Aims. Giant cell tumour of bone (GCTB) is a locally aggressive lesion that is difficult to treat as salvaging the joint can be associated with a high rate of local recurrence (LR). We evaluated the risk factors for tumour relapse after treatment of a GCTB of the limbs. Methods. A total of 354 consecutive patients with a GCTB underwent joint salvage by curettage and reconstruction with bone graft and/or cement or en bloc resection. Patient, tumour, and treatment factors were analyzed for their impact on LR. Patients treated with denosumab were excluded. Results. There were 53 LRs (15%) at a mean 30.5 months (5 to 116). LR was higher after curettage (18.4%) than after resection (4.6%; p = 0.008). Neither pathological fracture (p = 0.240), Campanacci grade (p = 0.734), soft-tissue extension (p = 0.297), or tumour size (p = 0.872) affected the risk of recurrence. Joint salvage was possible in 74% of patients overall (262/354), and 98% after curettage alone (262/267). Of 49 patients with LR after curettage, 44 (90%) underwent repeated curettage and joint salvage. For patients treated by curettage, only age less than 30 years (p = 0.042) and location in the distal radius (p = 0.043) predicted higher LR. The rate of LR did not differ whether cement or bone graft was used (p = 0.753), but may have been reduced by the use of hydrogen peroxide (p = 0.069). Complications occurred in 15.3% of cases (54/354) and did not differ by treatment. Conclusion. Most patients with a GCTB can undergo successful joint salvage by aggressive curettage, even in the presence of a soft-tissue mass, pathological fracture, or a large lesion, with an 18.4% risk of local recurrence. However, 90% of local relapses after curettage can be treated by repeat joint salvage. Maximizing joint salvage is important to optimize long-term function since most patients with a GCTB are
Clear cell sarcoma (CCS) of soft-tissue is a rare melanocytic subtype of mesenchymal malignancy. The aim of this study was to investigate the clinical and therapeutic factors associated with increased survival, stratified by clinical stage, in order to determine the optimal treatment. The study was a retrospective analysis involving 117 patients with histologically confirmed CCS, between July 2016 and November 2017, who were enrolled in the Bone and Soft Tissue Tumour Registry in Japan.Aims
Methods
While a centralized system for the care of patients with a sarcoma has been advocated for decades, regional variations in survival remain unclear. The aim of this study was to investigate regional variations in survival and the impact of national policies in patients with a soft-tissue sarcoma (STS) in the UK. The study included 1,775 patients with a STS who were referred to a tertiary sarcoma centre. The geographical variations in survival were evaluated according to the periods before and after the issue of guidance by the National Institute for Health and Care Excellence (NICE) in 2006 and the relevant evolution of regional management.Aims
Methods
Urgent referral to a specialist centre for patients with a soft-tissue sarcoma (STS) has been recommended by the National Institute for Health and Care Excellence (NICE) in the UK since 2006. However, the impact of this recommendation on the prognosis for these patients remains unclear. We aimed to determine the impact of the NICE guidelines on the disease-specific survival (DSS) of patients with an STS. A total of 2,427 patients with an STS referred to a supraregional centre in the ten-year periods before (n = 1,386) and after (n = 1,041) the issue of the NICE guidelines were evaluated.Aims
Methods
Local recurrence remains a challenging and common problem following curettage and joint-sparing surgery for giant cell tumour of bone (GCTB). We previously reported a 15% local recurrence rate at a median follow-up of 30 months in 20 patients with high-risk GCTB treated with neoadjuvant Denosumab. The aim of this study was to determine if this initial favourable outcome following the use of Denosumab was maintained with longer follow-up. Patients with GCTB of the limb considered high-risk for unsuccessful joint salvage, due to minimal periarticular and subchondral bone, large soft tissue mass, or pathological fracture, were treated with Denosumab followed by extended intralesional curettage with the goal of preserving the joint surface. Patients were followed for local recurrence, metastasis, and secondary sarcoma.Aims
Methods
To assess the correlation between the histological response to preoperative chemotherapy and event-free survival (EFS) or overall survival (OS) in patients with high-grade localized osteosarcoma. Out of 625 patients aged ≤ 40 years treated for primary high-grade osteosarcoma between 1997 and 2016, 232 patients without clinically detectable metastases at the time of diagnosis and treated with preoperative high-dose methotrexate, adriamycin and cisplatin (MAP) chemotherapy and surgery were included. Associations of chemotherapy-induced necrosis in the resected specimen and EFS or OS were assessed using Cox model and the Pearson’s correlation coefficients (r). Time-dependent receiver operating characteristic analysis was applied to determine the optimal cut-off value of chemotherapy-induced necrosis for EFS and OS.Aims
Methods
The aim of this study was to evaluate health-related quality of life (HRQoL) and joint function in tenosynovial giant cell tumour (TGCT) patients before and after surgical treatment. This prospective cohort study run in two Dutch referral centres assessed patient-reported outcome measures (PROMs; 36-Item Short-Form Health Survey (SF-36), visual analogue scale (VAS) for pain, and Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC)) in 359 consecutive patients with localized- and diffuse-type TGCT of large joints. Patients with recurrent disease (n = 121) and a wait-and-see policy (n = 32) were excluded. Collected data were analyzed at specified time intervals preoperatively (baseline) and/or postoperatively up to five years.Aims
Patients and Methods
Aims
Patients and Methods
The aim of this study was to identify any progression between
benign osteofibrous dysplasia (OFD), OFD-like adamantinoma and malignant
adamantinoma, and to investigate the rates of local recurrence,
metastases and survival, in order to develop treatment algorithms
for each. A single institution retrospective review of all patients presenting
with OFD, OFD-like adamantinoma and adamantinoma between 1973 and
2012 was undertaken. Complete data were available for 73 patients
(42 with OFD; ten with an OFD-like adamantinoma and 21 with an adamantinoma).
The mean follow-up was 10.3 years (3 to 25) for OFD, 9.2 years (3.0
to 26.3) for OFD-like and 11.6 years (0.25 to 33) for adamantinoma.Aims
Patients and Methods
Opinion remains divided as to whether the development
of pathological fracture affects the prognosis of patients with
an osteosarcoma of the extremities. We conducted a comprehensive systematic review and meta-analysis
of papers which reported the outcomes of osteosarcoma patients with
and without a pathological fracture. There were eight eligible papers
for final analysis which reported on 1713 patients, of whom 303
(17.7%) had a pathological fracture. The mean age for 1464 patients in
six studies was 23.2 years old (2 to 82). The mean follow-up for
1481 patients in seven studies was 90.1 months (6 to 240). The pooled estimates of local recurrence rates in osteosarcoma
patients with and without pathological fractures were 14.4% (8.7
to 20.0) The development of a pathological fracture is a negative prognostic
indicator in osteosarcoma and is associated with a reduced five-year
event-free survival and a possibly higher rate of local recurrence.
Our findings suggest that there is no absolute indication for amputation,
as similar rates of local recurrence can be achieved in patients
who are carefully selected for limb salvage. Cite this article:
Avascular necrosis (AVN) is a serious complication
of high-dose chemotherapy for haematological malignancy in childhood.
In order to describe its incidence and main risk factors and to
evaluate the current treatment options, we reviewed 105 children
with a mean age of 8.25 years (1 to 17.8) who had acute lymphoblastic
or acute myeloid leukaemia, or a non-Hodgkin’s lymphoma. Overall,
eight children (7.6%) developed AVN after a mean of 16.8 months (8
to 49). There were four boys and four girls with a mean age of 14.4
years (9.8 to 16.8) and a total of 18 involved sites, 12 of which
were in the femoral head. All these children were aged >
nine years
(p <
0.001). All had received steroid treatment with a mean cumulative
dose of prednisone of 5967 mg (4425 to 9599) compared with a mean
of 3943 mg (0 to 18 585) for patients without AVN (p = 0.005). No
difference existed between genders and no thrombophilic disorders
were identified. Their initial treatment included 11 core decompressions
and two bipolar hip replacements. Later, two salvage osteotomies
were done and three patients (four hips) eventually needed a total
joint replacement. We conclude that AVN mostly affects the weight-bearing
epiphyses. Its risk increases with age and higher steroid doses.
These high-risk patients may benefit from early screening for AVN. Cite this article:
A retrospective analysis was performed of eight patients with an open triradiate cartilage, who underwent resection for osteosarcoma and reconstruction of the proximal femur with a hemiarthroplasty, in order to identify changes of acetabular development. An analysis of the centre-edge angle, teardrop-to-medial prosthesis distance, superior joint space, teardrop-to-superior prosthesis distance, degree of lateral translation, and arthritic changes, was performed on serial radiographs. The median age at the time of the initial surgery was 11 years (5 to 14). All patients developed progressive superior and lateral migration of the prosthetic femoral head. Following hemiarthroplasty in the immature acetabulum, the normal deepening and enlargement of the acetabulum is arrested. The degree of superior and lateral migration of the prosthetic head depends on the age at diagnosis and the length of follow-up.
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.
We review the treatment of pelvic Ewing’s sarcoma by the implantation of extracorporeally-irradiated (ECI) autografts and compare the outcome with that of other reported methods. We treated 13 patients with ECI autografts between 1994 and 2004. There were seven males and six females with a median age of 15.7 years (interquartile range (IQR) 12.2 to 21.7). At a median follow-up of five years (IQR 1.8 to 7.4), the disease-free survival was 69% overall, and 75% if one patient with local recurrence after initial treatment elsewhere was excluded. Four patients died from distant metastases at a mean of 17 months (13 to 23). There were three complications which required operative intervention; one was a deep infection which required removal of the graft. The functional results gave a mean Musculoskeletal Tumor Society score of 85% (60% to 97%), a mean Toronto extremity salvage score of 86% (69% to 100%) and a mean Harris hip score of 92 (67 to 100). We conclude that ECI grafting is a suitable form of treatment for localised and resectable pelvic Ewing’s sarcoma.
