Introduction: 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. 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. Methods: Using our database 22 cases of OFD were identified. Management was diverse. Results: Definitive (extraperiosteal) surgery, in the majority of our patients, was localized 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 “shark-bite” 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. Five underwent bone transport & distraction osteogenesis and one had a proximal tibial replacement. Nine initially underwent curettage, but eight recurred. No recurrences occurred following localized extraperiosteal excisions and bone transport. There were three confirmed cases of adamantinoma. Discussion: 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

A nine-year-old girl presented with a four-year history of progressive bowing of the left tibia. She had been seen in our clinic three and four years earlier, when no treatment was advised. She had been complaining of mild pain in the left leg for one month but was otherwise not very perturbed about her deformed leg. Examination showed bowing of the left tibia, no leg length discrepancy, no limp, and a normal left knee and ankle. Radiological examination showed features of osteo-fibrous dysplasia of the left tibia, with eccentric expansion of the cortex, intracortical osteolysis, marginal sclerosis encroaching on the medulla and diffuse lesional calcification. No other bones were involved. Because of the progressive bowing of the tibia and the mild pain, the recommended loose observation of the patient was abandoned. Daily treatment with 30 mg IVI pamidronate for three days resolved the pain. One month after the latest presentation the lesion was biopsied. Histological examination confirmed the diagnosis of osteofibrous dysplasia of the tibia. There was no evidence of adamantinoma. The literature on this rare bone lesion in childhood supports the use of open biopsy if the deformity becomes painful, the bowing is progressive and the patient presents after the age of nine years. Important differential diagnoses include fibrous dysplasia and adamantinoma

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. A retrospective review of OFD was conducted. Using the Stanmore Bone Tumour 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 localized 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 & 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 localized extraperiosteal excisions and bone transport. There were three confirmed cases of adamantinoma. 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

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

Aims: Purpose of this study was to obtain long term follow-up in patients with Osteofibrous Dysplasia (OFD), in order to investigate natural history of the disease, late results of treatment, and potential risk of Adaman-tinoma development in this setting. Methods: A retrospective study of 48 patients with histologically proven OFD observed at our Institution between 1900 and 1997 was undertaken. Clinico-pathologic features of all cases were reviewed and found consistent with OFD. A clinical status update and current radiographs were obtained in all patients. A subgroup of 21 patients with minimum follow-up of 20 years (21 to 44 years, average 27) was analysed for functional result and adamantinoma development. Functional result according to MTS-ISOLS score correlated with surgical aggressiveness. Results: Best results were observed in patients that received a single biopsy or curettage; worse results were seen after multiple resections or osteotomies and associated with complications as infection or compartment syndrome. No patients had current symptoms or significant symptoms changes nor physical findings, radiographic clues or subsequent radiographic changes suggesting adamantinoma development. Conclusions: OFD is a benign condition; the natural history of the disease has minimal consequences in the adult life. Surgical treatment is usually not necessary and may actually worsen the result because of the potential for severe complications. The relationship with adamantinoma remains unclear, follow-up is suggested

The authors aim to report the long-term follow-up experience in the reconstruction of bony defects by Ilizarov’s distraction osteogenesis using bone transport method following en bloc resection of bone tumors. En bloc resection was performed nine patients with bone tumors between October 1991–January 2000. The mean age of the patients was 19.3 years. Histological diagnosis was osteosarcoma in four cases, Ewing sarcoma in two cases, giant cell tumor (aggressive) in one, osteofibrous dysplasia (latent) in one and osteoblastoma (aggressive) in one case. The average follow-up period was 122 months and bone defect after resection was 14 cm. The function of the affected leg was excellent in four patients, good in two and poor in one patient according to the modified system of the MSTS. In the case where reconstruction of the ulna was done, MSTS score was excellent and DASH skoru was 2.5. We imply that in patients with long life expectancies, reconstruction with distraction osteogenesis seems to be an efficient method in the long-term follow up, on condition that its complications are promptly managed

Young active patients with malignant tumours arising in the distal fibula, requiring excision, present a challenge to the treating surgeon. Wide local excision is advocated, to achieve clearance, however, disruption of the ankle mortise results and fusion is often required to restore stability. The loss of movement is poorly tolerated in the younger patient and leads to progressive degenerative changes in surrounding joints. Excision of the distal fibula lesion followed by harvesting of the proximal fibula and using this graft to recreate the ankle mortise restores ankle stability and retains ankle movement. Between 1998 and 2007, we have performed this technique on 4 patients. Diagnoses were Ewing’s sarcoma, chondrosarcoma, parosteal osteosarcoma and osteofibrous dysplasia. To date there has been no evidence of distant or local recurrence. One case was complicated by infection, which resolved with radical debridement and antibiotics; the other three fibula grafts survived. Good to excellent results were achieved. We will present the technical aspects of this procedure, with particular reference to the most recent case, performed on a young female patient with parosteal osteosarcoma. We believe this technique provides good oncological and functional results and recommend this treatment option is considered in young active patients requiring distal fibula excisions for sarcoma. Correspondence should be addressed to Major M Butler RAMC, Princess Elizabeth Orthopaedic Centre, Royal Devon and Exeter Hospital, Exeter, Devon

The purpose of this study was to review the outcomes of patients treated with injectable calcium phosphate cement (Norian SRS, Norian Corporation, Cupertino, California) for contained bone defects after resection of benign or low-grade malignant bone tumours. The clinical records and radiographs of 17 patients who had been treated with calcium phosphate cement were reviewed, looking for incorporation into bone, reabsorption of the material and complications. The 17 patients had a mean age of 29.8 years (range 7 to 64). The diagnosis was giant cell tumour in 9 cases, fibrous dyplasia in 2, low grade chondrosarcoma in 2, and one each of enchondroma, chondromyxoid fibroma, osteofibrous dysplasia, and chondroblastoma. The tibia was involved in 9 cases, the femur in 6 and the radius in 2. The mean follow up was 11 months (range 3 to 25). The material is radioopaque and well visualised on plain radiographs. In most cases, incorporation of the material into the bone structure appeared good, but there was little absorption of the material during the followup available. The exceptions were 2 cases in which the material was absorbed following local recurrence of giant cell tumour. One fracture associated with a giant cell tumour healed well in the presence of the material. In three patients, there were clinical and radiological features at follow up suggestive of periostitis related to the material. In one case a florid effusion of the knee may have been due to the material. Injectable calcium phosphate cement may have a role in the management of contained defects requiring mechanical support following resection of benign or low-grade malignant tumours of bone. However, problems with periostitis, possibly synovitis and absorption in the presence of local recurrence should be considered