We reviewed our experience with diaphyseal endoprostheses to determine the survival of this type of reconstruction and factors affecting that survival.
The weights of evidence (WE) are logs of the likelihood ratios and can be added and a probability then calculated. e.g. a 36 yr old with a 10cm, deep, painless lump that is increasing in size scores −0.39 + 0.4 + 0.4 – 0.11 + 0.58 = 0.88. This equates to a risk of the lump being malignant of 70%.
The generally unfavourable prognosis and perceived risks have led surgeons to palliate, stabilise in situ or amputate for distal femoral metastases despite recognised morbidity and life style restrictions. We conclude that DF EPR should be considered as a limb salvage option in patients with distal femoral mets.
The purpose of this paper is to present the rare occurrence of clear cell chondrosarcoma in a patient suffering from Niemann-Pick disease (NPD). NPD is a rare autosomal recessive inborn error of metabolism. It is a lysosomal storage disorder affecting children and adolescents often causing death in early childhood although in milder form patient may survive up to adulthood. Although chondrosarcoma is a relatively common malignant bone lesion, these lesions rarely affect patients suffering from Niemann-Pick disease. This article presents the case of a 50-year-old man with a chondrosarcoma in the olecranon of the left ulna who also suffered from milder form of Niemann-Pick disease. Radiography and MRI showed cortical disruption and an expansile lesion. Histologically, the tumour was classified as grade 2 clear cell chondrosarcoma with classical differentiation according to the American Joint Committee of Cancer pathologic staging system. Amputation of an above elbow type was performed to obtain clear surgical margins. A case report and discussion of this rare association of a patient suffering from milder form of NPD who developed a clear cell chondrosarcoma is presented here. NPD and clear cell chondrosarcoma are rare diseases and together their occurrence was not found reported in the literature. NPD patients rarely live to the age of 50 years. This raises a possibility that if Niemann-Pick patients survived long enough they might develop chondrosarcoma. Alternatively this could just be coincidental. More research is needed to clarify this possibility.
130 consecutive patients with metastatic tumours of the extremity bones treated with resection with or without major endoprosthetic reconstruction were studied retrospectively to determine the indication for surgery, complications, clinical outcome and oncological results of treatment. The mean age at diagnosis was 61 (22 – 84). The tumours originated from a variety of organs. Lower extremity was involved in 104 and upper extremity in 26. Metastatic disease was solitary in 55 patients and multiple in 75 at the time of surgery. The median follow-up possible from the time of operation to review was 18 months (0–103) The indication for surgery was radical treatment of solitary metastases with curative intent in 33, pathological fracture in 46, impending fracture in 27, failure of prior fixation devices in 17, painful swelling or extremity in 37. Surgical treatment included excision of expendable bones without reconstruction in 20 patients and resection with endoprosthetic reconstruction in 110 patients. 7 patients received adjuvant chemotherapy and the majority received adjuvant radiotherapy. At the time of review, 58 patients had died at a mean time of 23 months (0–90) from surgery (53 from progressive metastatic disease and 5 from other causes). 72 were alive at mean follow-up of 22 months (1–103) from surgery. 36 patients (28%) were alive at 2 years post-surgery and 8 (6%) at 5 years. One patient died intra-operatively. Post-operative complications occurred in 32 patients (25%). 18 patients required further surgical procedures for dislocation, infection haematoma, stiff joint, plastic surgical procedures. All the patients had control of pain and 90% achieved desired mobility. There was no difference in the survival of patients who presented with solitary and multiple metastases, renal and non-renal metastases, and upper or lower limb metastases. We conclude that selected patients with bone metastases can benefit from resection and major bone reconstruction with acceptable morbidity. We have not identified predictable prognostic factors in these selected patients.