Aims

The epiphyseal approach to a chondroblastoma of the intercondylar notch of a child’s distal femur does not provide adequate exposure, thereby necessitating the removal of a substantial amount of unaffected bone to expose the lesion. In this study, we compared the functional outcomes, local recurrence, and surgical complications of treating a chondroblastoma of the distal femoral epiphysis by either an intercondylar or an epiphyseal approach.

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.

Background. Death within one year has been shown for some cancers to be an indicator of late presentation. They may have widespread disease by the time of diagnosis and many will have experienced delays in diagnosis. The aim of this study is to identify if this holds true for patients with bone and soft tissue sarcomas. Method. A prospective sarcoma database was used to identify all patients with a newly diagnosed bone or soft tissue sarcoma and to compare those who had died within one year with those who had survived for at least that time. Results. 5633 patients were included in the analysis of whom 3039 had a bone sarcoma and 2593 a soft tissue sarcoma. 839 patients (15%) died within one year of diagnosis, of whom 430 had a bone sarcoma (14%) and 409 a soft tissue sarcoma (16%). Significant differences (p⋋0.0001) were seen with age, size and metastases at diagnosis for bone sarcomas and age, size, symptom duration, metastases at diagnosis, a previous whoops excision and superficial tumours for soft tissue sarcomas. Conclusion. 15% of patients with a newly diagnosed sarcoma will be dead within one year. This appears to be associated with known poor prognostic factors (metastases at diagnosis, age and tumour size) and is not clearly associated with duration of symptoms. Whether it is a surrogate for delays in diagnosis has not been proved by this study

Bone and Soft Tissue Sarcomas represent approximately 1% of all malignant tumours. Delays in diagnosis are frequent and the average size of Sarcomas at diagnosis has averaged 10cm for many years. In 1999 guidance was produced by NICE with the aim of leading to the earlier diagnosis of common cancers – including Sarcomas. We have attempted to analyze whether this guidance has had any impact on either the size of the tumours at diagnosis or the symptom duration prior to diagnosis experienced by the patients. Data for patients referred to the Royal Orthopaedic Hospital in Birmingham between 1992 and 2007 with Bone Sarcomas (n=1592) and Soft Tissue Sarcomas (n=2004) were analysed to determine the effect of the guidance. For Bone Sarcomas the mean size of the tumours decreased from 11.2cm prior to the guidance to 10.7cm after the guidance but the change was not statistically significant (p=0.09). The mean duration of symptoms increased from 18 to 21.2 weeks (p=0.01). For Soft Tissue Sarcomas, mean size fell from 10.8cm to 9.5cm (p<0.001), however the duration of symptoms actually increased from 27.3 to 32.1 weeks (p=0.01). Statistical modelling using restricted cubic splines confirmed these trends in the data. These results show that whilst there may have been a slight improvement in the size at diagnosis of Soft Tissue Sarcomas, overall most patients still experience a long delay between the onset of symptoms and diagnosis and commencement of treatment. It is difficult to conclude that the early diagnosis guidance produced in 1999 has had a significant effect on the basis of this study. Strategies to improve awareness of the symptoms and clinical features of Bone and Soft Tissue Sarcomas are still urgently required

Aims

Giant cell tumours (GCTs) of the proximal femur are rare, and there is no consensus about the best method of filling the defect left by curettage. In this study, we compared the outcome of using a fibular strut allograft and bone cement to reconstruct the bone defect after extended curettage of a GCT of the proximal femur.

Aims. The primary aim of this study was to determine the effect of the duration of symptoms (DOS) prior to diagnosis on the overall survival in patients with a primary bone sarcoma. Patients and Methods. In a retrospective analysis of a sarcoma database at a single institution between 1990 and 2014, we identified 1446 patients with non-metastatic and 346 with metastatic bone sarcoma. Low-grade types of tumour were excluded. Our data included the demographics of the patients, the characteristics of the tumour, and the survival outcome of patients. Cox proportional hazards analysis and Kaplan–Meier survival analysis were performed, and the survivorship of the non-metastatic and metastatic cohorts were compared. Results. In the non-metastatic cohort, a longer DOS was associated with a slightly more favourable survival (hazard ratio (HR) 0.996, 95% confidence interval (CI) 0.994 to 0.998, p < 0.001). In all types of tumour, there was no difference in survival between patients with a DOS of greater than four months and those with a DOS of less than four months (p = 0.566). There was no correlation between the year of diagnosis and survival (p = 0.741). A diagnosis of chondrosarcoma (HR 0.636, 95% CI 0.474 to 0.854, p = 0.003) had the strongest positive effect on survival, while location in the axial skeleton (HR 1.76, 95% CI 1.36 to 2.29, p < 0.001) had the strongest negative effect on survival. Larger size of tumour (HR 1.05, 95% CI 1.03 to 1.06, p < 0.001) and increased age of the patient (HR 1.02, 95% CI 1.01 to 1.03, p < 0.001) had a slightly negative effect on survival. Metastatic and non-metastatic cohorts had similar median DOS (16 weeks, p = 0.277), although the median survival (15.5 months vs 41 months) and rates of survival at one year (69% vs 89%) and five years (20% vs 59%) were significantly shorter in the metastatic cohort. Conclusion. A longer DOS prior to diagnosis is not associated with a poorer overall survival in patients with a primary bone sarcoma. Location in the axial skeleton remains the strongest predictor of a worse prognosis. This may be helpful in counselling patients referred for evaluation on a delayed basis. Cite this article: Bone Joint J 2018;100-B:652–61

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.