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
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A number of risk factors based upon mostly retrospective surgical data, have been formulated in order to identify impending pathological fractures of the femur from low-risk metastases. We have followed up patients taking part in a randomised trial of radiotherapy, prospectively, in order to determine if these factors were effective in predicting fractures. In 102 patients with 110 femoral lesions, 14 fractures occurred during follow-up. The risk factors studied were increasing pain, the size of the lesion, radiographic appearance, localisation, transverse/axial/circumferential involvement of the cortex and the scoring system of Mirels. Only axial cortical involvement >
30 mm (p = 0.01), and circumferential cortical involvement >
50% (p = 0.03) were predictive of fracture. Mirels’ scoring system was insufficiently specific to predict a fracture (p = 0.36). Our results indicate that most conventional risk factors overestimate the actual occurrence of pathological fractures of the femur. The risk factor of axial cortical involvement provides a simple, objective tool in order to decide which treatment is appropriate.
Low-grade surface tumours of bone may theoretically be treated by hemicortical resection, retaining part of the circumference of the cortex. An inlay allograft may be used to reconstruct the defect. Since 1988 we have performed 22 hemicortical procedures in selected patients with low-grade parosteal osteosarcoma (6), peripheral chondrosarcoma (6) and adamantinoma (10). Restricted medullary involvement was not a contraindication for this procedure. There was no evidence of local recurrence or distant metastasis at a mean follow-up of 64 months (27 to 135). Wide resection margins were obtained in 19 patients. All allografts incorporated completely and there were no fractures or infections. Fractures of the remaining hemicortex occurred in six patients and were managed successfully by casts or by osteosynthesis. The functional results were excellent or good in all except one patient. Hemicortical procedures for selected cases of low-grade surface tumours give excellent oncological and functional outcomes. There was complete remodelling and fewer complications when compared with larger intercalary procedures. The surgery is technically demanding but gives good clinical results.