Aims. The aim of this study was to identify any progression between benign osteofibrous dysplasia (OFD), OFD-like adamantinoma and malignant adamantinoma, and to investigate the rates of local recurrence, metastases and survival, in order to develop treatment algorithms for each. Patients and Methods. A single institution retrospective review of all patients presenting with OFD, OFD-like adamantinoma and adamantinoma between 1973 and 2012 was undertaken. Complete data were available for 73 patients (42 with OFD; ten with an OFD-like adamantinoma and 21 with an adamantinoma). The mean follow-up was 10.3 years (3 to 25) for OFD, 9.2 years (3.0 to 26.3) for OFD-like and 11.6 years (0.25 to 33) for adamantinoma. Results. The mean age at diagnosis for OFD was 13.5 years (1 to 49), 10.5 years (6 to 28) for OFD-like and 34 years (14 to 86) for adamantinoma. A total of 24 of the 42 patients with OFD (57%) have not required any treatment and have been managed with observation. A total of 18 of the 42 patients with OFD underwent surgery, 13 with curettage and five with resection. In all, three patients developed recurrence following curettage (23%) but none following resection. All these patients were cured with further limited surgery. A total of six patients initially diagnosed with OFD were subsequently found to have OFD-like adamantinoma. Of the ten patients initially diagnosed with OFD-like adamantinoma, three (30%) were managed with observation alone and seven underwent surgery, two with curettage and five with resection. Local recurrence arose in two patients, one each after curettage and resection. No patients with either OFD or an OFD-like adamantinoma developed metastases or had progression to adamantinoma. All patients with an adamantinoma were treated by surgery, three with curettage, six with amputation and 12 with excision. In all, two of the three treated with curettage developed local recurrence, requiring further surgery. Late development of both local recurrence and metastases led to a ten year disease specific survival of 93% which had dropped to 39% by 20 years. Conclusion. We found no evidence of progression from OFD to adamantinoma. Conservative management with observation or curettage is often successful for patients with OFD and OFD-like adamantinoma. Resection with clear margins is required for patients with adamantinoma. Late tumour recurrence is not uncommon in adamantinoma and prolonged follow-up should be considered. Cite this article: Bone Joint J 2017;99-B:409–16

Aims. 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. Methods. 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. Results. Mean age at presentation was 13.5 years (SD 12.4), and mean follow-up was 5.65 years (SD 5.51). At latest review, 62 lesions (61.4%) were in skeletally mature patients. The most common site of the tibial lesion was the anterior (76 lesions, 75.2%) cortex (63 lesions, 62.4%) of the middle third (52 lesions, 51.5%). Pain, swelling, and fracture were common presentations. Overall, 41 lesions (40.6%) presented with radiological deformity (> 10°): apex anterior in 97.6%. A total of 41 lesions (40.6%) were treated conservatively. Anterior bowing < 10° at presentation was found to be related to successful conservative management of OFD (p = 0.013, multivariable logistic regression). Intralesional excision was performed in 43 lesions (42.6%) and a wide excision of the lesion in 19 (18.8%). A high complication rate and surgical burden was found in those that underwent a wide excision regardless of technique employed. There was progression/recurrence in nine lesions (8.9%) but statistical analysis found no predictive factors. No OFD lesion transformed to adamantinoma. Conclusion. This study confirms OFD to be a benign bone condition with low rates of local progression and without malignant transformation. It is important to distinguish OFD from adamantinoma by a histological diagnosis. Focus should be on angular deformity, monitored with full-length tibial radiographs. Surgery is indicated in symptomatic patients and predicted by the severity of the initial angular deformity. Surgery should focus more on the deformity rather than the lesion. Cite this article: Bone Joint J 2022;104-B(2):302–308

Adamantinoma is a rare low-grade malignant epithelial bone tumour. We report a case of an expansile, osteolytic mid-diaphyseal tibial lesion found in a 12 year-old girl. An initial histological diagnosis of basaloid-type adamantinoma was made. Following excision, further histology demonstrated basaloid cells and acellular matrix focally surrounded by osteoclast giant cells with calcium deposits, features consistent with pilomatrixoma. Several histological variants of adamantinoma have been documented; this case details a previously unreported histological adamantinoma variant – pilomatrixoma-adaminatinoma

Adamantinoma is a primary low-grade, malignant bone tumor that is predominantly located in the mid-portion of the tibia. The aetiology of the tumor is still a matter of debate. Histologically, classic adamantinoma is a biphasic tumor characterized by epithelial and osteofibrous components that may be intermingled with each other in various proportions and differentiating patterns. We report here a particular morphologic variant of adamantinoma characterised by the loss of classic epithelial differentiation. A 17 year-old teenager presented with a long history of a tumor in the left leg. Explorations revealed an osteolytic lesion of the middle shaft of the left tibia with a huge mass invading soft tissues. An intra medullar bone lesion with benign appearance was observed in the right tibia. CT of the chest revealed lung metastases. Histologically, we observed in the left side a pattern of spinde-celled high grade sarcoma without any evident differentiation. Spindle-shaped tumor cells express cytokeratins and vimentin. Ultrasturctural study showed tonofibrils and desmosomes and was helpful to recognize the epithelial nature of the tumour. The diagnosis of dedifferentiated adamantinoma was retained. In the right tibia biopsy revealed fibrous dysplasia. Amputation above the knee was performed followed by chemotherapy. One year later, the patient is still alive with multiple lung metastases. In our study, we discussed the clinico-pathologic features of this particular pattern of adamantinoma as well as its association with fiobrous dysplasia in this case. A review of literature was done

Adamantinoma is a rare tumour of long bones that occurs most commonly in the tibia. Its pathogenesis is unknown. It is locally aggressive and recurrences are common after resection. Metastases have been reported in 10% to 20% of cases, most commonly in the lungs and rarely in the lymph nodes. We report a patient who developed a skeletal metastasis four years after resection of the primary tumour. There was no evidence of recurrence at the primary site or of secondary deposits in the lungs

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

1. A case of adamantinoma of the tibia was followed radiographically for seventeen years before a biopsy was performed. During this time there was an increase in size of the tumour and a gradual transformation of radio-opaque into polycystic areas. 2. Biopsy revealed a peculiar osteoid-like differentiation of the epithelial tissue

Two cases of adamantinoma of the tibia are reported. The first patient has survived twenty years after above-knee amputation and shows no sign of recurrence. The second patient shows no evidence of recurrence two and a half years after amputation through the knee. The clinical picture of the tumour is described and the theories of histogenesis are outlined

1. The so-called adamantinoma of long bones is a clinico-pathological entity, the pathogenesis of which is still in doubt. The case for its being a synovial sarcoma showing epithelial differentiation is in our view unconvincing. 2. The tumour is slowly growing, and of low grade malignancy. Apparent cure has been effected in a third of the total cases recorded by amputation or resection of the diseased bone. 3. These means, however, have not prevented metastases to the lungs and skeleton in a similar number. 4. A case is presented in which a metastasis appeared in the chest twenty-two years after amputation of the leg. This was sensitive to telecobalt irradiation, and is the first case in which a distant metastasis has been proved microscopically

Adamantinoma are rare, low grade malignant, bone tumors, making up only 0.1–0.48 percent of primary malignant bone tumors. They occur predominantly in the long bones, especially the tibia. Histogenetically it is thought that it originates from embryological displacement of basal epithelium of the skin, although other hypotheses have been proposed. Clinically most patients present with swelling and possible bending of the tibia, painful or painless. It's often noticed in an earlier stadium, but symptoms are non-specific and have a slow progressive character. Median patient age is 25 to 35 years, with a range from two to 86 years. It is slightly more common in men than woman, with a ratio of 5:4. Occurrence in children is even rarer. A study by Van Rijn et al. finds only 119 references, and presents six more cases. Treatment is the same. An MRI-scan should be performed to check for metastasis, loco regional staging and for operative planning. Operative excision and reconstruction is necessary to prevent metastasis and maintain load bearing capacity. Generally these resections and reconstructions are done without objective measurements. The surgeon uses a rule of thumb, like a sculptor, or ruler approach to recreate the excised bone, either with allo- or autograft materials. An optimal fit, i.e. a minimal space between tibia and graft, is not always achieved, possibly resulting in pathological fractures. This risk of pathological fractures lengthens recovery time. The fractures elongate hospitalization time and recovery time and are a heavy burden to patients. Computer assisted surgery (CAS) systems, used for example in prosthesis placement, offer objective measurements in 3d space of hard structures with high accuracy. These can be used to produce an accurate copy of the resected bone. If the reconstruction accurately fits the bone defect that's left after the resection, it's likely that the occurrence of pathological fractures decreases. An adamantinoma in the tibia of a 12 year old boy was treated. Surgery consisted of hemicortical resection and inlay allograft reconstruction. The software used was the Orthomap navigation software (Stryker). A donor bone was supplied with help from the bone bank. The technical approach to the reconstruction was the planning of resection planes around the tumor. As the CT scale for both the patient and allograft bone is the same, the resection planes in the patient navigation setup could be copied to the allograft creation setup. Normal CAS setup was performed after first incision, with a tracker attached to the tibia. It was planned that a navigated bone saw would be used for the cutting. The tracker was attached to the saw with a new attachment, and calibrated in the universal calibration tool. During the surgery the oscillating saw proved to be impossible to navigate. The instrument calibration module was not able to accurately registered the saw, this despite accurate registrations in pre-operative testing. The CAS system was used however for accurately determining the saw planes. The planes were traced with the pointer tool. Then a non-navigated saw was used to perform both trapezoid shaped resections. A similar CAS setup was performed on the donor bone. The reconstruction was a good fit. The skin was closed in layers. Post-operative x-ray control was performed. Operation time was just over two hours. Currently the follow-up time is five months. There have been no complications and the control x-rays show good allograft ingrowth. While the original operation plan couldn't be performed the principle of computer assisted reconstruction has its merits. This was a proof of concept. The navigation was accurate to less than 1 mm, and the trapezoid resection shape guarantees a good fit. However the method of resection of the drawn planes by non-navigated bone saw was not accurate enough, because of the saw oscillations. There was improvement in operation time. With more accurate means of resection, as for example a computer controlled laser or water-jet, this type of reconstruction could have other very interesting applications

Osteofibrous dysplasia is an unusual developmental condition of childhood, 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 and some authors believe that they are part of one histological process. We retrospectively reviewed 16 patients who were diagnosed as having osteofibrous dysplasia initially or on the final histological examination. Their management was diverse, depending on the severity of symptoms and the extent of the lesion. Definitive (extraperiosteal) surgery was localised ‘shark-bite’ excision for small lesions in five patients. Extensive lesions were treated by segmental excision and fibular autograft in six patients, external fixation and bone transport in four and proximal tibial replacement in one. One patient who had a fibular autograft required further excision and bone transport for recurrence. Six initially underwent curettage and all had recurrence. There were no recurrences after localised extraperiosteal excision or bone transport. There were three confirmed cases of adamantinoma. The relevant literature is reviewed. We recommend extraperiosteal excision in all cases of osteofibrous dysplasia, with segmental excision and reconstruction in more extensive lesions

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

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

We report a case which highlights the progression of osteofibrous dysplasia to adamantinoma and questions whether intralesional curettage is the appropriate treatment. The role of a joint-sparing massive endoprosthesis using cortical fixation is demonstrated and we describe a unique biomedical design which resulted in the manufacture of an end cap to allow amputation through a custom-made proximal tibial replacement, rather than an above-knee amputation following recurrence

This case highlights the close association between osteo-fibrous dysplasia (OFD) and adamantinoma, drawing attention to the role for more radical treatment options when treating OFD. We discuss the advancements in joint-sparing endoprostheses using bicortical fixation. Finally we describe a unique biomedical design allowing for manufacture of an end cap to allow amputation through a custom made joint-sparing proximal tibial replacement as opposed to an above knee amputation. A 37 year old presented 7 years ago having sustained a pathological fracture of her tibia. Subsequent biopsy revealed OFD, curettage with bone graft was performed. She later developed recurrence, two percutaneous biopsies confirmed OFD. 6 years following her initial diagnosis she was referred to RNOH with further recurrence, a biopsy at this stage revealed a de-differentiated adamantinoma. A joint-sparing proximal tibial replacement was performed and adjuvant chemotherapy administered, she remained well for one year. Recurrence was noted at the distal bone-prosthesis interface, histology revealed a high grade dedifferentiated osteosarcoma, limb preservation was not deemed possible and an amputation was performed through the prosthesis. The proximal tibial device was uncoupled leaving a residual 7 cms insitu, a small custom made end cap was attached to the remaining prosthesis and a myocutaneous flap fashioned over it, this ultimately enabled the patient to mobilise well with a below–knee orthotic device. This case highlights the need for more radical surgery when treating cases of OFD and the relationship between OFD and adamantinoma. It also introduces a joint-sparing proximal tibial device for use in proximal tibial tumours that do not invade the proximal tibial metaphysis. The biomechanical design solution has given us the unique option of preserving the knee joint allowing the patient a below knee amputation whereas previously an above knee amputation would have been performed thereby significantly reducing her functional outcome

We evaluated the oncological and functional outcome of 18 patients, whose malignant bone tumours were excised with the assistance of navigation, and who were followed up for more than three years. There were 11 men and seven women, with a mean age of 31.8 years (10 to 57). There were ten operations on the pelvic ring and eight joint-preserving limb salvage procedures. The resection margins were free of tumour in all specimens. The tumours, which were stage IIB in all patients, included osteosarcoma, high-grade chondrosarcoma, Ewing’s sarcoma, malignant fibrous histiocytoma of bone, and adamantinoma. The overall three-year survival rate of the 18 patients was 88.9% (95% confidence interval (CI) 75.4 to 100). The three-year survival rate of the patients with pelvic malignancy was 80.0% (95% CI 55.3 to 100), and of the patients with metaphyseal malignancy was 100%. The event-free survival was 66.7% (95% CI 44.9 to 88.5). Local recurrence occurred in two patients, both of whom had a pelvic malignancy. The mean Musculoskeletal Tumor Society functional score was 26.9 points at a mean follow-up of 48.2 months (22 to 79). We suggest that navigation can be helpful during surgery for musculoskeletal tumours; it can maximise the accuracy of resection and minimise the unnecessary sacrifice of normal tissue by providing precise intra-operative three-dimensional radiological information

The outcome of tibial allograft reconstruction after resection of a tumour is inconsistent and has a high rate of failure. There are few reports on the use of tibial allografts in children with open growth plates. We performed 21 allograft reconstructions (16 osteoarticular, five intercalary) in 19 consecutive patients between seven and 17 years of age. Two had Ewing’s sarcoma, one an adamantinoma and 16 osteosarcoma, one with multifocal disease. Five patients have died; the other 14 were free from disease at the time of follow-up. Six surviving patients (eight allograft reconstructions) continue to have good or excellent function at a mean of 59 months (14 to 132). One patient has poor function at 31 months. The other seven patients have a good or excellent function after additional procedures including exchange of the allograft and resurfacing or revision to an endoprosthesis at a mean of 101 months (43 to 198). The additional operations were performed at a mean of 47 months (20 to 84) after the first reconstruction. With the use of allograft reconstruction in growing children, joints and growth plates may be preserved, at least partially. Although our results remain inconsistent, tibial allograft reconstruction in selected patients may restore complete and durable function of the limb

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 management of pathological fractures in children remains controversial. The indications for surgical treatment are unclear and the need for histological diagnosis before or after definitive treatment is not clearly defined. We reviewed retrospectively the records of all patients under the age of 16 years who presented over the past 7 years with a fracture as the first manifestation of bone pathology. There were 26 patients (19 boys and 7 girls) of an average age of 12 years and 2 months (range 4.1–15.8 years). There were 9 cases of fracture through a simple bone cyst, 6 in the humerus and 3 in the femur. In all cases the fracture was treated conservatively initially. Subsequent management included needle biopsy in all, followed by bone marrow injection under the same anaesthetic. The patients suffered a refracture and were treated with flexible intra-medullary nail fixation. There were 5 cases of fibrous dysplasia, of which 2 in the femur, 2 in the tibia and one in the proximal radius. Histological diagnosis was obtained in all cases prior to definitive treatment. This included a locked intra-medullary nail in one patient and flexible nailing in another two. The remaining two patients are still under observation. There were 2 patients with giant cell tumour, 3 patients with aneurysmal bone cyst and one patient with chondroblastoma. Histological diagnosis preceded treatment with curettage and grafting in all these cases. There were 6 patients with malignant primary bone tumour, 1 adamantinoma, 2 osteosarcoma, and 3 with Ewings Sarcoma. The 3 patients with Ewing’s sarcoma involved the femur. One had extensive local disease and early intra-medullary nailing was performed for palliative reasons. The second patient was treated conservatively initially. Definitive surgery was performed after fracture healing and included segmental resection and vascularised fibular graft. The third patient was initially treated elsewhere. She was thought to have a benign lesion and internal fixation with a screw/plate device was performed. Histology from intra-operative specimens confirmed Ewing’s sarcoma. Definitive surgery required extensive resection and prosthetic replacement. The 2 patients with osteosarcoma had fracture of proximal humerus and distal femur. The former was treated by forequarter amputation as there was tumour involvement of brachial plexus and remains AWND at 7 years. The latter had resection and EPR of the distal femur. One patient with adamantinoma underwent segmental resection and reconstruction with VFFG. We recommend that primary fixation of pathological fractures should be avoided until histological diagnosis is obtained. However, if radiographic appearances are reassuringly benign, biopsy can be delayed until conservative fracture management is completed. Definitive treatment of benign lesions with protective intramedullary nailing or curettage and grafting can follow frozen section under the same anaesthetic