Pathologische Fraktur bei osteofibröser Dysplasie

Osteofibrous dysplasia Campanacci is a rare tumor-like lesion mostly presented in the meta-epiphyseal region of the tibia. Different options exist about the treatment modalities. The rare case of a 4 1/2-year-old girl suffering from a pathologic fracture of the tibia shaft due to a osteofibrous dysplasia is presented. The fracture was stabilized by intramedullary nailing using elastic titanium rods. During this procedure a biopsy of the lesion confirmed the radiological suspected diagnosis of an osteofibrous dysplasia. The implant was removed after ten months when fracture and lesion were healed. The treatment options and the differential diagnoses are discussed by review of the literature.     

Minimally invasive plate osteosynthesis for osteofibrous dysplasia of the tibia: a case report

Osteofibrous dysplasia occurs most frequently in the tibia and may result in deformity and pathological fracture. We report one such case in a 6-year-old girl who underwent minimally invasive plate osteosynthesis using a locking compression plate. The varus deformity of the tibia was manually corrected, and the plate was inserted without extensive surgical exposure. At 20-week follow-up, the fracture had healed completely, and she had returned to normal daily living. At 3-year follow-up, radiographs showed no progression of the varus deformity. Minimally invasive plate osteosynthesis is useful for treatment of pathological fractures caused by osteofibrous dysplasia. It preserves blood flow at the fracture site, leading to good bone healing and stability to prevent further deformity and an early return to daily activity.     

Osteofibrous dysplasia of the ulna

We experienced two patients with osteofibrous dysplasia of the ulna. Both of them showed typical radiographic findings of osteofibrous dysplasia, except for location. One patient underwent surgery at the age of 15 years to confirm the diagnosis histologically. Although the tumor recurred 5 months after the surgery, expansion of the lesion seemed to cease at the age of 25 years. In the other patient, the natural course was observed. The tumor showed a tendency toward spontaneous regression at the age of 11 years. Therefore, the nature of osteofibrous dysplasia in the ulna seems to be the same as that in the tibia and fibula. Surgery must be delayed for as long as possible in osteofibrous dysplasia of the ulna, as well as that of the tibia and fibula. Received: April 26, 2001 / Accepted: July 17, 2001     

Osteofibrous dysplasia-like adamantinoma versus osteofibrous dysplasia in children: A case report of challenging diagnosis

IntroductionOsteofibrous dysplasia (OFD) and Osteofibrous dysplasia-like Adamantinoma have a similar appearance both in clinical and radiography, but different in its histopathology. Despite this similarity, the treatment and prognosis are different, therefore the diagnosis should be established precisely.Case illustrationA three-year-old boy was admitted to hospital after falling on his lower leg. A bead size lump appeared on his tibia with pain and swelling, which later became enlarged. Diagnosis of osteofibrous dysplasia and adamantinoma was considered. We performed limb-salvage procedure by curretage, bone grafting, and internal fixation application. The histology section showed woven bone rimmed by polygonal osteoblast cell with intervening fibrous stroma and small nests of tumour cells raised the possibility of epithelial differentiation. The positivity for cytokeratin immunostaining confirmed the diagnosis as osteofibrous dysplasia-like adamantinoma. In this case it is a very rare spectrum of malignancy in children.DiscussionThese two tumor entities have identical radiographic characteristics, histopathology features the distinction between classic adamantinoma and OFD-like adamantinoma based on the predominant epithelial component. The relationship of osteofibrous dysplasia with adamantinoma is unclear. Several authors considered possible calling relationship osteofibrous dysplasia as “juvenile adamantinoma”. However, does not rule out the possible existence of de novo osteofibrous dysplasia not related to adamantinoma.ConclusionsOFD-like adamantinoma and Osteofibrous Dysplasia had similar histopathology pattern, a pathologist must be aware of this feature and perform immunohistochemical staining for keratin particularly when the histopathological feature of osteofibrous dysplasia showed small nests of tumor cells within the fibrous stroma. diagnostic challenging and require multidisciplinary approach.     

自体腓骨移植治疗胫骨骨纤维结构不良

目的：探讨病灶边缘切除+自体腓骨移植重建胫骨+内固定的手术方式治疗胫骨骨纤维结构不良的疗效。方法1997年1月至2010年12月间我科对10例胫骨骨纤维结构不良（osteofibrous dys?plasia, OFD）的患儿行胫骨病灶骨膜下边缘切除,保留胫骨后壁,所遗骨缺损以自体健侧腓骨移植加钢板内固定重建。移植腓骨长为6~20 cm,平均12.2 cm。术后随访时间为4~12年,平均为6.6年。结果10例患儿术后依据病检有3例最后诊断为似釉质细胞瘤（adamantinoma, AD）的OFD或典型的AD。骨纤维结构不良病灶行骨膜下病灶切除术均未发生术后复发。移植的自体腓骨术后3~8个月（平均4.5个月）达到骨性愈合,双下肢基本等长,功能接近正常。结论骨纤维结构不良因易与釉质细胞瘤混淆,且有发生病理性骨折和有恶变的可能性,建议以手术治疗取代保守治疗。自体腓骨移植重建胫骨骨纤维结构不良病灶切除后骨缺损,游离腓骨骨移植加钢板固定术与带血管腓骨移植效果相当,但方法简单、操作难度小,可最大限度地恢复患肢功能。     

Adamantinoma of the tibia masked by fibrous dysplasia. Report of three cases

Three cases of adamantinoma of the tibia associated with and mimicking fibrous dysplasia or osteofibrous dysplasia are reported in children aged three, nine, and 16 years. The roentgenographic features were typical of intracortical fibrous dysplasia (osteofibrous dysplasia). These entities are not two distinct diseases, but rather are different histologic expressions of essentially the same process, which varies depending on location (predominantly intracortical or medullary) and age. Adamantinoma in children under ten years of age is not as rare as reported in the literature and was found in two of 14 cases in our files (14.3%). In some cases the fibrous dysplasialike component predominates over the scarce epithelioid islands of tumor cells and consequently is not recognized as adamantinoma. That may explain the frequent recurrences after incomplete excisions of supposed intracortical fibrous dysplasia lesions in young children. An extensive histopathologic study of the biopsy and/or surgical specimen by a specialized pathologist is therefore advisable.     

自体腓骨移植治疗胫骨骨纤维结构不良及其相关疾病之探讨

【】目的：探讨病灶边缘切除+自体腓骨移植重建胫骨+内固定的手术方式治疗胫骨骨纤维结构不良的疗效。方法：对10例胫骨骨纤维结构不良儿童行胫骨病灶骨膜下边缘切除,保留胫骨后壁,所遗骨缺损以自体健侧腓骨移植加钢板内固定重建。移植腓骨长6cm～20cm ,平均12. 2cm。术后随访4年～12年,平均6.6年。结果：移植的自体腓骨术后平均4.5个月(3～8个月)达到骨性愈合,双下肢等长,功能接近正常。结论：1.骨纤维结构不良因易与釉质细胞瘤容易混淆,且有发生病理性骨折和有恶变的可能性,建议以手术治疗取代保守治疗;2.骨纤维结构不良病灶行骨膜下病灶切除术并未发生术后复发;3.自体腓骨移植重建胫骨骨纤维结构不良病灶切除后骨缺损,游离腓骨骨移植加钢板固定术与带血管腓骨移植效果相当,但方法简单,操作难度小,可最大限度地恢复患肢功能,是行之有效的方法。     

CD99 positive adamantinoma of the ulna with ipsilateral discrete osteofibrous dysplasia

An adamantinoma is a rare, low-grade malignant, osteolytic bone tumor occurring predominantly in the diaphysis of the tibia. Osteofibrous dysplasia has been suggested as a precursor lesion to adamantinoma. Evidence for the relationship between these two tumors is based on their similar histologic features, immunohistochemistry, shared clonal abnormalities, overlapping skeletal distribution, and simultaneous occurrence in the tibia and fibula. The ulna is an unusual site of involvement by adamantinoma and osteofibrous dysplasia. Simultaneous involvement of the ulna by adamantinoma and ossifying fibroma has not been previously reported. A case is presented of an adamantinoma of the distal ulna with unique pathologic features occurring with an ipsilateral discrete focus of osteofibrous dysplasia as additional evidence of the relationship between these two lesions.     

Osteofibrous dysplasia of the tibia. Is there a need for a radical surgical approach?

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.     

Adamantinoma of the tibia in a nine-year-old child

Adamantinoma of the long bones is a rare, low-grade malignancy with a marked predilection for the tibia and is usually seen in patients during the second to fifth decades of life. Adamantinomas have also been reported in children, but the histological pattern in this age group is different from that seen in adults. We report a case of adamantinoma of the tibia in a 9-year-old boy. Histologically, the lesion was osteofibrous dysplasia with an epithelial component, called "differentiated adamantinoma". An osteofibrous dysplasia-like adamantinoma (differentiated adamantinoma) may be the precursor lesion of the classic type of adamantinoma.     

Ossifying fibroma of long bones in adults: a case report

Ossifying fibroma (osteofibrous dysplasia) is a rare fibro-osseous lesion made up of fibrous tissue with woven bone formation. It is most commonly found in the tibia and fibula of children ten years of age or younger. The most important differential diagnosis is monostotic fibrous dysplasia, which is radiologically similar but without woven bone rimmed by active osteoblasts like ossifying fibroma on histological examination. No epitheloid cells are found as in adamantinoma. We report the case of a 45-year-old woman who had a 12-month history of pain and slight swelling. Radiographs showed a multilocular radiolucent lesion with sclerotic rim in the proximal tibia. The lesion was curetted and the defect was packed with bone graft and acrylic cement. Microscopic examination showed active osteoblasts rimming the irregulary woven bone. One-year follow-up showed good functional recovery without recurrence of the lesion.     

Neonatal osteofibrous dysplasia: a case report

We describe a case of neonatal osteofibrous dysplasia that was diagnosed 3 days after birth. Osteofibrous dysplasia is a fibro-osseous lesion rarely seen in long bones. It is also known as congenital osteitis fibrosa or ossifying fibroma of bones. Although it occurs in children under 10 years of age, it is extremely rare in newborn babies. It must be differentiated from adamantinoma due to their radiological appearance, predilection for tibia and fibula, clinical picture, and certain histological similarities.     

Osteofibrous dysplasia treated with distraction osteogenesis: a report of two cases

We treated two patients with osteofibrous dysplasia of the tibia using en bloc marginal excision of the lesion and bone transport, a distraction osteogenesis procedure. In one patient, we had performed curettage procedures twice, each time followed by local recurrence and deformity. In contrast, with marginal excision and bone transport using an Ilizarov apparatus, the disease healed. In the other patient, we performed marginal excision and bone transport as the first procedure, which resulted in a healed lesion. In patients with osteofibrous dysplasia requiring treatment by an open method, curettage frequently is followed by local recurrence and deformity. We now prefer marginal excision of the lesion to avoid long-term failures, as in our first case. Distraction osteogenesis has proven useful for restoring structure and function in the affected limb.     

Extraperiosteal segmental excision for osteofibrous dysplasia of tibia with reconstruction by liquid nitrogen-treated recycled autograft

Background

Osteofibrous dysplasia usually progresses until ten years of age and occasionally regresses spontaneously after puberty. Patients with osteofibrous dysplasia usually require close observation. Surgery is an option considered only for extensive, deforming lesions and those with pathological fractures and rapid progression prior to puberty. If surgery is indicated, the traditional intra-lesional curettage or subperiosteal resection usually leads to high recurrence. Hence, extraperiosteal wide excision and various methods of reconstruction after resection have been advocated for this lesion. We reviewed the clinical results of patients managed with extraperiosteal segmental excision and reconstruction by liquid nitrogen-treated tumor-bearing autograft combined with allograft.

Marginal excision and Ilizarov hemicallotasis for osteofibrous dysplasia of the tibia: a case report

The surgical management of osteofibrous dysplasia (OFD), a rare developmental tumour-like condition of childhood that has a predilection for the tibia, ranges from curettage or subperiosteal resection to extraperiosteal wide resection followed by reconstruction. En-bloc excision followed by distraction osteogenesis has been described earlier for OFD. We describe a novel technique in which marginal excision was performed but instead of excising an entire segment of tibia, only the anterior portion containing the lesion was excised, sparing the uninvolved posterior cortex. Distraction osteogenesis was carried out on the anterior portion of the tibia (hemicallotasis) after acute correction of the deformity. Although the potential advantage is a decrease in time required for consolidation of the bone regenerate, this technique is dependent on the anatomical characteristics of the lesion. We describe a case in which this technique was adopted on a child with tibial OFD with resultant disease-free excision margins and deformity correction.     

Osteofibrous dysplasia of the tibia: case report and review of the literature

A case of osteofibrous dysplasia (OFD) of the tibia with 10 years of follow-up is presented. Spontaneous healing of this lesion occurred without any surgical intervention at the age of 10 years. The diagnosis was made retrospectively on the basis of clinical and radiographic appearance and evolution. The capricious nature and indolent course of this neoplasm has led to uncertainty regarding its etiology, evolution, and treatment. A review of the literature and the ongoing discussion about this matter is presented.     

Management of fibrous dysplasia. A report on 36 cases

A consensus on the clinical course of fibrous dysplasia has not yet emerged in the literature. We retrospectively evaluated 36 patients who were diagnosed with fibrous dysplasia in our institution and were followed for a mean duration of 56.5 months (range 7-210 months). Their mean age was 25.8 years (range 5-67 years); 46.7% were male. The most frequent presenting complaints were pain (66% of patients) and pathological fracture (20%). Osteosarcoma developed in one patient 20 years after he had undergone radiation therapy for fibrous dysplasia in the tibia. Mazabraud syndrome was encountered in two patients, and aneurysmal bone cyst associated with fibrous dysplasia was seen in one patient. Fibrous dysplasia is generally considered a static disease, but with long-term follow-up it is found to have a more dynamic nature. For this reason, patients with fibrous dysplasia should be followed carefully over the long-term.     

Osteofibröse Dysplasie Campanacci der Tibia

Pathologic fractures and deformities are common complications of osteofibrous dysplasia. This article concerns a rare tumor-like lesion primarily in the tibial diaphysis in young patients. The case is presented of a girl with stress fracture, distinct antecurvation, relapse, and refracture. Surgical treatment is difficult and requires resection and reconstruction. Despite the controversial indication, we recommend this method due to the disorder’s putative relationship with malignant adamantinoma.     

Cortical osteofibrous dysplasia of long bone and its relationship to adamantinoma. A clinicopathologic study of 30 cases.

Thirty cases of cortical osteofibrous dysplasia (COFD) were studied in an attempt at defining the relationship of COFD to adamantinoma. The patients ranged in age from newborn to 39 years (mean 13.4 years). The male:female ratio was 1:1. Presenting symptoms were most often pain or a mass. The tibia was involved in all 30 patients; in addition, the ipsilateral fibula was involved in five patients (17%). The histologic appearance of the lesions was dominated by the combination of woven bone trabeculae with prominent osteoblastic rimming and a loose, slightly myxoid stroma (less heavily collagenized in most instances than usually encountered in intramedullary fibrous dysplasia). Results of immunohistochemical study showed isolated cytokeratin-positive cells in the stroma of 28 of the lesions (93%). However, hyperchromatic epithelial islands characteristic of adamantinoma were not found in any of the 30 cases. A control population of 50 fibro-osseous lesions (intramedullary fibrous dysplasia, sclerosing fibroxanthoma, and cranial ossifying fibroma) was studied immunohistochemically; in none of these control cases were cytokeratin-positive cells found. Follow-up data were obtained in 17 cases (57%); the period ranged from 1 to 16 years (mean 6.05 years). Certain overlapping clinical features (including the location of the vast majority of the lesions in the tibia and, less often, the fibula) and the morphologic similarities of many areas of COFD and adamantinoma (particularly the shared presence of cytokeratin-positive cells) suggest a more than coincidental association between COFD and a adamantinoma. However, to date none of the 30 cases of COFD evaluated in this study has developed an adamantinoma.     

A simple management of massive bone defect after en-bloc resection of osteofibrous dysplasia of tibial shaft: A case report

IntroductionOsteofibrous dysplasia is a relatively rare disease, exclusively found in children, affecting the tibial diaphysis. Various management approaches are already available, but an internationally approved management guideline is not yet established. There is a major concern in the current management of wide excision technique as it frequently results in massive bone defect.Case presentationHere we present a case of osteofibrous dysplasia on a 10-year-old girl in Cipto Mangunkusumo Hospital with chief complaint of mild persistent pain of her lower leg since two years before with slight bowing deformity. The radiograph and histopathological examination support the diagnosis of osteofibrous dysplasia. She was managed with en-bloc resection (wide excision) of the tumor, followed with reconstruction using biomaterials substitute; combination between demineralized bone matrix (Bonegener^R) and bone substitute “hydroxyapatite and calcium sulphate” and internal fixation using plate and screw.ResultsClinical and radiological evaluation showed successful improvement and outcome. The patient showed progressive functional outcomes and achieved functional score of 100% LEFS at 3 years follow-up. The plate and screw was removed at 48 weeks after adequate callus formation andradiological union was achieved.ConclusionSimple reconstruction using biomaterial bone substitute not only created new bone formation with good stability, but also enabled patient to have an improved quality of life. This method is recommended to overcome the massive bone defect after tumor resection in osteofibrous dysplasia patient.