Osteofibrous dysplasia (OFD) is a rare benign lesion predominantly affecting the tibia in children. Its potential link to adamantinoma has influenced management. This international case series reviews the presentation of OFD and management approaches to improve our understanding of OFD. A retrospective review at three paediatric tertiary centres identified 101 cases of tibial OFD in 99 patients. The clinical records, radiological images, and histology were analyzed.Aims
Methods
We have evaluated the survivorship, outcomes, and failures of an interlocking, reconstruction-mode stem-sideplate implant used to preserve the native hip joint and achieve proximal fixation when there is little residual femur during large endoprosthetic reconstruction of the distal femur. A total of 14 patients underwent primary or revision reconstruction of a large femoral defect with a short remaining proximal femur using an interlocking, reconstruction-mode stem-sideplate for fixation after oncological distal femoral and diaphyseal resections. The implant was attached to a standard endoprosthetic reconstruction system. The implant was attached to a standard endoprosthetic reconstruction system. None of the femoral revisions were amenable to standard cemented or uncemented stem fixation. Patient and disease characteristics, surgical history, final ambulatory statusAims
Methods
Rarely, the extent of a malignant bone tumour
may necessitate resection of the complete humerus to achieve adequate
oncological clearance. We present our experience with reconstruction
in such cases using a total humeral endoprosthesis (THER) in 20
patients (12 male and eight female) with a mean age of 22 years
(6 to 59). We assessed the complications, the oncological and functional
outcomes and implant survival. Surgery was performed between June
2001 and October 2009. The diagnosis included osteosarcoma in nine,
Ewing’s sarcoma in eight and chondrosarcoma in three. One patient
was lost to follow-up. The mean follow-up was 41 months (10 to 120)
for all patients and 56 months (25 to 120) in survivors. There were
five local recurrences (26.3%) and 11 patients were alive at time
of last follow-up, with overall survival for all patients being
52% (95% confidence interval (CI) 23.8 to 74) at five years. The
mean Musculoskeletal Tumor Society score for the survivors was 22
(73%; 16 to 23). The implant survival was 95% (95% CI 69.5 to 99.3)
at five years. The use of a THER in the treatment of malignant tumours of bone
is oncologically safe; it gives consistent and predictable results
with low rates of complication.
