Introduction and Aim: The purpose of this study is to demonstrate that definitive surgery (extraperiosteal excision) is required in patients with osteofibrous dysplasia (OFD) due to the risk of recurrence and co-existent adamantinoma. OFD is an unusual childhood condition, which almost exclusively affects the tibia. It is thought to follow a slowly progressive course and to stabilise after skeletal maturity. The possible link with adamantinoma is controversial, with some authors believing that they are part of one histological process. This therefore provides difficulty in recommending treatment options.

Method and Results: A retrospective review of OFD was conducted. Using the Stanmore Bone Tumor Unit database, 22 cases were identified who were initially diagnosed with OFD or were diagnosed on final histology. All cases were tibial except one lesion in the ulna and one in the fibula. Management was diverse, depending on the severity of symptoms and the extent of the lesions encountered. Definitive (extraperiosteal) surgery in the majority of our patients was localised excision for small lesions (less than 50% of the bony circumference) and segmental excision followed by reconstructive surgery for more extensive ones. Seven patients had a ‘sharkbite’ excision and a further seven were treated with fibula autografting. Of the latter group, one required further excision and bone transport due to recurrence of OFD. An additional five underwent bone transport and distraction osteogenesis using the Ilizarov technique and one had a proximal tibial replacement. Nine initially underwent curettage, but eight recurred (recurrence rate 88.9%). No recurrences occurred following localised extraperiosteal excisions and bone transport. There were three confirmed cases of adamantinoma

Conclusion: In view of the risk of association of OFD with adamantinoma, and to some extent the continuous morbidity of OFD if left untreated, we believe that radical extraperiosteal excision is indicated in most if not all cases of OFD

Aim: Sacral tumours are rare and can form a wide variety of differential diagnoses. We present a series of sacral tumour patients treated at a regional tumour centre; describing our experience of their management. Method: A retrospective study reviewing 76 sacral tumour patients, presenting to the Royal National Orthopaedic Hospital, Stanmore, from April 1976 to April 2002. The minimum follow-up period was 6 months. For each tumour type we looked at the incidence, diagnosis and outcome. Results: 69 of the lesions were primary bone tumours, 3 metastatic and 4 haematopoietic tumours. 33% of all tumours were chordomas. Osteosarcoma (10%), chondrosarcoma (8%) and giant cell tumour (8%) were the next most common. The commonest presenting symptom was lower back pain (64 cases). Good survival was demonstrated with chordomas and giant cell tumours. Osteosarcomas and chondrosarcomas had poor survival. Tissue diagnosis was accurately achieved with image-guided needle biopsy (61 cases). Magnetic resonance imaging (MRI) and computed tomography (CT) provided sufficient details for preoperative planning. Conclusion: The symptoms and signs of sacral tumours are non-specific and may lead to a misdiagnosis of degenerative disease of the spine. In our series chordomas account for only a third of all sacral tumours. Early diagnosis and staging are essential in order to determine definitive management and infl uence outcome. Surgery remains the most effective method for treating the malignant tumours.