The aim of this study is to determine the predictors of overall survival (OS) and predictive factors of poor prognosis of conventional high-grade osteosarcoma of the limbs in a single-centre in South Africa. We performed a retrospective cross-sectional analysis to identify the prognostic factors that predict the OS of patients with histologically confirmed high-grade conventional osteosarcoma of the limbs over ten years. We employed the Cox proportional regression model and the Kaplan-Meier method for statistical analysis.Aims
Methods
Low-grade central osteosarcoma (LGCOS), a rare type of osteosarcoma, often has misleading radiological and pathological features that overlap with those of other bone tumours, thereby complicating diagnosis and treatment. We aimed to analyze the clinical, radiological, and pathological features of patients with LGCOS, with a focus on diagnosis, treatment, and outcomes. We retrospectively analyzed the medical records of 49 patients with LGCOS (Broder’s grade 1 to 2) treated between January 1985 and December 2017 in a single institute. We examined the presence of malignant features on imaging (periosteal reaction, cortical destruction, soft-tissue invasion), the diagnostic accuracy of biopsy, surgical treatment, and oncological outcome.Aims
Methods
Cell-free DNA (cfDNA) and circulating tumour DNA (ctDNA) are used for prognostication and monitoring in patients with carcinomas, but their utility is unclear in sarcomas. The objectives of this pilot study were to explore the prognostic significance of cfDNA and investigate whether tumour-specific alterations can be detected in the circulation of sarcoma patients. Matched tumour and blood were collected from 64 sarcoma patients (n = 70 samples) prior to resection of the primary tumour (n = 57) or disease recurrence (n = 7). DNA was isolated from plasma, quantified, and analyzed for cfDNA. A subset of cases (n = 6) underwent whole exome sequencing to identify tumour-specific alterations used to detect ctDNA using digital droplet polymerase chain reaction (ddPCR).Aims
Methods
Aims Although chondrosarcomas (CSs) display true malignant features, including local recurrence (LR) and metastases, their behaviour in the hands and feet is thought to differ from that in other parts of the axial and appendicular skeleton by having a lower metastatic potential. The purpose of this study was to investigate the disease-specific and surgical factors that affect the local and systemic prognosis of CS of the hands and feet. A multicentre retrospective study was carried out at two tertiary sarcoma centres. A database search identified all patients with a CS treated between January 1995 and January 2018. There were 810 CSs of which 76 (9.4%) were located in the fingers, toes, metacarpals, and metatarsal bones. The median age of the study population was 55 years (36 to 68) with a median follow-up of 52 months (22 to 87) months. Overall, 70% of the tumours were in the hand (n = 54) and 30% in the foot (n = 22). Predictors for LR were margin (p = 0.011), anatomical location (p = 0.017), and method of surgical management (p = 0.003). Anatomical location (p = 0.026), histological grade between 1 and 3 (p = 0.004) or 2 and 3 (p = 0.016), and surgical management (p = 0.001) were significant factors for LR-free survival. Disease-specific survival was affected by histological grade (p < 0.001), but not by LR (p = 0.397).Methods
Results
Our aim was to develop and validate nomograms that would predict the cumulative incidence of sarcoma-specific death (CISSD) and disease progression (CIDP) in patients with localized high-grade primary central and dedifferentiated chondrosarcoma. The study population consisted of 391 patients from two international sarcoma centres (development cohort) who had undergone definitive surgery for a localized high-grade (histological grade II or III) conventional primary central chondrosarcoma or dedifferentiated chondrosarcoma. Disease progression captured the first event of either metastasis or local recurrence. An independent cohort of 221 patients from three additional hospitals was used for external validation. Two nomograms were internally and externally validated for discrimination (c-index) and calibration plot.Aims
Methods
The aim of this study was to identify factors that determine outcomes of treatment for patients with chondroblastic osteosarcomas (COS) of the limbs and pelvis. The authors carried out a retrospective review of prospectively collected data from 256 patients diagnosed between 1979 and 2015. Of the 256 patients diagnosed with COS of the pelvis and the limbs, 147 patients (57%) were male and 109 patients (43%) were female. The mean age at presentation was 20 years (0 to 90).Aims
Patients and Methods
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
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
Osteosarcoma of the pelvis is a particularly difficult tumour
to treat as it often presents late, may be of considerable size
and/or associated with metastases when it presents, and is frequently
chondroid in origin and resistant to chemotherapy. The aim of this study was to review our experience of managing
this group of patients and to identify features predictive of a
poor outcome. Between 1983 and 2014, 121 patients, (74 females and 47 males)
were treated at a single hospital: 74 (61.2%) patients had a primary
osteosarcoma and 47 (38.8%) had an osteosarcoma which was secondary
either to Paget’s disease (22; 18.2%) or to previous pelvic irradiation
(25; 20.7%). The mean age of those with a primary osteosarcoma was 29.3 years
(nine to 76) and their mean follow-up 2.9 years (0 to 29). The mean
age of those with a secondary sarcoma was 61.9 years (15 to 85)
and their mean follow-up was one year (0 to 14). A total of 22 patients with a primary sarcoma (52.4%) and 20
of those with a secondary sarcoma (47.6%) had metastases at the
time of presentation. Aims
Patients and Methods
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.
Dedifferentiated chordoma is a rare and aggressive
Between December 1995 and March 2003, 38 adult patients with intermediate or high-grade liposarcoma in a limb were treated by limb-sparing surgery and post-operative radiotherapy. The ten-year local recurrence-free survival was 83%, the ten-year metastasis-free survival 61%, the ten-year disease-free survival 51% and the ten-year overall survival 67%. Analysis of failure and success showed no association with the age of the patients, gender, the location of the primary tumour, the type of liposarcoma and the quality of resection. Our results indicate that liposarcoma may recur even ten years after the end of definitive therapy and may spread to unexpected sites as for soft-tissue sarcoma.
The most appropriate protocol for the biopsy of musculoskeletal tumours is controversial, with some authors advocating CT-guided core biopsy. At our hospital the initial biopsies of most musculoskeletal tumours has been by operative core biopsy with evaluation by frozen section which determines whether diagnostic tissue has been obtained and, if possible, gives the definitive diagnosis. In order to determine the accuracy and cost-effectiveness of this protocol we have undertaken a retrospective audit of biopsies of musculoskeletal tumours performed over a period of two years. A total of 104 patients had biopsies according to this regime. All gave the diagnosis apart from one minor error which did not alter the management of the patient. There was no requirement for re-biopsy. This protocol was more labour-intensive and 38% more costly than CT-guided core biopsy (AU$1804
