and proximal tibia in three. The
mean follow-up was 44 months (15 to 86) and 27 patients (79%) remain
alive. The prostheses were lengthened by an electromagnetic induction
mechanism in an outpatient setting and a mean extension of 32 mm
(4 to 80) was achieved without anaesthesia. There were lengthening
complications in two children: failed lengthening in one and the
formation of scar tissue in the other. Deep infection developed
in six patients (18%) and local recurrence in three. A total of
11 patients required further surgery to the leg. Amputation was
necessary in five patients (20%) and a two-stage revision in another.
There were no cases of loosening, but two patients had implant breakage
and required revision. The mean Musculoskeletal Tumor Society functional
score was 85% (60% to 100%) at last known follow-up. These early
results demonstrate that the non-invasive extendible prosthesis
allows successful lengthening without surgical intervention, but
the high incidence of infection is a cause for concern.
The aim of this study was to define the treatment
criteria for patients with recurrent chondrosarcoma. We reviewed the
data of 77 patients to examine the influence of factors such as
the intention of treatment (curative/palliative), extent of surgery,
resection margins, status of disease at the time of local recurrence
and the grade of the tumour. A total of 70 patients underwent surgery
for recurrent chondrosarcoma. In seven patients surgery was not
a viable option. Metastatic disease occurred in 41 patients, appearing
synchronously with the local recurrence in 56% of cases. For patients
without metastasis at the time of local recurrence, the overall
survival at a mean follow-up after recurrence of 67 months (0 to
289) was 74% (5 of 27) compared with 19% (13 of 50) for patients
with metastasis at or before the development of the recurrence.
Neither the type/extent of surgery, site of tumour, nor the resection margins
for the recurrent tumour significantly influenced the overall survival. With limited survival for patients with metastatic disease at
the time of local recurrence (0% for patients with grade III and
de-differentiated chondrosarcoma), palliative treatment, including
local radiation therapy and debulking procedures, should be discussed
with the patients to avoid long hospitalisation and functional deficits. For
patients without metastasis at the time of local recurrence, the
overall survival of 74% justifies an aggressive approach including
wide resection margins and extensive reconstruction.
We aimed to identify the incidence, outcome and prognostic factors associated with spindle cell sarcomas of bone (SCSB). We studied 196 patients with a primary non-metastatic tumour treated with the intent to cure. The results were compared with those of osteosarcoma patients treated at our hospital during the same period. The overall incidence of SCSB was 7.8% of all patients with a primary bone sarcoma. The five- and ten-year survival rates were 67.0% and 60.0%, respectively, which were better than those of patients with osteosarcoma treated over the same period. All histological subtypes had similar outcomes. On univariate analysis, factors that were significantly associated with decreased survival were age >
40 years, size >
8 cm, the presence of a pathological fracture, amputation, involved margins and a poor response to pre-operative chemotherapy. Multivariate analyses showed that age >
65 years, amputation and involved margins were all statistically significant prognostic factors. Involved margins and poor response to pre-operative chemotherapy were associated with an increased risk of local recurrence. SCSB has a better prognosis than osteosarcoma when matched for age. Most prognostic factors for osteosarcoma also seem to apply to SCSB. Patients with SCSB should be treated in the same way as patients of the same age with osteosarcoma.
We retrospectively studied 14 patients with proximal and diaphyseal tumours and disappearing bone (Gorham’s) disease of the humerus treated with wide resection and reconstruction using an allograft-resurfacing composite (ARC). There were ten women and four men, with a mean age of 35 years (8 to 69). At a mean follow-up of 25 months (10 to 89), two patients had a fracture of the allograft. In one of these it was revised with a similar ARC and in the other with an intercalary prosthesis. A further patient had an infection and a fracture of the allograft that was revised with a megaprosthesis. In all patients with an ARC, healing of the ARC-host bone interface was observed. One patient had failure of the locking mechanism of the total elbow replacement. The mean post-operative Musculoskeletal Tumor Society score for the upper extremity was 77% (46.7% to 86.7%), which represents good and excellent results; one patient had a poor result (46.7%). In the short term ARC effectively relieves pain and restores shoulder function in patients with wide resection of the proximal humerus. Fracture and infection remain significant complications.
The best method of reconstruction after resection of malignant tumours of the tibial diaphysis is unknown. In the absence of any long-term studies analysing the results of intercalary endoprosthetic replacement, we present a retrospective review of 18 patients who underwent limb salvage using a tibial diaphyseal endoprosthetic replacement following excision of a malignant bone tumour. There were ten men and eight women with a mean age of 42.5 years (16 to 76). Mean follow-up was 58.5 months (20 to 141) for all patients and 69.3 months (20 to 141) for the 12 patients still alive. Cumulative patient survival was 59% (95% confidence interval (CI) 32 to 84) at five years. Implant survival was 63% (95% CI 35 to 90) at ten years. Four patients required revision to a proximal tibial replacement at a mean follow-up of 29 months (10 to 54). Complications included metastases in five patients, aseptic loosening in four, peri-prosthetic fracture in two, infection in one and local recurrence in one. The mean Musculoskeletal Tumor Society score and the mean Toronto Extremity Salvage Score were 23 (17 to 28) and 74% (53 to 91), respectively. Although rates of complication and revision were high, custom-made tibial diaphyseal replacement following resection of malignant bone tumours enables early return to function and provides an attractive alternative to other surgical options, without apparent compromise of patient survival.
We set out to determine the impact of surgery on quality of life and function in patients who had undergone surgery for symptomatic peri-acetabular metastases. From a prospective database we retrospectively reviewed 46 consecutive patients who had been treated operatively between June 2003 and June 2009. The mean age of the patients was 56.4 years (20 to 73) and the mean post-operative follow-up was 19.2 months (4 to 70). Functional evaluation and quality-of-life assessments were performed. At the most recent follow-up, 26 patients (56.5%) were alive. Their median survival time was 25.0 months. Ten major postoperative complications had occurred in eight patients (17.4%). The mean post-operative Musculoskeletal Tumor Society score (MSTS 93) was 56.3% (6.7% to 90.0%). Improvement in the Eastern Cooperative Oncology Group (ECOG) performance status was seen in 32 patients (69.6%). On the European Organisation for Research and Treatment of Cancer core quality-of-life questionnaire (QLQ-C30) measure of global health status there was a statistically significant improvement from the patients’ pre-operative status (42.8 ( Surgery for patients with peri-acetabular metastases reduces pain and improves their quality of life, and has a low rate of surgical complications.
We describe 22 cases of bizarre parosteal osteochondromatous proliferation, or Nora’s lesion. These are surface-based osteocartilaginous lesions typically affecting the hands and feet. All patients were identified from the records of a regional bone tumour unit and were treated between 1985 and 2009. Nine lesions involved the metacarpals, seven the metatarsals, one originated from a sesamoid bone of the foot and five from long bones (radius, ulna, tibia, and femur in two). The mean age of the patients was 31.8 years (6 to 66), with 14 men and eight women. Diagnosis was based on the radiological and histological features. The initial surgical treatment was excision in 21 cases and amputation of a toe in one. The mean follow-up was for 32 months (12 to 162). Recurrence occurred in six patients (27.3%), with a mean time to recurrence of 49 months (10 to 120). Two of the eight patients with complete resection margins developed a recurrence (25.0%), compared with four of 14 with a marginal or incomplete resection (28.6%). Given the potential surgical morbidity inherent in resection, our data suggest that there may be a role for a relatively tissue-conserving approach to the excision of these lesions.
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.
We investigated the eventual diagnosis in patients referred to a tertiary centre with a possible diagnosis of a primary bone malignancy. We reviewed our database from between 1986 and 2010, during which time 5922 patients referred with a suspicious bone lesion had a confirmed diagnosis. This included bone sarcoma in 2205 patients (37%), benign bone tumour in 1309 (22%), orthopaedic conditions in 992 (17%), metastatic disease in 533 (9%), infection in 289 (5%) and haematological disease in 303 (5%). There was a similar frequency of all diagnoses at different ages except for metastatic disease. Only 0.6% of patients (17 of 2913) under the age of 35 years had metastatic disease compared with 17.1% (516 of 3009) of those over 35 years (p <
0.0001). Of the 17 patients under 35 years with metastatic disease, only four presented with an isolated lesion, had no past history of cancer and were systematically well. Patients under the age of 35 years should have suitable focal imaging (plain radiography, CT or MRI) and simple systemic studies (blood tests and chest radiography). Reduction of the time to biopsy can be achieved by avoiding an unnecessary investigation for a primary tumour to rule out metastatic disease.
Endoprosthetic replacement of the pelvis is one of the most challenging types of limb-salvage surgery, with a high rate of complications. In an attempt to reduce this and build greater versatility into the reconstruction process, a new type of pelvic endoprosthesis was developed in 2003, based on the old McKee-Farrar prosthesis. This study reviews the outcomes in 27 patients who had an ice-cream cone pelvic prosthesis inserted at two different specialist bone tumour centres in the United Kingdom over the past six years. The indications for treatment included primary bone tumours in 19 patients and metastatic disease in two, and six implants were inserted following failure of a previous pelvic reconstruction. Most of the patients had a P2+P3 resection as classified by Enneking, and most had resection of the ilium above the sciatic notch. The mean age of the patients at operation was 49 years (13 to 81). Complications occurred in ten patients (37.0%), of which dislocation was the most common, affecting four patients (14.8%). A total of three patients (11.1%) developed a deep infection around the prosthesis but all were successfully controlled by early intervention and two patients (7.4%) developed a local recurrence, at the same time as widespread metastases appeared. In one patient the prosthesis was removed for severe pain. This method of treatment is still associated with high morbidity, but early results are promising. Complications are diminishing with increasing experience.
The use of allografts for the treatment of bone tumours in children is limited by nonunion and the difficulty of finding a suitable graft. Furthermore, appositional growth can’t be expected of an allograft. We used an overlapping allograft in 11 children, with a mean age of ten years (4 to 15), with a mean follow-up of 24.1 months (20 to 33). There were five intercalary and six intra-articular resections, and the tumours were in the femur in six cases and the humerus in five. Rates of union, times to union, remodelling patterns and allograft-associated complications were evaluated. No allograft was removed due to a complication. Of the 16 junctional sites, 15 (94%) showed union at a mean of 3.1 months (2 to 5). Remodelling between host and allograft was seen at 14 junctions at a mean of five months (4 to 7). The mean Musculoskeletal Tumor Society score was 26.5 of 30 (88.3%). One case of nonunion and another with screw protrusion required re-operation. Overlapping allografts have the potential to shorten time to union, decrease rates of nonunion and have positive appositional growth effect.
The aim of this study was to identify whether there was any difference in patient, tumour, treatment or outcome characteristics between patients with skeletal or extra-skeletal Ewing’s sarcoma. We identified 300 patients with new primary Ewing’s sarcoma diagnosed between 1980 and 2005 from the centres’ local database. There were 253 (84%) with skeletal and 47 (16%) with extra-skeletal Ewing’s sarcomas. Although patients with skeletal Ewing’s were younger (mean age 16.8 years) than those with extra-skeletal Ewing’s sarcoma (mean age 27.5 years), there was little difference between the groups in terms of tumour stage or treatment. Nearly all the patients were treated with chemotherapy and most had surgery. There was no difference in the overall survival of patients with skeletal (64%) and extra-skeletal Ewing’s sarcoma (61%) (p = 0.85), and this was also the case when both groups were split by whether they had metastases or not. This large series has shown that the oncological outcomes of Ewing’s sarcoma are related to tumour characteristics and patient age, and not determined by whether they arise in bone or soft tissue.
Between 1997 and 2007, 68 consecutive patients underwent replacement of the proximal humerus for tumour using a fixed-fulcrum massive endoprosthesis. Their mean age was 46 years (7 to 87). Ten patients were lost to follow-up and 16 patients died. The 42 surviving patients were assessed using the Musculoskeletal Tumor Society (MSTS) Score and the Toronto Extremity Salvage Score (TESS) at a mean follow-up of five years and 11 months (one year to ten years and nine months). The mean MSTS score was 72.3% (53.3% to 100%) and the mean TESS was 77.2% (58.6% to 100%). Four of 42 patients received a new constrained humeral liner to reduce the risk of dislocation. This subgroup had a mean MSTS score of 77.7% and a mean TESS of 80.0%. The dislocation rate for the original prosthesis was 25.9; none of the patients with the new liner had a dislocation at a mean of 14.5 months (12 to 18). Endoprosthetic replacement for tumours of the proximal humerus using this prosthesis is a reliable operation yielding good results without the documented problems of unconstrained prostheses. The performance of this prosthesis is expected to improve further with a new constrained humeral liner, which reduces the risk of dislocation.
We have reviewed five adult patients treated with endoprosthetic reconstruction of the proximal radius following resection of non-traumatic lesions. The patients had a mean age of 33.4 years (20 to 60) at the time of surgery and the mean follow-up was 7.6 years (0.8 to 16). Following surgery, all elbows were clinically stable and there was 100% survivorship of the prosthesis. Evaluation of function was assessed clinically and by the Mayo Elbow Performance Score, achieving a mean of 86% (70 to 100). Results at medium-term follow-up are encouraging with regards to elbow stability, implant survivorship and functional outcome.
Several techniques have been described to reconstruct a mobile wrist joint after resection of the distal radius for tumour. We reviewed our experience of using an osteo-articular allograft to do this in 17 patients with a mean follow-up of 58.9 months (28 to 119). The mean range of movement at the wrist was 56° flexion, 58° extension, 84° supination and 80° pronation. The mean ISOLS-MSTS score was 86% (63% to 97%) and the mean patient-rated wrist evaluation score was 16.5 (3 to 34). There was no local recurrence or distant metastases. The procedure failed in one patient with a fracture of the graft and an arthrodesis was finally required. Union was achieved at the host-graft interface in all except two cases. No patient reported more than modest non-disabling pain and six reported no pain at all. Radiographs showed early degenerative changes at the radiocarpal joint in every patient. A functional pain-free wrist can be restored with an osteo-articular allograft after resection of the distal radius for bone tumour, thereby avoiding the donor site morbidity associated with an autograft. These results may deteriorate with time.
