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
This study aimed to compare the performance of survival prediction models for bone metastases of the extremities (BM-E) with pathological fractures in an Asian cohort, and investigate patient characteristics associated with survival. This retrospective cohort study included 469 patients, who underwent surgery for BM-E between January 2009 and March 2022 at a tertiary hospital in South Korea. Postoperative survival was calculated using the PATHFx3.0, SPRING13, OPTIModel, SORG, and IOR models. Model performance was assessed with area under the curve (AUC), calibration curve, Brier score, and decision curve analysis. Cox regression analyses were performed to evaluate the factors contributing to survival.Aims
Methods
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. 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.Aims
Methods
The aim of this study was to investigate the feasibility of application of a 3D-printed megaprosthesis with hemiarthroplasty design for defects of the distal humerus or proximal ulna following tumour resection. From June 2018 to January 2020, 13 patients with aggressive or malignant tumours involving the distal humerus (n = 8) or proximal ulna (n = 5) were treated by en bloc resection and reconstruction with a 3D-printed megaprosthesis with hemiarthroplasty, designed in our centre. In this paper, we summarize the baseline and operative data, oncological outcome, complication profiles, and functional status of these patients.Aims
Methods
Time to treatment initiation (TTI) is generally defined as the time from the histological diagnosis of malignancy to the initiation of first definitive treatment. There is no consensus on the impact of TTI on the overall survival in patients with a soft-tissue sarcoma. The purpose of this study was to determine if an increased TTI is associated with overall survival in patients with a soft-tissue sarcoma, and to identify the factors associated with a prolonged TTI. We identified 23,786 patients from the National Cancer Database who had undergone definitive surgery between 2004 and 2015 for a localized high-grade soft-tissue sarcoma of the limbs or trunk. A Cox proportional hazards model was used to examine the relationship between a number of factors and overall survival. We calculated the incidence rate ratio (IRR) using negative binomial regression models to identify the factors that affected TTI.Aims
Methods
We have evaluated the survivorship, outcomes, and failures of an interlocking, reconstruction-mode stem-sideplate implant used to preserve the native hip joint and achieve proximal fixation when there is little residual femur during large endoprosthetic reconstruction of the distal femur. A total of 14 patients underwent primary or revision reconstruction of a large femoral defect with a short remaining proximal femur using an interlocking, reconstruction-mode stem-sideplate for fixation after oncological distal femoral and diaphyseal resections. The implant was attached to a standard endoprosthetic reconstruction system. The implant was attached to a standard endoprosthetic reconstruction system. None of the femoral revisions were amenable to standard cemented or uncemented stem fixation. Patient and disease characteristics, surgical history, final ambulatory statusAims
Methods
Accurate estimations of the risk of fracture due to metastatic bone disease in the femur is essential in order to avoid both under-treatment and over-treatment of patients with an impending pathological fracture. The purpose of the current retrospective in vivo study was to use CT-based finite element analyses (CTFEA) to identify a clear quantitative differentiating factor between patients who are at imminent risk of fracturing their femur and those who are not, and to identify the exact location of maximal weakness where the fracture is most likely to occur. Data were collected on 82 patients with femoral metastatic bone disease, 41 of whom did not undergo prophylactic fixation. A total of 15 had a pathological fracture within six months following the CT scan, and 26 were fracture-free during the five months following the scan. The Mirels score and strain fold ratio (SFR) based on CTFEA was computed for all patients. A SFR value of 1.48 was used as the threshold for a pathological fracture. The sensitivity, specificity, positive, and negative predicted values for Mirels score and SFR predictions were computed for nine patients who fractured and 24 who did not, as well as a comparison of areas under the receiver operating characteristic curves (AUC of the ROC curves).Aims
Methods
The aim of this study is to evaluate the clinical results of operative intervention for femoral metastases which were selected based on expected survival and to discuss appropriate surgical strategies. From 2002 to 2017, 148 consecutive patients undergoing surgery for femoral metastasis were included in this study. Prognostic risk assessments were performed according to the Katagiri and revised Katagiri scoring system. In general, the low-risk group underwent resection and reconstruction with endoprosthetic replacement (EPR), while the high-risk group underwent internal fixation (IF) and radiation therapy. For the intermediate-risk group, the operative choice depended on the patient’s condition, degree of bone destruction, and radio-sensitivity. Overall survival, local failure, walking ability, and systemic complications were evaluated.Aims
Methods
The consensus is that bipolar hemiarthroplasty (BHA) in allograft-prosthesis composite (APC) reconstruction of the proximal femur following primary tumour resection provides more stability than total hip arthroplasty (THA). However, no comparative study has been performed. In this study, we have compared the outcome and complication rates of these two methods. In a retrospective study, 57 patients who underwent APC reconstruction of proximal femur following the primary tumour resection, either using BHA (29) or THA (28), were included. Functional outcome was assessed using the Musculoskeletal Tumour Society (MSTS) scoring system and Harris Hip Score (HHS). Postoperative complications of the two techniques were also compared.Aims
Methods
We reviewed retrospectively 236 consecutive patients seen in our soft-tissue sarcoma clinic. Of these, 95 had had a primary soft-tissue sarcoma excised elsewhere, but with histologically inadequate resection margins. All these patients had a secondary and wider re-excision. The tissues removed at the secondary re-excision were examined histologically for the presence of residual tumour. Definite tumour tissue was found in 29 of 55 lower-limb specimens, 16 of 25 upper-limb, 7 of 10 trunk and 4 of 5 head and
The aim of this study was to validate the Mirels score in predicting
pathological fractures in metastatic disease of the lower limb. A total of 62 patients with confirmed metastatic disease met
the inclusion criteria. Of the 62 patients, 32 were female and 30
were male. The mean age of patients was 65 years (35 to 89). The
primary malignancy originated from the breast in 27 (44%) patients,
prostate in 15 (24%) patients, kidney in seven (11%), and lung in
four (6%) of patients. One patient (2%) had metastatic carcinoma
from the lacrimal gland, two patients (3%) had multiple myeloma,
one patient (2%) had lymphoma of bone, and five patients (8%) had
metastatic carcinoma of unknown primary. Plain radiographs at the
time of initial presentation were scored using Mirels system by
the four authors. The radiographic components of the score (anatomical
site, size, and radiographic appearance) were scored two weeks apart.
Inter- and intraobserver reliability were calculated with Fleiss’
kappa test. Bland-Altman plots were created to compare the variances
of the individual components of the score and the total Mirels score.Aims
Patients and Methods
The aim of the study was to compare measures of the quality of
life (QOL) after resection of a chordoma of the mobile spine with
the national averages in the United States and to assess which factors
influenced the QOL, symptoms of anxiety and depression, and coping
with pain post-operatively in these patients. A total of 48 consecutive patients who underwent resection of
a primary or recurrent chordoma of the mobile spine between 2000
and 2015 were included. A total of 34 patients completed a survey
at least 12 months post-operatively. The primary outcome was the
EuroQol-5 Dimensions (EQ-5D-3L) questionnaire. Secondary outcomes were
the Patient-Reported Outcome Measurement Information System (PROMIS)
anxiety, depression and pain interference questionnaires. Data which
were recorded included the indication for surgery, the region of
the tumour, the number of levels resected, the status of the surgical
margins, re-operations, complications, neurological deficit, length
of stay in hospital and rate of re-admission.Aims
Patients and Methods
The purpose of the study was to investigate whether closed intramedullary
(IM) nailing with percutaneous cement augmentation is better than
conventional closed nailing at relieving pain and suppressing tumours
in patients with metastases of the femur and humerus. A total of 43 patients (27 men, 16 women, mean age 63.7 years,
standard deviation (Aims
Patients and Methods
Myxofibrosarcomas (MFSs) are malignant soft-tissue sarcomas characteristically
presenting as painless slowly growing masses in the extremities.
Locally infiltrative growth means that the risk of local recurrence
is high. We reviewed our experience to make recommendations about
resection strategies and the role of the multidisciplinary team
in the management of these tumours. Patients with a primary or recurrent MFS who were treated surgically
in our unit between 1997 and 2012 were included in the study. Clinical
records and imaging were reviewed. A total of 50 patients with a
median age of 68.4 years (interquartile range 61.6 to 81.8) were
included. There were 35 men; 49 underwent surgery in our unit.Aims
Patients and Methods
We report a prospective cohort study of the midterm results of
surgical dislocation of the hip (according to Ganz) to perform resection
of osteochondromas involving the femoral neck in patients with multiple
hereditary exostoses (MHE). Hip range of movement (ROM) was assessed pre- and post-operatively.
Patients’ judgment of post-operative reduction of pain, symptoms,
the Rand 36-item Health Survey (RAND-36) and complications were
analysed. Aims
Methods
The purpose of this study was to develop a prognostic model for
predicting survival of patients undergoing surgery owing to metastatic
bone disease (MBD) in the appendicular skeleton. We included a historical cohort of 130 consecutive patients (mean
age 64 years, 30 to 85; 76 females/54 males) who underwent joint
arthroplasty surgery (140 procedures) owing to MBD in the appendicular
skeleton during the period between January 2003 and December 2008.
Primary cancer, pre-operative haemoglobin, fracture Aims
Methods
We retrospectively reviewed the outcomes of 33
consecutive patients who had undergone an extra-articular, total or
partial scapulectomy for a malignant tumour of the shoulder girdle
between 1 July 2001 and 30 September 2013. Of these, 26 had tumours
which originated in the scapula or the adjacent soft tissue and
underwent a classic Tikhoff–Linberg procedure, while seven with
tumours arising from the proximal humerus were treated with a modified
Tikhoff-Linberg operation. We used a Ligament Advanced Reinforcement
System for soft-tissue reconstruction in nine patients, but not
in the other 24. The mean Musculoskeletal Tumor Society score (MSTS) was 17.6
(95% confidence interval (CI) 15.9 to 19.4); 17.6 (95% CI 15.5 to
19.6) after the classic Tikhoff–Linberg procedure and 18.1 (95%
CI 13.8 to 22.3) after the modified Tikhoff–Linberg procedure. Patients
who had undergone a LARS soft-tissue reconstruction had a mean score
of 18.6 (95% (CI) 13.9 to 22.4) compared with 17.2 (95% CI 15.5
to 19.0) for those who did not. The Tikhoff–Linberg procedure is a useful method for wide resection
of a malignant tumour of the shoulder girdle which helps to preserve
hand and elbow function. The method of soft-tissue reconstruction
has no effect on functional outcome. Cite this article:
Monostotic fibrous dysplasia of the proximal
femur has a variable clinical course, despite its reported limited tendency
to progress. We investigated the natural history and predisposing factors
for progression of dysplasia in a group of 76 patients with a mean
follow-up of 8.5 years (2.0 to 15.2). Of these, 31 (41%) presented
with an asymptomatic incidental lesion while 45 (59%) presented
with pain or a pathological fracture. A group of 23 patients (30%)
underwent early operative treatment for pain (19: 25%) or pathological
fracture (4: 5%). Of the 53 patients who were initially treated non-operatively,
45 (85%) remained asymptomatic but eight (15%) needed surgery because
of pain or fracture. The progression-free survival of the observation
group was 81% ( The risk of experiencing pain or pathological fracture is considerable
in monostotic fibrous dysplasia of the proximal femur. Patients
presenting with pain, a limp or radiological evidence of microfracture
have a high chance of needing surgical treatment. Cite this article:
In this case study, we describe the clinical
presentation and treatment of 36 patients with periosteal chondrosarcoma
collected over a 59-year period by the archive of the Netherlands
Committee on Bone Tumours. The demographics, clinical presentation,
radiological features, treatment and follow-up are presented with
the size, location, the histological grading of the tumour and the
survival. We found a slight predominance of men (61%), and a predilection
for the distal femur (33%) and proximal humerus (33%). The metaphysis
was the most common site (47%) and the most common presentation
was with pain (44%). Half the tumours were classified histologically
as grade 1. Pulmonary metastases were reported in one patient after
an intra-lesional resection. A second patient died from local recurrence
and possible pulmonary and skin metastases after an incomplete resection. It is clearly important to make the diagnosis appropriately because
an incomplete resection may result in local recurrence and metastatic
spread. Staging for metastatic disease is recommended in grade II
or III lesions. These patients should be managed with a contrast-enhanced MRI
of the tumour and histological confirmation by biopsy, followed
by Cite this article:
The purpose of this study was to assess whether
the use of a joint-sparing technique such as curettage and grafting was
successful in eradicating giant cell tumours of the proximal femur,
or whether an alternative strategy was more appropriate. Between 1974 and 2012, 24 patients with a giant cell tumour of
the proximal femur were treated primarily at our hospital. Treatment
was either joint sparing or joint replacing. Joint-sparing treatment
was undertaken in ten patients by curettage with or without adjunctive
bone graft. Joint replacement was by total hip replacement in nine patients
and endoprosthetic replacement in five. All 11 patients who presented
with a pathological fracture were treated by replacement. Local recurrence occurred in five patients (21%): two were treated
by hip replacement, three by curettage and none with an endoprosthesis.
Of the ten patients treated initially by curettage, six had a successful
outcome without local recurrence and required no further surgery.
Three eventually needed a hip replacement for local recurrence and
one an endoprosthetic replacement for mechanical failure. Thus 18
patients had the affected joint replaced and only six (25%) retained
their native joint. Overall, 60% of patients without a pathological
fracture who were treated with curettage had a successful outcome. Cite this article:
