Chondrosarcoma is the second most common primary sarcoma of bone: conventional chondrosarcoma accounts for 85% of all cases. Conventional chondrosarcoma may be central or peripheral. Most studies group central and peripheral chondrosarcomas together, although there is growing evidence that their clinical behaviour and prognosis differ. The aims of this study were to analyze any differences in characteristics between central and peripheral chondrosarcomas and to investigate the incidence and role of different syndromes. Data from two international tertiary referral sarcoma centres between January 1995 and December 2018 were retrospectively reviewed. The study population consisted of 714 patients with surgically treated conventional chondrosarcoma of the pelvis and limbs.Aims
Methods
Survival rates and local control after resection of a sarcoma of the pelvis compare poorly to those of the limbs and have a high incidence of complications. The outcome for patients who need a hindquarter amputation (HQA) to treat a pelvic sarcoma is poor. Our aim was to evaluate the patient, tumour, and reconstructive factors that affect the survival of the patients who undergo HQA for primary or recurrent pelvic sarcoma. We carried out a retrospective review of all sarcoma patients who had undergone a HQA in a supraregional sarcoma unit between 1996 and 2018. Outcomes included oncological, surgical, and survival characteristics.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 determine the risk of local recurrence and survival in patients with osteosarcoma based on the proximity of the tumour to the major vessels. A total of 226 patients with high-grade non-metastatic osteosarcoma in the limbs were investigated. Median age at diagnosis was 15 years (4 to 67) with the ratio of male to female patients being 1.5:1. The most common site of the tumour was the femur (n = 103) followed by tibia (n = 66). The vascular proximity was categorized based on the preoperative MRI after neoadjuvant chemotherapy into four types: type 1 > 5 mm; type 2 ≤ 5 mm, > 0 mm; type 3 attached; type 4 surrounded.Aims
Patients and Methods
The purpose of this study was to describe the effect of histological
grade on disease-specific survival in patients with chondrosarcoma. A total of 343 patients with a chondrosarcoma were included.
The histological grade was assessed on the initial biopsy and on
the resection specimen. Where the histology showed a mixed grade,
the highest grade was taken as the definitive grade. When only small
focal areas showed higher grade, the final grade was considered
as both.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
The purpose of this study was to review a large cohort of patients
and further assess the correlation between the histological response
to chemotherapy in patients with Ewing’s sarcoma with the overall
(OS) and event-free survival (EFS). All patients treated for Ewing’s sarcoma between 1980 and 2012
were reviewed. Of these, 293 patients without metastases at the
time of diagnosis and treated with chemotherapy and surgery were
included. Patients were grouped according to the percentage of necrosis
after chemotherapy: Group I: 0% to 50%, Group II: 51% to 99% and Group
III: 100%.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
The aim of this study was to evaluate the prognostic
and therapeutic factors which influence the oncological outcome
of parosteal osteosarcoma. A total of 80 patients with a primary parosteal osteosarcoma
were included in this retrospective study. There were 51 females
and 29 males with a mean age of 29.9 years (11 to 78). The mean follow-up was 11.2 years (1 to 40). Overall survival
was 91.8% at five years and 87.8% at ten years. Local recurrence
occurred in 14 (17.5%) patients and was associated with intralesional
surgery and a large volume of tumour. On histological examination,
80% of the local recurrences were dedifferentiated high-grade tumours.
A total of 12 (14.8%) patients developed pulmonary metastases, of
whom half had either a dedifferentiated tumour or a local recurrence.
Female gender and young age were good prognostic factors. Local
recurrence was a poor prognostic factor for survival. Medullary
involvement or the use of chemotherapy had no impact on survival. The main goal in treating a parosteal osteosarcoma must be to
achieve a wide surgical margin, as inadequate margins are associated
with local recurrence. Local recurrence has a significant negative
effect on survival, as 80% of the local recurrences are high-grade
dedifferentiated tumours, and half of these patients develop metastases. The role of chemotherapy in the treatment of parosteal osteosarcoma
is not as obvious as it is in the treatment of conventional osteosarcoma.
The mainstay of treatment is wide local excision. Cite this article:
We identified eight patients of 2900 with a primary malignant bone tumour who had coexisting neurofibromatosis type 1. This was a much higher incidence than would be expected by chance. The patients had a mean age of 22.4 years (9 to 54): five were male. Two patients subsequently developed a second bone sarcoma, one of which was radiation induced. Four of the primary tumours were osteosarcomas, four were spindle-cell sarcomas and one a Ewing’s sarcoma. All the patients were treated with chemotherapy and surgery: six of the eight appear to be cured. This study suggests a possible relationship between neurofibromatosis type 1 and the development of a bone sarcoma, the increased risk being estimated at eight times that of the normal population. We recommend that further research into this possible link should be considered.
