Non-ossifying Fibroma Research Articles

Biological Incorporation of Lyophilized Radiation Sterilized Bone Allograft and Mixed Bone Graft for the Management of Cysts and Cyst Like Lesions in Bone

Cystic lesions include Simple bone cyst and Aneurysmal bone cyst, and among cyst like lesions studied Giant cell tumor, fibrous dysplasia and Non-ossifying fibroma. This prospective interventional study was conducted in the Department of orthopaedic surgery Bangubandhu Sheikh Mujib Mdical university (BSMMU) and Biomedical research division, Atomic energy centre Savar, Dhaka Bangladesh from January 2004 to December 2019. Out of 155 Cysts and cysts like lesions were operated, among which cystic lesions were 73 (47.10%), among cystic lesions SBC was 51 (69.86%), and ABC-22 (30.14%) and cyst like lesions were 82 (52.90%), among cyst like lesions GCT was 68 (82.92%), FD was 12 (14.63%) & NOF was 2 (2.43%). All cases were operated by thorough curettage and cavity filled with Lyophilized radiation sterilized bone allograft impregnated with autogenous bone marrow for children and mixed bone graft in adult. Clinical and radiological evaluation was done in all cases in which 61 (83.56%) out of 73 cystic lesions were healed and 12 (16.44%) lesions were recurred, on the other hand 58 (70.73%) out of 82 cyst like lesions were healed and 24 (29.27%) lesions were recurred. Out of 155 cysts and cyst like lesions 119 (76.78%) were healed / satisfactory and 36 (23.22%) were recurred. Follow up period were 9 month to 15 years. P value is <.001. Main aims to evaluate the complete healing of cystic and cyst like lesions of bone with in corporation of allograft. Bone marrow impregnated Lyophilized radiation sterilized bone allograft and mixed bone graft is useful graft material for healing of the lesional area and restoring structural integrity as well as function for management of cysts and cyst like lesions in bone.

The epidemiology of musculoskeletal tumors in Aseer Central Hospital, south of Saudi Arabia

Background: Musculoskeletal neoplasms lead to significantly high morbidity and mortality rates among cases. Musculoskeletal tumors (MSTs) are relatively uncommon. The current study aimed to assess the magnitude, pattern, and predictors of MSTs among cases. Methodology: A record-based retrospective approach was applied for the current study. The study was conducted at Aseer Central Hospital. Medical records for patients with complete files and no errors and confirmed cases of any type of musculoskeletal tumor were included during the period from 2018 to 2020. Data on both patient and tumor, including patient’s demographic data, such as name, gender, and age, were collected. Results: The study included 69 cases with MSTs. The cases’ age ranged from 8 to 72 years, with a mean age of 31.5 ± 16.3 years. There were 35 female (50.7%) and 34 male (49.3%) participants. The femur was the most reported site for MSTs in the study (30.4%; 21), followed by foot and tibia (11.6%; 8 for each), hand (7.2%; 5), and elbow (5.8%; 4). The most recorded diagnosis was osteochondroma (12.7%), followed by ganglion cyst (9.1%), Ewing’s sarcoma (7.3%), soft tissue sarcoma (5.5%), osteosarcoma (5.5%), lipoma (5.5%), chondroblastoma (5.5%), non-ossifying fibroma (3.6%), benign lesion (3.6%), and Giant cell tumours (3.6%). Conclusions: This study revealed that most of the diagnosed MSTs among the studied cases were benign (osteochondroma and ganglion cyst) with uniform distribution of incidence by age and gender. Also, lower extremities were the most reported sites for MSTs, followed by upper extremities.

Symptomatic nonossifying fibroma: Is prophylactic fixation warranted?

Nonossifying fibroma (NOF) is a nonneoplastic lesion of the long bones which are rarely seen after skeletal maturity. The disorder is most often asymptomatic, discovered incidentally on plain radiographs and spontaneous regression over the years is typical. Nevertheless, larger lesions are at an increased risk of pathological fracture and together with atypical radiographic features can cause concern leading to consultations in orthopedic oncology clinics. A correct diagnosis can be established after a careful study of the patient's history and clinical status together with different imaging modalities and histologic characterization of the lesion. Surgical treatment may be required to forestall fractures in symptomatic lesions. Recurrence is rare after surgical intervention. Here, we report an interesting case of a pathological fracture through a symptomatic NOF with a 1-year follow-up, after surgical intervention.

Evaluation of curettage, synthetic bone grafting and fixation with implant in benign osteolytic and cystic lesions of long bones in children and young adults

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Curettage is one of the most common treatment options for benign lytic bone tumors and cystic lesions. In children and young adults, the resultant defect is usually filled with synthetic bone graft, which is a natural calcium phosphate hydroxyappatite (HA) in crystalline ceramic form.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; Sixteen cases of benign lytic and cystic lesions of bone were managed by simple curettage and grafting using hydroxyappatite blocks. Displaced pathological fractures or impending peritrochanteric fractures were fixed by implant. Commercially available HA was used for this purpose. Mean duration of follow up was 17.5 months (range 12&lt;strong&gt; &lt;/strong&gt;–30 months). Mean patient age was 11.2 years (range 6–20 years). Radiological staging of graft incorporation was done as per criteria of Irwin et al.&lt;strong&gt;&lt;/strong&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; Among 16 cases, 2 patients presented with non-ossifying fibroma and 2 patients with simple bone cyst, 12 cases were aneurysmal bone cyst. 5 patients had pathological fracture. Healing of lesion and graft incorporation is seen in all of the cases. At 6 months of follow up 3 cases were in Irwin stage III, 9 cases were in Irwin stage II and 4 cases were in Irwin stage I. At final follow up 10 cases were in Irwin stage III and rest was in Irwin stage II. No recurrence seen till date.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; We conclude that meticulous curettage and filling of bone cavity by calcium phosphate hydroxyappatite and fixation with implant in displaced pathological fracture or impending fracture is the procedure of choice of management of benign lytic lesions of bone.&lt;/p&gt;

Making sense of giant cell lesions of the jaws (GCLJ): lessons learned from next-generation sequencing.

Next-generation sequencing has revealed mutations in several bone-related lesions and was recently used to uncover the genetic basis of giant cell lesions of the jaws (GCLJ). Consistent with their benign nature, GCLJ show a low tumor mutation burden. They also harbor somatic, heterozygous, mutually exclusive mutations in TRPV4, KRAS, or FGFR1. These signature mutations occur only in a subset of lesional cells, suggesting the existence of a 'landscaping effect', with mutant cells inducing abnormal accumulation of non-mutant cells that form the tumor mass. Osteoclast-rich lesions with histological similarities to GCLJ can occur in the jaws sporadically or in association with genetically inherited syndromes. Based on recent results, the pathogenesis of a subgroup of sporadic GCLJ seems closely related to non-ossifying fibroma of long bones, with both lesions sharing MAPK pathway-activating mutations. In this review, we extrapolate from these recent findings to contextualize GCLJ genetics and we highlight the therapeutic implications of this new information. © 2019 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Qafzeh 9 mandible (ca 90–100 kyrs BP, Israel) revisited: μ-CT and 3D reveal new pathological conditions

ObjectiveThe aim of this paper is to provide new insights into growth patterns and health of Mousterian hunter-gatherers dated to ca. 90–100 kyrs B.P. from the Qafzeh site. MaterialsAn almost complete skeleton, including the mandible from the Qafzeh site (Qafzeh 9). MethodsMicro-CT and medical imaging techniques are used to explore inaccessible inner structures and to assess the etiology of identified lesion. ResultsMandibular and dental conditions appear to be growth-related skeletal disorders. ConclusionTo our knowledge, Qafzeh 9 offers the earliest evidence of associated mandibular and dental pathological conditions (i.e. non-ossifying fibroma of the mandible, pre-eruptive intracoronal resorption and osteochondritis dissecans of the temporomandibular joint) among early anatomically modern humans, and more generally among Middle Palaeolithic hominins in Southwestern Asia. The diagnoses can be added to other growth-related disorders of skulls previously documented from the Qafzeh site (Tillier, 1999; Tillier et al., 2001), suggesting a quite high and exceptional incidence of these conditions compared to those of Palaeolithic populations. SignificanceIdentification of pathological conditions in subadults from Eurasia dated to the Middle Palaeolithic is documented less often than in adult skeletons. Hence, new results from immature fossil remains are particularly important to our understanding of the past. Suggestions for further researchThis research will be extended to the rest of the skeleton, then to all Qafzeh specimens in order to broaden our understanding of this anatomically modern group.

Heterozygous FGFR1 mutation may be responsible for an incomplete form of osteoglophonic dysplasia, characterized only by radiolucent bone lesions and teeth retentions

Non-ossifying fibromas are seen in different disorders recognizable by specific features. Indeed, osteoglophonic dysplasia (OD) is characterized by radiolucent bone lesions associated with severe short stature, dysmorphism and failure of dental eruption. This syndrome is caused by heterozygous activating mutations in the immunoglobulin-like D3 domain of the FGFR1 gene, encoding a tyrosine kinase. Here, we report three patients from the same family presenting with radiolucent bone lesions and teeth retentions. Exome sequencing allowed identification of a novel mutation c.917C > T, p. Pro306Leu in exon 7 of the FGFR1 gene. Our patients present with normal stature and no severe dysmorphism. This report describes a mild form of OD and expands the phenotype related to FGFR1 mutations. These findings emphasize the need to consider FGFR1 variants in the case of multiple non-ossifying bone lesions associated with dental eruption anomalies.

The study of the value of H3F3A G34W immunohistochemical staining in the diagnosis of giant cell tumor of bone

Objective: To investigate the diagnostic role of H3F3A G34W immunohistochemistry in giant cell tumor of bone. Methods: A total of 275 tumors were collected from 2016 to 2018 at Shanghai Jiaotong University Affiliated Sixth People's Hospital, including 136 giant cell tumors of bone, 31 general osteosarcomas, 3 osteoclast-rich osteosarcomas, 3 brown tumors, 34 chondroblastomas, 29 giant cell tumors of tendon sheath, 20 primary arteromatoid bone cysts and 19 non-ossifying fibromas. Results: Among the 136 cases of giant cell tumor of bone,82 patients were male and 54 were female. The age ranged from 15 to 72 years (median age 38 years). Nuclear positivity for H3F3A G34W was seen in 119/136(87.5%) giant cell tumors of bone and 1/31(3.2%) general osteosarcoma,while all osteoclast-rich osteosarcomas, brown tumors, chondroblastomas, giant cell tumors of tendon sheath, primary arteromatoid bone cysts and non-ossifying fibroma were negative. Conclusions: The monoclonal antibody against the G34W mutated site of H3F3A is a specific biomarker for giant cell tumor of bone and useful for the diagnosis and differential diagnosis of giant cell tumor of bone. Meanwhile,for those cases in which giant cell tumor of bone are diagnosed basing on clinical,pathologic and radiographic features but are negative for H3F3A G34W,should be tested for rare mutations or H3F3A wild type.

Excisional Curettage of Benign Cystic Lesions.

Treatment of benign cystic lesions of bone with the use of excisional curettage requires careful preoperative planning and patient positioning before the initial incision is made. The initial incision must be carefully planned to expose the entire lesion without violating multiple compartments unnecessarily. A sizeable cortical window must then be made using a high-speed burr followed by evacuation of all cystic contents via curettage. The cavity is copiously irrigated before an adjuvant is used, and the lesion is stabilized, if necessary, before closing. There are many types of alternatives to curettage, such as wide resection, radiation, and embolization of feeding vessels. In orthopaedics, as in all medical specialties, many interventions and techniques have been rendered obsolete and, ultimately, replaced by newer, safer, and more efficient ones. The appeal of curettage has remained because of its procedural simplicity and adaptability in the management of a plethora of diseases such as benign cystic lesions of bone. Additionally, curettage, unlike wide resection, radiation, and embolization of feeding vessels, is minimally invasive and often definitive in nature when used as a treatment modality2. Lastly, curettage grants the performing surgeon the ability to maintain a contained cavity that can be treated with a variety of adjuvant therapies3-17. These reasons listed above make curettage a viable option for the surgical treatment of benign cystic lesions such as giant cell tumors of bone, aneurysmal bone cysts, unicameral bone cysts, chondromyxoid fibromas, and symptomatic nonossifying fibromas.

Non-ossifying fibroma: A RAS-MAPK driven benign bone neoplasm.

Non‐ossifying fibroma (NOF) has been an intriguing entity since its first description. It is the most common bone tumour, is usually asymptomatic affecting children and adolescents, is composed of a heterogeneous cell population, and undergoes spontaneous regression after puberty. In a recent article in The Journal of Pathology, Baumhoer and colleagues demonstrate mutations activating the RAS‐MAPK pathway (KRAS, FGFR1 and NF1) in ∼80% of the tumours. Activation of the RAS‐MAPK pathway by somatic mutations is found in a plethora of tumour types, both benign and malignant, while germline mutations cause a wide range of syndromes collectively termed the RASopathies. Their findings indicate that NOF, for long thought to be reactive, should be considered a true neoplasm. Moreover, their data suggest that only a subset of cells in the lesion contain the mutation. A second cell population consisting of histiocytes and osteoclast‐like giant cells appears to be reactive. This intimate relation between WT and mutant cells is also frequently encountered in other benign and locally aggressive bone tumours and seems essential for tumourigenesis. The spontaneous regression remains enigmatic and it is tempting to speculate that pubertal hormonal signalling, especially increased oestrogen levels, affect the balance between mutant and WT cells. © 2019 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of Pathological Society of Great Britain and Ireland.

Pathologic Fracture of a Non-Ossifying Fibroma

The non-ossifying fibroma (NOF) is a benign osseous lesion that is a subtype within a spectrum of multiple fibrous osseous lesions.

Activating mutations in the MAP-kinase pathway define non-ossifying fibroma of bone.

Non-ossifying fibroma (NOF), which occasionally results in pathologic fracture, is considered the most common benign and self-limiting lesion of the growing skeleton. By DNA sequencing we have identified hotspot KRAS, FGFR1 and NF1 mutations in 48 of 59 patients (81.4%) with NOF, at allele frequencies ranging from 0.04 to 0.61. Our findings define NOF as a genetically driven neoplasm caused in most cases by activated MAP-kinase signalling. Interestingly, this driving force either diminishes over time or at least is not sufficient to prevent autonomous regression and resolution. Beyond its contribution to a better understanding of the molecular pathogenesis of NOF, this study adds another benign lesion to the spectrum of KRAS- and MAP-kinase signalling-driven tumours. Copyright © 2018 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Non-ossifying fibroma of the radius: A rare case report

Non-ossifying fibroma of the radius: A rare case report

Non-ossifying fibroma with a pathologic fracture in a 12-year-old girl with tricho-rhino-phalangeal syndrome: a case report

BackgroundTricho-rhino-phalangeal syndrome (TRPS) is a rare autosomal dominant genetic disorder characterized by distinctive craniofacial and skeletal abnormalities, while non-ossifying fibroma (NOF) is a common benign bone tumour in children and adolescents. To date, no case of TRPS coexisting with NOF has been reported. This report presents a 12-year-old girl who had the characteristic features of tricho-rhino-phalangeal syndrome and non-ossifying fibroma with a fibula fracture.Case presentationA 12-year-old girl was admitted to the Department of Endocrinology and Diabetes for evaluation of brachydactyly and a right fibula fracture. Clinical examination revealed sparse scalp hair, a characteristic bulbous pear-shaped nose, and brachydactyly with significant shortening of the fourth metatarsal. Neither intellectual disability nor multiple exostoses were observed. Radiography of both hands showed brachydactyly and cone-shaped epiphyses of the middle phalanges of the digits of both hands with deviation of the phalangeal axis. Genetic analysis of TRPS1 identified a heterozygous germline sequence variant (p.Ala932Thr) in exon 6 in the girl and her father. Approximately 1 month before being admitted to our department, the girl experienced a minor fall and suffered a fracture of the proximal fibula in the right lower limb. The pathological cytological diagnosis of the osteolytic lesion was NOF. Ten months following the surgery, the lesion on the proximal fibula of the girl disappeared.ConclusionsIn conclusion, the present study is the first to report a rare case of NOF with a pathologic fracture in the fibula of a girl with TRPS. The identification of a missense mutation, (p.Ala932Thr), in exon 6 of TRPS1 in this kindred further suggested that the patient had type I TRPS and indicated that mutations in this exon may be correlated with more pronounced features of the syndrome. Radiological techniques and genetic analysis played key roles in the definitive diagnosis.

CLINICAL REASONING IN THE FACE OF UNCERTAINTY: CONSERVATIVE PHYSICAL THERAPY MANAGEMENT OF A TEENAGE ATHLETE DIAGNOSED WITH A PROXIMAL HUMERAL NON-OSSIFYING FIBROMA

There have been few published studies regarding the treatment of patients with non-ossifying fibromas (NOFs), either conservatively or operatively. The purpose of this case report is to discuss the clinical presentation and conservative management of a teenage athlete diagnosed with a proximal humerus NOF. The subject was a 13-year-old male middle school student with a diagnosis of left shoulder pain over the prior year preventing him from participating in sports activities. The combination of radiological findings revealing a NOF and a thorough physical examination allowed for the development of a physical therapy plan of care to address impairments and functional limitations. The subject was seen for eight visits where a combination of manual therapy techniques, neural mobilizations, and therapeutic exercises were administered to the cervical and upper quarter regions. The subject's progress was tracked by measuring pain-free shoulder active range of motion (AROM) and monitoring changes using the Numerical Pain Rating Scale (NRPS) values throughout sessions. After four sessions, AROM shoulder flexion and abduction increased from 123 º and 119 º to 160 º and 180 º respectively, and worst NRPS decreased from 9/10 to 3/10. Upon discharge after the eighth visit, the subject's DASH improved from 11.66 to 2.5. The subject remained pain free at an eight month follow up and returned to activity. Thorough assessment of both neuromechanical sensitivity and musculoskeletal impairments may provide for the utilization of conservative treatment options for individuals with symptomatic NOFs. 4.

Non-Ossifying Fibroma on the Mandibular Condyle and Ramus: a Case Report and Immunohistochemical Analysis

Non-ossifying fibroma (NOF) is a non-neoplastic process commonly observed in the metaphysis of the long bones of children. NOF in the gnathic skeleton is uncommon and has a predilection for the mandible. Microscopically, NOF consists of spindle-cell fibrous tissue in a storiform pattern associated with multinucleated giant cells, hemosiderin pigment, and xanthoma cells. A 33-year-old woman was referred for evaluation of an asymptomatic radiolucency in the mandible, first noticed 3 years prior to presentation. Her medical history was not contributory. Radiographic examination revealed a well-demarcated, expansile radiolucency with sclerotic borders in the right mandibular condyle and ramus. Incisional biopsy was performed, and microscopic analysis evidenced NOF. Immunohistochemical analysis showed positivity for CD68, CD163, FXIIIA, and focally for CD11c and α-SMA. NOF in the gnathic bones may mimic other odontogenic or non-odontogenic cysts and tumors; thus a strict clinicopathologic correlation is required for a correct diagnosis.

Treatment of proximal femoral benign lesions by proximal femoral nail anti-rotation combined with curettage and bone graft through the Watson-Jones approach

To evaluate the feasibility and effectiveness of proximal femoral nail anti-rotation (PFNA) combined with curettage and bone graft through Watson-Jones approach in the treatment of proximal femur benign tumors and tumor like lesions. The clinical data of 38 patients with benign tumors and tumor like lesions in the proximal femur who were treated through the Watson-Jones approach with PFNA combined with curettage and bone graft between January 2008 and January 2015 were retrospective analysed. There were 24 males and 14 females with an average age of 28 years (range, 15-57 years). Pathological types included 20 cases of fibrous dysplasia, 7 cases of bone cyst, 5 cases of aneurysmal bone cyst, 3 cases of giant cell tumor of bone, 2 cases of enchondroma, and 1 case of non-ossifying fibroma. Before operation, hip pain occurred in 19 patients, pathological fracture occurred in 12 patients, limb shortening and coxa varus deformity was found in 4 patients, and 3 patients received surgery for the local recurrence. The operation time, intraoperative blood loss, and full-weight bearing time after operation were recorded. Patients were followed up to observe union of bone graft and the position of internal fixator on X-ray films and CT images. Visual analogue scale (VAS) score was used to evaluate the level of pain. The Musculoskeletal Tumor Society (MSTS93) score was used to evaluate lower limb function. Harris hip score was used to evaluate hip joint function. The operation time was 130-280 minutes (mean, 182 minutes) and the intraoperative blood loss was 300-1 500 mL (mean, 764 mL). After operation, 3 cases of fat liquefaction of incision healed successfully by carefully dressing, and the rest incisions healed by first intention. All patients started partially weight-bearing exercise at 2-4 weeks after operation. The total weight-bearing time was 3-6 months (mean, 4.2 months). All the patients were followed up 24-108 months (median, 60 months). Imaging examination showed that the bone graft fused and the fusion time was 8-18 months (mean, 11.4 months). During the follow-up period, there was no complication such as pathological fracture, femoral head ischemic necrosis, hip joint dislocation, internal fixation loosening and fracture, and no tumor recurrence or distant metastasis occurred. At last follow-up, the VAS score, MSTS93 score, and Harris score were significantly improved when compared with preoperative ones ( P<0.05). The treatment of proximal femoral benign lesions by PFNA combined with curettage and bone graft through the Watson-Jones approach is safe and effective, with advantages of better mechanical stability, less residual tumor, and less postoperative complications.

Low-Intensity Pulsed Ultrasound (LIPUS) for the Treatment of Non-ossifying Fibroma in the Long Bones of the Lower Extremity

Objective: Non-ossifying fibroma (NOF) usually occurs in the second decade of life. Patients with lower extremity lesions are at risk for developing pathologic fractures. The etiology is thought to be faulty ossification rather than true neoplasm. LIPUS promotes bone healing in fractures and might be able to be applied to this rare condition. To our knowledge, however, there have been no reports on this attempt. We experienced 2 cases with large non-ossifying fibromas treated by LIPUS and here present their clinical outcomes. Cases and Results: 12 year-old (case 1) and 13 year-old (case 2) boys had NOF in the distal femur and the proximal tibia. Because NOF occupied the large area of the long bones, we attempted to use LIPUS in both cases. In case 1, NOF gradually disappeared at 2 years after LIPUS treatment. In case 2, NOF was acutely healed after minor fracture of the lesion and spontaneously disappeared. Discussion: Non-ossifying fibroma tend to regress as the skeleton matures. LIPUS might stimulate bone ossification and shorten the duration of NOF healing. Minor fracture or non-invasive drilling will help osteogenesis and NOF healing.

Outcomes of embolization of bone tumors in the pelvic and shoulder girdles: Initial experience

PurposeTo assess the effectiveness of transarterial embolization of bone tumors as preoperative technique to reduce blood loss or as a curative method in bone tumors of shoulder and pelvic girdles. Patients and methodsThe study included 25 patients with different bone tumors which are benign tumors (n = 16) and bone metastasis (n = 9). Their ages ranged from 6 to 65 years. Embolization was a palliative treatment in bone metastasis (n = 9), non-ossifying fibroma (n = 2) and osteoblastoma (n = 1). In patients with ABCs (n = 12) and osteoblastoma (n = 1), the technique was used as a curative method. The effectiveness of the technique was assessed using plain X-ray of the affected region, CT scan, CT angiography. ResultsIn 7 patients operated, reduction of blood loss reached about 400 mL. No perioperative complication or wound complications. Additionally surgery was easier. In the remaining 18 patients tumor regression in (ABCs n = 12, osteoblastoma n = 1) and pain reduction (metastasis n = 5) were detected during the follow up period 1–2 years. ConclusionPreoperative embolization is useful in reduction of intraoperative blood loss in tumors at shoulder and pelvic girdles. The technique is considered curative method in inaccessible lesions of pelvic and shoulder girdles with multiple interlacing vascular anastomosis.

A Unique Adult-Type Distal Tibia Non-Ossifying Fibroma with Secondary Aneurysmal Bone Cysts Presenting with a Pathological Fracture

A Unique Adult-Type Distal Tibia Non-Ossifying Fibroma with Secondary Aneurysmal Bone Cysts Presenting with a Pathological Fracture