Aims. 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. Methods. 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. Results. This study comprised 77 patients at a three-year minimum follow-up. The predictors of poor OS were: the median age of ≤ 19 years (hazard ratio (HR) 0.96; 95% confidence interval (CI) 0.92 to 0.99; p = 0.021); median duration of symptoms ≥ five months (HR 0.91; 95% CI 0.83 to 0.99; p < 0.037); metastasis at diagnosis (i.e. Enneking stage III) (HR 3.33; 95% CI 1.81 to 6.00; p < 0.001); increased alkaline phosphatase (HR 3.28; 95% CI 1.33 to 8.11; p < 0.010); palliative treatment (HR 7.27; 95% CI 2.69 to 19.70); p < 0.001); and amputation (HR 3.71; 95% CI 1.12 to 12.25; p < 0.032). In contrast, definitive surgery (HR 0.11; 95% CI 0.03 to 0.38; p < 0.001) and curative treatment (HR 0.18; 95% CI 0.10 to 0.33; p < 0.001) were a protective factor. The Kaplan-Meier median survival time was 24 months, with OS of 57.1% at the three years. The projected five-year event-free survival was 10.3% and OS of 29.8% (HR 0.76; 95% CI 0.52 to 1.12; p = 0.128). Conclusion. In this series of high-grade conventional osteosarcoma of the appendicular skeleton from South Africa, 58.4% (n = 45) had detectable metastases at presentation; hence, an impoverished OS of five years was 29.8%. Large-scale future research is needed to validate our results. Cite this article: Bone Jt Open 2024;5(3):210–217

Aims. The purpose of this study was to clarify the clinical behaviour, prognosis, and optimum treatment of dedifferentiated low-grade osteosarcoma (DLOS) diagnosed based on molecular pathology. Patients and Methods. We retrospectively reviewed 13 DLOS patients (six men, seven women; median age 32 years (interquartile range (IQR) 27 to 38)) diagnosed using the following criteria: the histological coexistence of low-grade and high-grade osteosarcoma components in the lesion, and positive immunohistochemistry of mouse double minute 2 homolog (MDM2) and cyclin-dependent kinase 4 (CDK4) associated with MDM2 amplification. These patients were then compared with 51 age-matched consecutive conventional osteosarcoma (COS) patients (33 men, 18 women; median age 25 years (IQR 20 to 38)) regarding their clinicopathological features. Results. The five-year overall survival (OAS) rates in the DLOS and COS patients were 85.7% and 77.1% (p = 0.728), respectively, and the five-year progression-free survival (PFS) rates were 57.7% and 44.9% (p = 0.368), respectively. A total of 12 DLOS patients received chemotherapy largely according to regimens for COS. Among the nine cases with a histological evaluation after chemotherapy, eight showed a poor response, and seven of these had a necrosis rate of < 50%. One DLOS patient developed local recurrence and five developed distant metastases. Conclusion. Based on our study of 13 DLOS cases that were strictly defined by histological and molecular means, DLOS showed a poorer response to a standard chemotherapy regimen than COS, while the clinical outcomes were not markedly different. Cite this article: Bone Joint J 2019;101-B:745–752

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: Bone Joint J 2015;97-B:1698–1703

AIMS: Parosteal osteosarcoma is a surface osteogenic sarcoma less agressive than conventional osteosarcoma. Most cases begin to show symptoms in the third and fourth decades, and it affects females more often than males. The tumor affected long bones of the limbs, with evident predilection for the distal femur. The aim of this study is to analyze the prognosis of parosteal osteosarcoma, studying its evolution to know its better treatment. Methods: This is a retrospective study of 25 patients with parosteal osteosarcoma. 23 cases have been treated at our institution from 1983 through June 2009, and 2 cases were treated in another center with a follow-up of at least 1 year. We studied the relation between different aspects of these patients: time of evolution, medullar canal invasion, size of tumor, dedifferentiated parosteal osteosarcoma or recurrence, images of RMI and kind of parosteal osteosarcoma, diagnoses with biopsy, relation between surgery and recurrences. Results: We had 4 men and 21 women with a mean age of 30,48 years (13–56).6 cases were dedifferentiated parosteal osteosarcoma. We observed hyperintensity sign in 57,1% cases of dedifferentiated parosteal osteosarcoma. From 18 trephine biopsies, only 9 cases were diagnosed with parosteal osteosarcoma, 8 cases out of 8 incisional biopsies were also diagnosed as parosteal osteosarcoma. There were 5 local recurrences in the first year follow-up. All patients with marginal excision recurred. All patients were alive, free from disease and without metastatic lesions when the study finished. There was relation between the time of evolution of the tumor and the medular canal invasion and the dedifferentiation, but we not found any relation with the tumoral recurrence. The presence of dedifferentiation and the recurrence were more common in bigger tumours. 3 patients were treated with chemotherapy. Conclusions: Parosteal osteosarcoma is a slow-growing tumor with a significantly better prognosis than the conventional osteosarcoma. The diagnosis of parosteal osteosarcoma requires an overall evaluation of the clinical, radiology and pathology findings. The initial study by RMI could help to guide our selective biopsy. First surgical procedure performed is a very important prognosis factor. Wide resection allows a better local control of the disease with fewer recurrences. Time of evolution and canal medullar invasion are more frequent in dedifferentiated parosteal osteosarcoma, but they not affect the rate of recurrence if the initial resection has been appropiate. In our opinion, postoperative chemotherapy should be given to patients with dedifferentiation and canal medular invasion in parosteal osteosarcoma

The aim of this study was to determine whether the level of circulating C-reactive protein (CRP) before treatment predicted overall disease-specific survival and local tumour control in patients with a sarcoma of bone. We retrospectively reviewed 318 patients who presented with a primary sarcoma of bone between 2003 and 2010. Those who presented with metastases and/or local recurrence were excluded. Elevated CRP levels were seen in 84 patients before treatment; these patients had a poorer disease-specific survival (57% at five years) than patients with a normal CRP (79% at five years) (p < 0.0001). They were also less likely to be free of recurrence (71% at five years) than patients with a normal CRP (79% at five years) (p = 0.04). Multivariate analysis showed the pre-operative CRP level to be an independent predictor of survival and local control. Patients with a Ewing’s sarcoma or chondrosarcoma who had an elevated CRP before their treatment started had a significantly poorer disease-specific survival than patients with a normal CRP (p = 0.02 and p < 0.0001, respectively). Patients with a conventional osteosarcoma and a raised CRP were at an increased risk of poorer local control. We recommend that CRP levels are measured routinely in patients with a suspected sarcoma of bone as a further prognostic indicator of survival. Cite this article: Bone Joint J 2013;95-B:411–18

Aims

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.

The October 2023 Oncology Roundup³⁶⁰ looks at: Are pathological fractures in patients with osteosarcoma associated with worse survival outcomes?; Spotting the difference: how secondary osteosarcoma manifests in retinoblastoma survivors versus conventional cases; Accuracy of MRI scans in predicting intra-articular joint involvement in high-grade sarcomas around the knee; Endoprosthetic reconstruction for lower extremity soft-tissue sarcomas with bone involvement; Local relapse of soft-tissue sarcoma of the extremities or trunk wall operated on with wide margins without radiation therapy; 3D-printed, custom-made prostheses in patients who had resection of tumours of the hand and foot; Long-term follow-up for low-grade chondrosarcoma; Evaluation of local recurrence and diagnostic discordance in chondrosarcoma patients undergoing preoperative biopsy; Radiological scoring and resection grade for intraosseous chondrosarcoma.

Intracortical osteosarcoma is a comparatively rare and distinct tumor separated from conventional osteosarcoma and other osteosarcoma variants. At first, the lesion was described in two patients by Jaffe in 1960. To date, 17 cases have been described in the literature. In all cases, the tumor occured in the cortex of the shaft of the femur or tibia. En-bloc resection was performed as primary therapeutic approach in most cases, sometimes in combination with neoadjuvant or adjuvant chemotherapy. We, for the first time, describe a case of intracortical osteosarcoma of the midshaft of the right ulna which was found accidentaly in a patient taking part in a screening programm for heriditary breast cancer. The patient underwent en-bloc resection without additional chemotherpapy and shows no reccurence after 18-months follow up

The clinical, radiographic and pathological features are described of eight cases of a bone tumour which we propose to classify as "malignant osteoblastoma". It presents the characteristics of genuine osteoblastoma but of an aggressive pattern, with more abundant and often plump hyperchromatic nuclei, greater nuclear atypia, and numerous giant cells of osteoclastic type. This rare tumour is regarded as the malignant counterpart of osteoblastoma and appears to be only locally aggressive. It should be separated from conventional osteosarcoma not only because of its peculiar histological pattern, but also because of its different clinical and radiological features and better prognosis. Thus seven of the eight patients were alive and free of disease from one and a half to eleven years after the initial surgical treatment, which in only two cases included amputation. Excision or block resection is the preferred method of treatment

Introduction. Spinal osteosarcomas are quite rare and the optimal treatment strategy is unknown. We report a series of 9 cases of osteosarcoma of the spine treated with intralesional resection and adjuvant combination therapy in order to evaluate their clinico-pathological correlation, recurrence rate and survival. Materials and Methods. Between 1980 and 2009, nine histologically confirmed cases of primary conventional osteogenic sarcoma of the spine were identified from Scottish Bone Tumour Registry. This prospectively collected registry database was retrospectively reviewed. Results. There were 9 cases with male predominance (77.7%) at a median age of 39 years (range, 16 – 73 years) and were anatomically distributed as 2-cervical, 6-thoracic, 1-lumbar and 1-sacral. Local and distant staging and histological grading was carried out in all. Seven patients underwent intralesional (including open biopsies) and 2 marginal resections. Seven patients received adjuvant chemotherapy and 8 adjuvant radiotherapy. The overall local recurrence rate was 66.6%. All but one patient died of the disease with a median length of survival of 30 months. The 2-year and 5-year survival rates were 66.6% and 22.2% respectively. Two patients who survived more than 5 years were male patients below the age of 40 years involving thoracic spine (one had a marginal resection without receiving any adjuvant therapy subsequently had a recurrence; other one underwent intralesional resection with adjuvant chemo- and radiotherapy, died with no evidence of disease). Conclusions. Spinal osteosarcoma in Scottish patients showed a higher median age, thoracic spine and male gender predilection with overall poor prognosis. Local recurrence did not affect the survival adversely, however patients older than 40 years and metastases at presentation showed poor survival compared to age under 40 and non-metastatic presentations

Osteosarcoma is the most common second malignancy seen in retinoblastoma survivors. Risk of developing osteosarcoma in this group is estimated approximately 500 times higher than the general population. Prognosis in this setting has been reported significantly worse than conventional osteosarcoma despite multimodal management. Purpose of this study was to evaluate clinical features, molecular aspects and outcome of treatment in this subgroup of osteosarcoma patients. Between 1985 and 2004, from a total of about 1100 osteosarcomas, 7 survivors of retinoblastoma developing high-grade osteosarcoma as second malignancy presented at the authors’ Institution. Retrospective study was undertaken to analyze presentation, tissue expression of RB1, P53, PGP and DHFR, treatment and outcome of both retinoblastoma and osteosarcoma. Retinoblastoma was bilateral in 5 cases and unilateral in two. All the patients had been treated with a combination of surgery +/− chemotherapy +/− radiation. None of them had evidence of retinoblastoma at the time of second malignancy diagnosis. Average age at diagnosis of osteosarcoma was 14 years (9–17 years), mean interval between the two malignancies was 155 months. All the osteosarcomas were in the appendicular skeleton, all but one around the knee. Molecular analysis showed defective RB1 gene in all cases All the seven patients received contemporary multimodal management for osteosarcoma. All but one patient died of osteosarcoma within 30 months from diagnosis. The living patient had local recurrence 9 years after limb salvage and is currently disease free following amputation. Prognosis of osteosarcoma in retinoblastoma patients remains poor as compared to conventional high grade osteosarcoma despite multimodal management. No obvious correlation was found between poor prognosis and P53, PGP and DHFR expression

Purpose: To evaluate the clinico-pathological features and outcome of osteosarcoma in patients over the age of 40 in Scotland. Methods: A retrospective review was performed using data collected by the Scottish Bone Tumour Registry on patients diagnosed with osteosarcoma over the age of 40 between 1960 and 2004. Information about tumour location, age of diagnosis, gender, lung metastasis, and survival was analysed. Histological slides were reviewed again and the diagnosis of osteosarcoma confirmed. The overall survival was calculated using Kaplan-Meier survival curves. Results: 145 patients were identified. 78 patients had malignant change in pre-existing Paget’s disease. 60 patients had osteosarcoma and 18 malignant fibrous histiocytoma. Average age of diagnosis of Paget’s osteosarcoma was 67.8 years, male to female ratio of 2:1 and 27% of cases were within the pelvis. Median survival was 6 months. 30% had lung metastasis at presentation. 54 patients had conventional osteosarcoma. Average age of diagnosis of 58.8 years, male to female ratio of 3:2 and 37% were femoral. Median survival was 11 months. 13 patients had radiation-induced osteosarcoma. Average age of diagnosis of 67.2, male to female ratio of 1:6 and 5 out of the 13 had pelvic osteosarcoma. Median survival was 8 months. Conclusion: We present the clinico-pathological features and outcome of osteosarcoma in patients over the age of 40 in Scotland between 1960 and 2004. Pelvic disease and metastasis at presentation were prevalent in patients with Paget’s and radiation-induced osteosarcoma reflecting their poor outcome

Introduction: Only 4–13 % of all spine tumours are primary bone tumours. We report on 180 cases of primary malignant bone tumours of the spine from the Scottish Bone Tumour Registry. The aim of the study was to analyse the incidence, demography, pathology and survival patterns of primary malignant bone tumours of the vertebral column. Materials and Methods: All of the data in the Scottish Bone Tumour Registry is compiled prospectively. This report is based on a retrospective review of all the data from 180 cases of primary malignant bone tumours (excluding metastatic lesions). Results: Of 4,301 registry cases 4.1% were primary malignant lesions (n=180). Seventy two percent of all spinal tumours were primary malignant neoplasms. There were 22 cervical, 72 thoracic, 45 lumbar and 41 sacrococcygeal lesions. There was a male preponderance (103 males & 77 females). The mean age at presentation was 54 years (range, 4–86 years). The top two ranked tumours were myeloma (42) and chordoma (41). Ewing’ sarcoma (15), Leukaemik-Lymphomatous lesions (13), conventional osteosarcoma (10) and Paget’s sarcoma (9) followed thereafter. The predominant presenting symptom was pain. Pathological fracture occurred in 7 patients. The operative treatment consisted of curettage (21), excision (51) and resection (9) with supplemented bone grafting (13). Adjuvant chemo (=61) and radiotherapy (=131) was also used in selective cases. Thirty patients were alive with no evidence of disease at a mean 5 year follow-up. Six were alive with persistent primary disease and/or local recurrence and/or metastases at the time of review. Eighty four patients died with persistent primary disease, 30 patients died of metastatic disease, 9 due to local recurrence and 17 of unrelated causes. Conclusions: Only 4.1% of the musculoskeletal tumours were spinal: 40% involved the thoracic spine. Mean age at presentation was 54 years. Myeloma, chordoma, chondrosarcoma and Ewing’ sarcoma were the most common pathologies. Myeloma predominated: osteosarcoma was much less common in our series compared to previous reports. Early diagnoses resulted into improved outcome

Aims

Resection of the proximal humerus for the primary malignant bone tumour sometimes requires en bloc resection of the deltoid. However, there is no information in the literature which helps a surgeon decide whether to preserve the deltoid or not. The aim of this study was to determine whether retaining the deltoid at the time of resection would increase the rate of local recurrence. We also sought to identify the variables that persuade expert surgeons to choose a deltoid sparing rather than deltoid resecting procedure.

We identified 42 patients who presented to our unit over a 27-year period with a secondary radiation-induced sarcoma of bone. We reviewed patient, tumour and treatment factors to identify those that affected outcome. The mean age of the patients at presentation was 45.6 years (10 to 84) and the mean latent interval between radiotherapy and diagnosis of the sarcoma was 17 years (4 to 50). The median dose of radiotherapy given was estimated at 50 Gy (mean 49; 20 to 66). There was no correlation between radiation dose and the time to development of a sarcoma. The pelvis was the most commonly affected site (14 patients (33%)). Breast cancer was the most common primary tumour (eight patients; 19%). Metastases were present at diagnosis of the sarcoma in nine patients (21.4%). Osteosarcoma was the most common diagnosis and occurred in 30 cases (71.4%). Treatment was by surgery and chemotherapy when indicated: 30 patients (71.4%) were treated with the intention to cure. The survival rate was 41% at five years for those treated with the intention to cure but in those treated palliatively the mean survival was only 8.8 months (2 to 22), and all had died by two years. The only factor found to be significant for survival was the ability to completely resect the tumour. Limb sarcomas had a better prognosis (66% survival at five years) than central ones (12% survival at five years) (p = 0.009).

Radiation-induced sarcoma is a rare complication of radiotherapy. Both surgical and oncological treatment is likely to be compromised by the treatment received previously by the patient.

Between 1986 and 2002, 42 patients with synchronous multifocal osteosarcoma were treated with two different protocols of neoadjuvant chemotherapy. When feasible, the primary and secondary tumours were excised as a combined procedure.

After initial chemotherapy 26 patients were excluded from simultaneous excision of all their secondary bone lesions as their disease was too advanced. In 12 patients only isolated excision of the primary lesion was possible. For 16 patients simultaneous operations were conducted to excise the primary and secondary lesions. This involved two supplementary sites in 15 patients and four additional sites in one patient. Of these, 15 attained remission but 12 relapsed and died (11 within two years). Three patients remained disease-free at five, six and 17 years. The histological response to pre-operative chemotherapy of the primary and secondary lesions was concordant in 13 of the 16 patients who underwent simultaneous operations at more than one site.

The prognosis for synchronous multifocal osteosarcoma remains poor despite combined chemotherapy and surgery. The homogeneous histological responses in a large proportion of the primary and secondary lesions implies that synchronous multifocal osteosarcoma tumours are not multicentric in origin, but probably represent bone-to-bone metastases from a single tumour.