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1.
Br J Cancer ; 118(12): 1682, 2018 06.
Article in English | MEDLINE | ID: mdl-29808016

ABSTRACT

Since the publication of this paper, the authors noticed an error in Fig. 1. The X-axis on all the figure panels should read 'Time (years)', not 'Time (months)'. The corrected Fig. 1 is shown below.

2.
Br J Surg ; 105(4): 401-409, 2018 03.
Article in English | MEDLINE | ID: mdl-29405251

ABSTRACT

BACKGROUND: Radiation-associated angiosarcoma of the breast (RAAS) is a rare complication of adjuvant radiotherapy associated with poor survival. The British Sarcoma Group guidelines recommend that all angiosarcomas are referred to a sarcoma multidisciplinary team, although there is no recommendation that patients are managed within a sarcoma service. The aims of this study were to compare survival, complete excision rates and local recurrence rates of patients managed within a sarcoma service and those managed within local hospitals. METHODS: All patients with RAAS referred to a regional sarcoma service between 1998 and 2015 were identified from prospective databases. Patient records, and radiology, pathology and operation notes were reviewed retrospectively. RESULTS: Thirty-six patients were operated on with curative intent; 26 were managed by the sarcoma service (of whom 21 underwent radical excision of the irradiated field followed by chest wall reconstruction) and ten were managed locally. Median age was 69·5 (range 43-85) years. Disease-specific survival was significantly longer in patients managed by the sarcoma service than in those managed locally: median 91·1 (range 69·2-113·0) versus 48·8 (18·6-79·1) months respectively (P = 0·012). Overall survival rates were similar (P = 0·112). There was no difference in complete excision rate (18 of 26 in sarcoma service versus 5 of 10 in local services; P = 0·456), although the local recurrence rate was significantly lower among patients managed by the sarcoma service (9 of 26 versus 8 of 10; P = 0·015). CONCLUSION: Specialist management of RAAS leads to fewer local recurrences and improved disease-specific survival. Early referral and management within specialist units is recommended.


Subject(s)
Breast Neoplasms/mortality , Breast Neoplasms/surgery , Hemangiosarcoma/mortality , Hemangiosarcoma/surgery , Mastectomy , Neoplasms, Radiation-Induced/mortality , Neoplasms, Radiation-Induced/surgery , Radiotherapy, Adjuvant/adverse effects , Surgical Oncology , Adult , Aged , Aged, 80 and over , Breast Neoplasms/etiology , Female , Follow-Up Studies , Hemangiosarcoma/etiology , Humans , Middle Aged , Neoplasm Recurrence, Local/epidemiology , Neoplasms, Radiation-Induced/etiology , Referral and Consultation , Retrospective Studies , Survival Analysis , Treatment Outcome
3.
Br J Cancer ; 117(3): 326-331, 2017 Jul 25.
Article in English | MEDLINE | ID: mdl-28654633

ABSTRACT

BACKGROUND: An increasing number and proportion of cancer patients with apparently localised disease are treated with chemotherapy and radiation therapy in contemporary oncology practice. In a pilot study of radiation-induced sarcoma (RIS) patients, we demonstrated that chemotherapy was associated with a reduced time to development of RIS. We now present a multi-centre collaborative study to validate this association. METHODS: This was a retrospective cohort study of RIS cases across five large international sarcoma centres between 1 January 2000 to 31 December 2014. The primary endpoint was time to development of RIS. RESULTS: We identified 419 patients with RIS. Chemotherapy for the first malignancy was associated with a shorter time to RIS development (HR 1.37; 95% CI: 1.08-1.72; P=0.009). In the multi-variable model, older age (HR 2.11; 95% CI 1.83-2.43; P<0.001) and chemotherapy for the first malignancy (HR 1.61; 95% CI 1.26-2.05; P<0·001) were independently associated with a shorter time to RIS. Anthracyclines and alkylating agents significantly contribute to the effect. CONCLUSIONS: This study confirms an association between chemotherapy given for the first malignancy and a shorter time to development of RIS.


Subject(s)
Antineoplastic Agents, Alkylating/adverse effects , Antineoplastic Agents/adverse effects , Neoplasms, Radiation-Induced/etiology , Neoplasms, Second Primary/etiology , Sarcoma/etiology , Adolescent , Adult , Age Factors , Aged , Aged, 80 and over , Anthracyclines/adverse effects , Child , Child, Preschool , Female , Humans , Infant , Male , Middle Aged , Neoplasms/drug therapy , Neoplasms/radiotherapy , Retrospective Studies , Time Factors , Young Adult
4.
Acta Orthop Belg ; 82(2): 216-221, 2016 Aug.
Article in English | MEDLINE | ID: mdl-27682283

ABSTRACT

We are reporting our experience on patients with -pelvic Ewing's Sarcoma treated in our unit. We retrospectively reviewed a series of patients with non-metastatic pelvic Ewing's sarcoma treated between 1977 and 2009. Patients were classified into three groups according to the local treatment received : Group 1. radiotherapy-chemo ; Group 2. surgery-chemo and Group 3. radiotherapy-surgery-chemo. Recurrence free and overall survival rates were calculated using the Kaplan-Meier method. Influence of various factors (age at diagnosis, gender, tumour site and size, chemotherapy response, surgical margins and type of treatment) on survival was assessed with a logistic regression model. A total of 85 patients were treated with a mean follow-up of 65.8 months and mean -tumour volume of 435ml. The 5-year survival for all patients was 40.7% decreased to 36.2% at 10 years. A significant prognostic factor identified was chemotherapy response only. There was a trend for improved survival and local control rates for patients who had chemotherapy and surgery and the results were apparent for all tumours irrespective of size but not statistically significant. Currently, the optimal management of pelvic Ewing's sarcoma is contro-versial but our study shows a trend for improved -survival for patients treated with chemotherapy and surgery.


Subject(s)
Bone Neoplasms/mortality , Bone Neoplasms/therapy , Pelvic Bones , Sarcoma, Ewing/mortality , Sarcoma, Ewing/therapy , Adolescent , Adult , Bone Neoplasms/pathology , Child , Child, Preschool , Combined Modality Therapy , Disease-Free Survival , Female , Humans , Kaplan-Meier Estimate , Male , Middle Aged , Neoplasm Recurrence, Local , Retrospective Studies , Sarcoma, Ewing/pathology , Young Adult
5.
Ann Oncol ; 26(2): 407-14, 2015 Feb.
Article in English | MEDLINE | ID: mdl-25421877

ABSTRACT

BACKGROUND: Four international study groups undertook a large study in resectable osteosarcoma, which included two randomised controlled trials, to determine the effect on survival of changing post-operative chemotherapy based on histological response. PATIENTS AND METHODS: Patients with resectable osteosarcoma aged ≤40 years were treated with the MAP regimen, comprising pre-operatively of two 5-week cycles of cisplatin 120 mg/m(2), doxorubicin 75 mg/m(2), methotrexate 12 g/m(2) × 2 (MAP) and post-operatively two further cycles of MAP and two cycles of just MA. Patients were randomised after surgery. Those with ≥10% viable tumour in the resected specimen received MAP or MAP with ifosfamide and etoposide. Those with <10% viable tumour were allocated to MAP or MAP followed by pegylated interferon. Longitudinal evaluation of quality of life was undertaken. RESULTS: Recruitment was completed to the largest osteosarcoma study to date in 75 months. Commencing March 2005, 2260 patients were registered from 326 centres across 17 countries. About 1334 of 2260 registered patients (59%) were randomised. Pre-operative chemotherapy was completed according to protocol in 94%. Grade 3-4 neutropenia affected 83% of cycles and 59% were complicated by infection. There were three (0.13%) deaths related to pre-operative chemotherapy. At definitive surgery, 50% of patients had at least 90% necrosis in the resected specimen. CONCLUSIONS: New models of collaboration are required to successfully conduct trials to improve outcomes of patients with rare cancers; EURAMOS-1 demonstrates achievability. Considerable regulatory, financial and operational challenges must be overcome to develop similar studies in the future. The trial is registered as NCT00134030 and ISRCTN 67613327.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Bone Neoplasms/drug therapy , Osteosarcoma/drug therapy , Adolescent , Bone Neoplasms/surgery , Child , Cisplatin/administration & dosage , Cisplatin/adverse effects , Combined Modality Therapy , Doxorubicin/administration & dosage , Doxorubicin/adverse effects , Etoposide/administration & dosage , Etoposide/adverse effects , Female , Humans , Ifosfamide/administration & dosage , Ifosfamide/adverse effects , Interferon-alpha/administration & dosage , Interferon-alpha/adverse effects , Male , Methotrexate/administration & dosage , Methotrexate/adverse effects , Neoadjuvant Therapy , Osteosarcoma/surgery , Polyethylene Glycols/administration & dosage , Polyethylene Glycols/adverse effects , Quality of Life , Research Design , Young Adult
6.
Ann Oncol ; 26(5): 865-872, 2015 May.
Article in English | MEDLINE | ID: mdl-25070543

ABSTRACT

BACKGROUND: The use of potential surrogate end points for overall survival, such as disease-free survival (DFS) or time-to-treatment failure (TTF) is increasingly common in randomized controlled trials (RCTs) in cancer. However, the definition of time-to-event (TTE) end points is rarely precise and lacks uniformity across trials. End point definition can impact trial results by affecting estimation of treatment effect and statistical power. The DATECAN initiative (Definition for the Assessment of Time-to-event End points in CANcer trials) aims to provide recommendations for definitions of TTE end points. We report guidelines for RCT in sarcomas and gastrointestinal stromal tumors (GIST). METHODS: We first carried out a literature review to identify TTE end points (primary or secondary) reported in publications of RCT. An international multidisciplinary panel of experts proposed recommendations for the definitions of these end points. Recommendations were developed through a validated consensus method formalizing the degree of agreement among experts. RESULTS: Recommended guidelines for the definition of TTE end points commonly used in RCT for sarcomas and GIST are provided for adjuvant and metastatic settings, including DFS, TTF, time to progression and others. CONCLUSION: Use of standardized definitions should facilitate comparison of trials' results, and improve the quality of trial design and reporting. These guidelines could be of particular interest to research scientists involved in the design, conduct, reporting or assessment of RCT such as investigators, statisticians, reviewers, editors or regulatory authorities.


Subject(s)
Endpoint Determination/standards , Gastrointestinal Stromal Tumors/therapy , Randomized Controlled Trials as Topic/standards , Research Design/standards , Sarcoma/therapy , Terminology as Topic , Consensus , Delphi Technique , Disease Progression , Disease-Free Survival , Endpoint Determination/classification , Gastrointestinal Stromal Tumors/diagnosis , Gastrointestinal Stromal Tumors/mortality , Humans , Randomized Controlled Trials as Topic/classification , Sarcoma/diagnosis , Sarcoma/mortality , Time Factors , Treatment Failure
7.
Skeletal Radiol ; 44(7): 963-9, 2015 Jul.
Article in English | MEDLINE | ID: mdl-25744812

ABSTRACT

OBJECTIVE: To assess if diffusion-weighted MRI (DWI) can differentiate between central enchondromas and chondrosarcomas (CS) and if DWI can distinguish low-grade chondroid lesions from high-grade CS. MATERIALS AND METHODS: Fifty-two patients with central cartilage tumors were included. Patients underwent conventional MRI and DWI with ADC mapping. The slice on MRI with the most aggressive imaging feature was identified. The corresponding mean and minimum ADC maps of the tumor at this position were measured. RESULTS: There were 24 enchondromas, five atypical cartilaginous lesions, 15 grade 1, three grade 2, two grade 3, and three dedifferentiated CS. Mean ADC values (×10(-6) mm2/s) for enchondromas, atypical cartilaginous tumors, grade 1 CS, grade 2, CS, grade 3 CS and dedifferentiated CS were 1,896, 2,048, 2,152, 2,170, 2,076, and 1,261, respectively. Minimum ADC values (×10(-6) mm2/s) for enchondromas, atypical cartilaginous tumors, grade 1 CS, grade 2, CS, grade 3 CS and dedifferentiated CS were 1,820, 1,752, 2,010, 1,829, 1,752, and 767, respectively. ANOVA test demonstrated a statistically significant difference in mean and minimum ADC values in all groups. Post hoc analysis revealed this was due to difference in mean and minimum ADC values in dedifferentiated CS. The mean ADC value in low-grade chondroid lesions was 2,001, while the ADC value for high-grade CS were 2,132. The minimum ADC value for low-grade chondroid lesions was 1,896, while the minimum ADC for high-grade CS was 1,837. The difference in minimum and mean ADC values was not statistically significant. CONCLUSIONS: DWI cannot differentiate between enchondromas and CS and DWI does not aid in the distinction of low-grade chondroid tumors from high-grade CS.


Subject(s)
Bone Neoplasms/pathology , Chondroma/pathology , Chondrosarcoma/pathology , Diffusion Magnetic Resonance Imaging/methods , Image Interpretation, Computer-Assisted/methods , Adolescent , Adult , Aged , Aged, 80 and over , Child , Diagnosis, Differential , Female , Humans , Male , Middle Aged , Observer Variation , Reproducibility of Results , Sensitivity and Specificity , Young Adult
8.
Skeletal Radiol ; 43(2): 179-89, 2014 Feb.
Article in English | MEDLINE | ID: mdl-24240323

ABSTRACT

OBJECTIVE: Sacrectomy and ilio-lumbar reconstruction is an uncommonly performed complex surgical procedure for the treatment of sacral neoplasia. There are many challenges in the post-operative period including the potential for tumor recurrence, infection, and construct failure. We present our experience of this patient cohort and describe the complications and imaging appearances that can be encountered during the follow-up period. MATERIALS AND METHODS: Retrospective review of our Orthopaedic Oncology database was undertaken which has been collected over a 30-year period to identify patients that had undergone sacrectomy and ilio-lumbar reconstruction. Pre and post-operative imaging including radiographs, CT, and MRI was reviewed. These were viewed by two experienced musculoskeletal radiologists with consensus opinion if there was disagreement over the imaging findings. Data regarding patient demographics, tumor type, and dimensions was collected. Serial review of radiographs, CT, and MRI was performed to assess implant position and integrity, strut graft position and union, and for the presence of recurrence within the surgical bed. RESULTS: Five male and two female patients (mean age 36 years, age range 15-54 years) were treated with this procedure. Histological diagnoses included chordoma, chondrosarcoma, osteosarcoma, and spindle cell sarcoma. Mean maximal tumor size on pre-operative imaging was 10.7 cm (range, 6-16 cm). Post-operative follow-up ranged from 10-46 months. A total of 76 imaging studies were reviewed. Commonly identified complications included vertical rod and cross-connector fracture and screw loosening. Fibula strut graft non-union and fracture was also evident on imaging review. Two patients demonstrated disease recurrence during the follow-up period. CONCLUSIONS: This study demonstrates the spectrum and frequency of complications that can occur following sacrectomy and ilio-lumbar reconstruction for sacral neoplasia.


Subject(s)
Ilium/surgery , Lumbar Vertebrae/surgery , Magnetic Resonance Imaging , Sacrum/surgery , Spinal Neoplasms/diagnosis , Spinal Neoplasms/surgery , Tomography, X-Ray Computed , Adolescent , Adult , Child , Female , Humans , Ilium/diagnostic imaging , Ilium/pathology , Lumbar Vertebrae/diagnostic imaging , Lumbar Vertebrae/pathology , Male , Middle Aged , Plastic Surgery Procedures , Retrospective Studies , Sacrum/diagnostic imaging , Sacrum/pathology , Treatment Outcome , Vertebroplasty , Young Adult
9.
Clin Radiol ; 68(12): e676-9, 2013 Dec.
Article in English | MEDLINE | ID: mdl-24034551

ABSTRACT

AIM: To present the imaging features of post-traumatic heterotopic ossification (HO) of the distal tibiofibular syndesmosis initially suspected to be a surface osteosarcoma. MATERIALS AND METHODS: A retrospective review was conducted of the presenting complaint and imaging features of patients with a final diagnosis of HO referred over an 8 year period to a specialist orthopaedic oncology centre. RESULTS: Five patients with HO were identified. All were adult males with an age range of 19-41 years. There was a history of prior ankle trauma in all cases but the significance was not recognized at the time of referral to the specialist centre. There was radiographic evidence of HO arising from the inner aspects of the distal tibia and fibula approximately 3 cm proximal to the ankle joint. The HO was "kissing" in two cases and partially fused (synostosis) in two. The HO in the fifth case was arising on the inner fibular cortex alone. Magnetic resonance imaging (MRI), available in four cases, showed predominantly low signal intensity due to the dense bone formation. CONCLUSION: The history of prior ankle trauma with ossification arising from the inner aspects of both the distal tibia and fibula is typical of post-traumatic HO and distinguish this benign condition from the rare surface osteosarcoma at this site.


Subject(s)
Ankle Joint/diagnostic imaging , Bone Neoplasms/diagnostic imaging , Ossification, Heterotopic/diagnostic imaging , Osteosarcoma/diagnostic imaging , Adult , Ankle Injuries/complications , Ankle Joint/pathology , Bone Neoplasms/pathology , Diagnosis, Differential , Humans , Magnetic Resonance Imaging , Male , Ossification, Heterotopic/pathology , Osteosarcoma/pathology , Tomography, X-Ray Computed , Young Adult
10.
Ann Oncol ; 23(6): 1607-16, 2012 Jun.
Article in English | MEDLINE | ID: mdl-22015453

ABSTRACT

BACKGROUND: Neoadjuvant chemotherapy improves outcome in osteosarcoma. Determination of optimum regimens for survival, toxicity and prognostic factors requires randomised controlled trials to be conducted. PATIENTS AND METHODS: Between 1983 and 2002, the European Osteosarcoma Intergroup recruited 1067 patients with localised extremity osteosarcoma to three randomised controlled trials. Standard treatment in each was doxorubicin 75 mg/m(2) and cisplatin 100 mg/m(2). Comparators were addition of methotrexate (BO02/80831), a multidrug regimen (BO03/80861) and a dose-intense schedule (BO06/80931). Standard survival analysis methods were used to identify prognostic factors, temporal and other influences on outcome. RESULTS: Five- and 10-year survival were 56% (95% confidence interval 53% to 59%) and 52%, respectively (49% to 55%), with no difference between trials or treatment arms. Median follow-up was 9.4 years. Age range was 3-40 years (median 15). Limb salvage was achieved in 69%. Five hundred and thirty-three patients received the standard arm, 79% completing treatment. Good histological response to preoperative chemotherapy, distal tumour location (all sites other than proximal humerus/femur) and female gender were associated with improved survival. CONCLUSIONS: Localised osteosarcoma will be cured in 50% of patients with cisplatin and doxorubicin. Large randomised trials can be conducted in this rare cancer. Failure to improve survival over 20 years argues for concerted collaborative international efforts to identify and rapidly test new treatments.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Arm Bones/pathology , Bone Neoplasms/drug therapy , Leg Bones/pathology , Osteosarcoma/drug therapy , Survival , Adolescent , Adult , Bone Neoplasms/mortality , Bone Neoplasms/pathology , Child , Female , Humans , Kaplan-Meier Estimate , Male , Multivariate Analysis , Neoplasm Grading , Neoplasm Recurrence, Local , Osteosarcoma/mortality , Osteosarcoma/pathology , Proportional Hazards Models , Randomized Controlled Trials as Topic , Retrospective Studies , Treatment Outcome , Young Adult
11.
Skeletal Radiol ; 41(12): 1537-42, 2012 Dec.
Article in English | MEDLINE | ID: mdl-22422023

ABSTRACT

OBJECTIVE: It has been widely postulated that enchondromas arise from cartilage remnants that have been displaced from the growth plate into the metaphysis. However, this theory remains unproven. Based on the common occurrence of enchondromas on routine knee MR imaging (2.9 %), one would expect to find displaced cartilage in the metaphysis of skeletally immature individuals on routine knee MR examinations if the above theory was to be supported. MATERIALS AND METHODS: The electronic databases of a specialist orthopedic hospital and children's hospital were searched for skeletally immature patients who underwent MR imaging of the knee for a variety of indications. Individuals with Ollier disease or hereditary multiple exostoses were excluded. The MR images were subsequently reviewed by a musculoskeletal radiologist for evidence of displaced cartilage into the metaphysis. RESULTS: We reviewed 240 MR examinations of the knee that were performed in 209 patients. There were 125 MR studies in male and 115 MR examinations in female patients (age range: 5 months-16 years; median age: 13 years). In 97.1 %, the growth plates around the knee demonstrated a regular appearance. Seven cases (2.9 %) in six patients showed cartilage extension from the growth plate into the metaphysis, which remained in continuity with the growth plate. There were no cases of displaced cartilage into the metaphysis on MRI. CONCLUSIONS: Our study challenges the widely believed theory that enchondromas arise from displaced growth plate remnants.


Subject(s)
Bone Neoplasms/pathology , Chondroma/pathology , Knee/pathology , Magnetic Resonance Imaging/methods , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Reproducibility of Results , Sensitivity and Specificity
12.
Sarcoma ; 2012: 636849, 2012.
Article in English | MEDLINE | ID: mdl-22550421

ABSTRACT

Leiomyosarcoma of bone is just one of the variants of spindle cell sarcoma of bone characterised by the expression of desmin and other markers indicating a significant element of smooth muscle in the tumour, without osteoid production we have investigated the management and outcome of this rare type of primary malignant bone tumour. Method. Retrospective review of data stored on a prospective database. Results. In a database of 3364 patients with primary malignant bone sarcomas, 31 patients were identified with a primary leiomyosarcoma of bone. There were 12 males and 19 females with a mean age of 46 and tumour size of 8 cm. The most common site was the distal femur followed by the proximal tibia. Treatment was with chemotherapy and surgical resection. Seven of the patients had metastases at diagnosis. Surgery was carried out in 28 patients, 8 having amputation and 20 limb salvage. Three patients developed local recurrence, but half developed metastases. All patient disease-specific survival was 57% at five years and 44% at 10 yrs but for those without metastases was 82% and 60%, respectively. The only prognostic factors were metastases at diagnosis. Conclusion. Leiomyosarcoma of bone is a very rare primary malignant bone tumour affecting a predominantly older population. Despite the high incidence of metastases, survival is better than for other bone sarcomas for those without metastases at diagnosis.

13.
Sarcoma ; 2012: 764796, 2012.
Article in English | MEDLINE | ID: mdl-22851905

ABSTRACT

Low-grade central osteosarcoma (LGCO) is a rare variant of osteosarcoma which is difficult to diagnose. If not treated appropriately, the tumour can recur with higher-grade disease. We reviewed our experience of this condition to try and identify factors that could improve both diagnosis and outcome. 18 patients out of 1540 osteosarcoma cases (over 25 years) had LGCO (1.2%). Only 11 patients (61%) were direct primary referrals. Almost 40% (7 of 18) cases were referred after treatment elsewhere when the diagnosis had not been made initially and all presented with local recurrence. Of the 11 who presented primarily, the first biopsy was diagnostic in only 6 (55%) cases. Of the remaining cases, up to three separate biopsies were required before a definitive diagnosis was made. Overall survivorship at 5 years was 90%. 17 patients were treated with limb salvage procedures, and one patient had an amputation. The diagnosis of LGCO remains challenging due to the relatively nonspecific radiological and histological findings. Since treatment of LGCO is so different to a benign lesion, accurate diagnosis is essential. Any difficult or nondiagnostic biopsies of solitary bone lesions should be referred to specialist tumour units for a second opinion.

14.
Sarcoma ; 2011: 538085, 2011.
Article in English | MEDLINE | ID: mdl-22190864

ABSTRACT

Background. This study aimed to investigate prognostic factors for patients with myxoid/round-cell liposarcoma (MRCLS), in particular the significance of the round cell component, and to identify metastatic patterns as well as possibly suggest a suitable strategy for followup. Methods. Clinical, morphologic, and follow-up data from 160 patients with MRCLS was reviewed and statistically analysed. Results. Of 130 tumours with the round cell component evaluated, 61 had no round cell component, 27 had <5% round cell component, and 42 had >5%. All patients underwent surgical excision, 15 requiring amputation. 107 patients received adjuvant radiotherapy. Local recurrence occurred in 19 patients (12%), predominantly in patients with marginal or intralesional margins and a round cell component. Overall disease specific survival was 75% at 5 years and 56% at 10 years and was related to the proportion of round cell component. Of 52 patients who developed metastases, 38 (73%) had purely extrapulmonary metastases. We could not identify any factors predicting the site of metastases developing. Conclusions. The occurrence of any round cell component is the most important adverse prognostic factor for patients with MRCLS; patients with >5% round cell component are at higher risk of local recurrence, metastasis and tumour-related death and should be considered for adjuvant radiotherapy and possibly chemotherapy. The best method of monitoring extrapulmonary metastases remains to be established.

15.
Paediatr Perinat Epidemiol ; 24(5): 470-8, 2010 Sep.
Article in English | MEDLINE | ID: mdl-20670227

ABSTRACT

Malignant bone tumours in the paediatric age group (0-14 years) are uncommon; various aetiological theories exist and few reports of incidence, age and sex distributions have been published. We examined the incidence of childhood malignant bone tumours in one large single region of the UK over an extended period of 50 years. The West Midlands specialist regional children's tumour registry holds data on all malignancies and benign brain tumours in children under 15 years in the West Midlands region, which has a population of around 1 million children. Demographic and clinical data have been abstracted and diagnoses reviewed by a panel of expert pathologists. During the period 1957-2006, 259 cases of malignant paediatric bone tumours were diagnosed. There were 153 osteosarcomas, 78 Ewing sarcomas and 28 other primary bone tumours. The overall age standardised rate was 4.66, with no increase over time, although there was a significant increase in the incidence of Ewing sarcomas in the period 1965-92. Sixty-eight per cent of tumours were in patients over 10 years, whereas the incidence in patients under 10 years showed a non-significant increase. Survival rates increased dramatically post-chemotherapy introduction, with Ewing sarcoma demonstrating better survival overall. This is a large study giving an overview of malignant bone tumours in the childhood population of a single region over an extended period, showing results consistent with national reports. It also examines late effects, which were mostly mobility/orthopaedic, although almost one-fifth of patients had cardiac problems and five went on to develop second malignancies.


Subject(s)
Bone Neoplasms/therapy , Sarcoma, Ewing/therapy , Adolescent , Bone Neoplasms/epidemiology , Child , Child, Preschool , Female , Humans , Incidence , Infant , Male , Proportional Hazards Models , Sarcoma, Ewing/epidemiology , Survival Rate , Treatment Outcome , United Kingdom
16.
Clin Radiol ; 65(5): 382-6, 2010 May.
Article in English | MEDLINE | ID: mdl-20380937

ABSTRACT

AIM: To investigate the association of a fatigue-type stress fracture and a fibrous cortical defect/non-ossifying fibroma (FCD/NOF) of the lower limb long bones in skeletally immature patients. MATERIALS AND METHODS: The patient database of a specialist orthopaedic oncology centre was searched to determine the number of skeletally immature patients (

Subject(s)
Bone Neoplasms/complications , Fibroma, Ossifying/complications , Fractures, Stress/etiology , Leg Injuries/etiology , Osteosarcoma/complications , Adolescent , Bone Neoplasms/diagnostic imaging , Child , Female , Femoral Fractures/diagnostic imaging , Femoral Fractures/etiology , Fibroma, Ossifying/diagnostic imaging , Fibula/injuries , Fractures, Stress/diagnostic imaging , Humans , Leg Injuries/diagnostic imaging , Male , Osteosarcoma/diagnostic imaging , Radiography , Tibial Fractures/diagnostic imaging , Tibial Fractures/etiology
17.
Eur Spine J ; 19(7): 1189-94, 2010 Jul.
Article in English | MEDLINE | ID: mdl-20076978

ABSTRACT

To investigate the outcome of our management of patients with giant cell tumour of the sacrum and draw lessons from this. A retrospective review of medical records and scans for all patients treated at our unit over the past 20 years with a giant cell tumour of the sacrum. Of the 517 patients treated at our unit for giant cell tumour over the past 20 years, only 9 (1.7%) had a giant cell tumour in the sacrum. Six were female, three male with a mean age of 34 (range 15-52). All, but two tumours involved the entire sacrum and there was only one purely distal to S3. The mean size was 10 cm and the most common symptom was back or buttock pain. Five had abnormal neurology at diagnosis, but only one presented with cauda equina syndrome. The first four patients were treated by curettage alone, but two patients had intraoperative cardiac arrests and although both survived all subsequent curettages were preceded by embolisation of the feeding vessels. Of the seven patients who had curettage, three developed local recurrence, but all were controlled with a combination of further embolisation, surgery or radiotherapy. One patient elected for treatment with radiotherapy and another had excision of the tumour distal to S3. All the patients are alive and only two patients have worse neurology than at presentation, one being impotent and one with stress incontinence. Three patients required spinopelvic fusion for sacral collapse. All patients are mobile and active at a follow-up between 2 and 21 years. Giant cell tumour of the sacrum can be controlled with conservative surgery rather than subtotal sacrectomy. The excision of small distal tumours is the preferred option, but for larger and more extensive tumours conservative management may well avoid morbidity whilst still controlling the tumour. Embolisation and curettage are the preferred first option with radiotherapy as a possible adjunct. Spinopelvic fusion may be needed when the sacrum collapses.


Subject(s)
Bone Neoplasms/surgery , Decision Support Techniques , Giant Cell Tumor of Bone/surgery , Sacrum/surgery , Adolescent , Adult , Bone Neoplasms/pathology , Bone Neoplasms/radiotherapy , Embolization, Therapeutic , Female , Giant Cell Tumor of Bone/pathology , Giant Cell Tumor of Bone/radiotherapy , Humans , Male , Middle Aged , Retrospective Studies , Sacrum/pathology , Treatment Outcome
18.
Foot Ankle Int ; 31(2): 131-5, 2010 Feb.
Article in English | MEDLINE | ID: mdl-20132749

ABSTRACT

BACKGROUND: Aneurysmal bone cysts (ABC) mainly occur in the long bones but only rarely in the bones of the feet. This study reviews the 25-year experience of a tertiary orthopaedic oncology referral center treating ABC's in the bones of the feet, using prospectively collected data to document their incidence, treatment and the outcome. MATERIALS AND METHODS: Information on the diagnosis, treatment, complications and outcomes were collected for all eligible patients from our prospectively collected database. Radiologic healing was used as our primary outcome measure. Cases were considered to have failed treatment if there was progression of the lesion that needed further treatment. RESULTS: Of 273 patients with ABC, 16 patients (5.9%) were found to have their lesions in the foot. There were ten female and six male patients, aged from 4 to 52 (mean, 18.2) years. The metatarsals were the most commonly affected site (44%). Curettage of the lesion without additional adjuvant measures (14 of 16 patients) was sufficient for the treatment in 11 of 14 patients (79%). This gave a recurrence rate of 21% (three of 14 patients) in those treated by curettage alone and 19% (three of 16 patients) if the whole cohort is considered. CONCLUSION: Although relatively rare, there is no reason to assume that ABCs of the feet will respond to treatment or recur any differently from ABCs that occur elsewhere in the body. Our experience has been that surgical curettage alone is sufficient to treat most ABCs of the feet.


Subject(s)
Bone Cysts, Aneurysmal/surgery , Curettage/methods , Foot Bones/pathology , Foot Bones/surgery , Adolescent , Adult , Bone Cysts, Aneurysmal/diagnosis , Bone Cysts, Aneurysmal/epidemiology , Child , Child, Preschool , Female , Humans , Incidence , Magnetic Resonance Imaging , Male , Middle Aged , Prospective Studies , Treatment Outcome , United Kingdom/epidemiology
19.
Int Orthop ; 34(3): 413-7, 2010 Mar.
Article in English | MEDLINE | ID: mdl-19387641

ABSTRACT

Chondroblastoma of the femoral head presents particular problems in treatment because the tumour is surrounded by articular cartilage on one side and epiphyseal plate on the other. Ten patients underwent treatment for a chondroblastoma involving the proximal femoral capital epiphysis. The patients were aged between eight and 19 years and in four the epiphysis was not yet fused. Five had curettage via a drill hole created up the femoral neck, of whom two developed local recurrence. Five had a direct approach to the chondroblastoma through the femoral neck, and there were no local recurrences in this group. Both patients with local recurrence were under 14 years of age-one was cured by a direct approach through the neck of the femur and the other by lifting a trap door of articular cartilage. Apart from one patient with a temporary leg length discrepancy, there were no other complications. We conclude that a direct approach is likely to lead to the best outcome for this rare condition.


Subject(s)
Bone Neoplasms/pathology , Chondroblastoma/pathology , Femur Head/pathology , Adolescent , Bone Neoplasms/surgery , Child , Chondroblastoma/surgery , Curettage , Female , Femur Head/surgery , Femur Neck/surgery , Humans , Male , Neoplasm Recurrence, Local , Postoperative Complications , Retrospective Studies , Treatment Outcome , Young Adult
20.
Sarcoma ; 20102010.
Article in English | MEDLINE | ID: mdl-20936113

ABSTRACT

Introduction. The ''two-week wait" was established as a potential means of diagnosing malignant tumours earlier. This paper investigated whether these clinics are leading to earlier diagnosis of malignant soft-tissue lumps. Method. We identified all referrals to our centre from a database over a 4-year period. Results. 2225 patients were referred to our unit for investigation of a soft-tissue mass. 576 (26%) were referred under the ''two-week wait" criteria. 153 (27%) of which were found to have a malignant or borderline malignant diagnosis. 1649 patients were referred nonurgently. 855 (52%) of which were diagnosed with a malignant or borderline lesion. The average size at diagnosis was 9.4 cm with no difference in size between the different referral routes. Conclusion. There is little evidence that the two-week wait clinic is leading to earlier diagnosis of soft-tissue sarcomas with the majority still being referred nonurgently.

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