[Fibrous dysplasia]. / Fibröse Dysplasie.

Fibrous dysplasia is a sporadically occurring benign skeletal disease characterized by the replacement of normal bone tissue with excessively proliferating cellular fibrous tissue. It can occur in a monostotic or polyostotic form. Depending on the location, number and size of the lesions, the clinical picture can vary from an asymptomatic disease to a severe disability. Typical problems are bone pain, bone deformities and pathological fractures. In combination with endocrinopathies and/or skin manifestations (café au lait spots), it is referred to as the McCune-Albright syndrome. The diagnosis is mainly carried out radiologically and the bony lesions are characterized by a cloudy, frosted glass-like aspect. Causal treatment is not possible. Orthopedic treatment includes pain relief, bone stabilization, deformity correction and, if necessary, lesion cleansing as well as the prevention of progression by means of antiresorptive medication. Pathological fractures are preferably stabilized with intramedullary osteosynthesis procedures.

Distal Radius Reconstruction Using Proximal Non-vascularized Fibula Graft in a Patient During the Syrian Conflict: A Case Study of Treatment Outcomes.

In conflict zones like Syria, accessing specialized medical care presents significant challenges. Here, we present the case of a 22-year-old female with a giant cell tumor in her distal forearm, exacerbated by limited access to healthcare due to the Syrian conflict. Despite these obstacles, we successfully performed en bloc resection and reconstructed the defect with a proximal non-vascularized fibular graft, restoring arm function. This case underscores the critical importance of adapting to adverse circumstances to deliver essential medical interventions in conflict-affected regions.

Diagnosis and Management of Monostotic Fibrous Dysplasia of the Tibia in an Adolescent Patient: A Case Report.

A rare benign bone condition called monostotic fibrous dysplasia (MFD) is characterized by the growth of fibrous tissue in place of a normal bone. It may lead to deformity in the affected bone, pain, and a pathologic fracture due to bone weakness. Hereunder, a case report of MFD in a 17-year-old male adolescent presenting to the hospital with localized bone pain and swelling in his right tibia is presented. After clinical examination and radiographic imaging, a provisional diagnosis of benign osteolytic lesion was considered. A magnetic resonance imaging (MRI) scan of the leg suggested the possibility of fibrous dysplasia or adamantinoma. The patient was managed with an intralesional curettage of the dysplastic bone and packing the cavity with blocks of a synthetic bone. The excised material was sent for histopathology, which established the diagnosis of fibrous dysplasia.

Diagnosis and Current Treatment of Aneurysmal Bone Cysts.

The purpose of this review is to increase awareness about the evolution and development of current trends in the diagnosis and treatment of aneurysmal bone cysts (ABCs). ABCs are benign, but locally aggressive bone tumors that mainly affect children. ABCs comprise 1% of all primary bone tumors and occur most frequently during the first two decades of life. The diagnosis is made using a variety of imaging modalities and has the characteristic features of an expansile, radiolucent lesion that is often seen in the metaphyseal region of the bone and has fluid-fluid levels that are apparent on MRI. In the pediatric population, telangiectatic osteosarcoma and unicameral bone cyst (UBC) are the main differential diagnoses of an ABC. Giant cell tumors (GCTs) also include in differential diagnosis, which often manifest in patients older than 15 and do not penetrate the open physis although they develop after the physeal closure. Imaging alone cannot rule out telangiectatic osteosarcoma; therefore, a biopsy is recommended. A variety of treatment options have been described; traditionally, most patients are treated with curettage and bone grafting. Curettage alone, however, usually results in tumor recurrence following excision. A variety of adjuvants have been utilized with varying degrees of effectiveness to reduce the risk of local recurrence. When a cyst is in the pelvis, its location and size are such that surgery is a very risky option. Selective arterial embolization has significantly contributed to the development of effective treatments for these situations. Embolization or radiation, as well as denosumab therapy, are widely used as therapies for ABCs in anatomic locations where surgery would significantly increase morbidity.

Infrequent Pediatric Subungual Injury Diagnosed by Intraoperative Anatomopathological Material: A Case Report.

Subungual lesions are very common in clinical practice. We present the clinical case of a 10-year-old female patient who presented with progressive nail deformity. The onset of the condition was approximately five years prior to presentation with an injury in the left hallux, according to the mother. She denied pain or change in the color of the area from the onset of the injury to the day of consultation. There was no previous trauma. Examination revealed subungual bone injury to the distal extremity (distal phalanx of the left hallux), and imaging tests (X-ray and soft tissue ultrasound) found bone injury. Subungual exostosis was considered as a possible diagnosis, thus prompting the indication for exeresis of the tumoral process. After surgical removal, the resected specimen was sent for pathological assessment, which found that an intraosseous hemangiolymphangioma was the origin of the tumor. A subungual exostosis is a slow-growth benign osseous tumor mainly located in the distal phalanx of the hallux that especially affects young adults, being less frequent in children. This condition results from a process of bone neoformation involving different stages, the clinical symptoms of which depend on its size and associated processes. Hemangiolymphangiomas are angiomatous lesions of the blood and lymphatic vessels that have a controversial etiology and present slow, painless, and progressive growth; these lesions are mostly benign. It is worth emphasizing that subungual injuries are not always caused by an underlying bone; therefore, potential differential diagnoses, both benign and malignant, should be considered, based on the location of the injury.

Treatment trends of benign bone lesions in a suburban New York healthcare system.

Introduction: The management of benign bone lesions is controversial as it is dependent on a multitude of factors such as age, anatomic location, comorbidities, lesion metabolic activity, surgeon preferences, and goals of care, among others. Thus far, many studies have attempted to report on these lesions; however, most are heterogeneous compilations of benign and malignant lesions with nearly all failing to report patient treatment and none of which have originated from a suburban area of the United States. The goal of this study was to establish a modern database dedicated solely to benign bone tumors to reflect current diagnosis and treatment trends in suburban New York. Materials and Methods: This was a multicenter retrospective observational study with inclusion criteria limited to benign bone lesions of all ages. Malignant lesions were excluded. Patients were drawn from both primary care provider and surgeon records, with documentation of their associated management. Results: A total of 689 patients met inclusion criteria. The overall operative rate for this cohort was 71.6%. In agreement with current literature, aneurysmal bone cysts, giant cell tumors, and osteochondromas underwent surgery more frequently than enchondromas; older patients underwent surgery less frequently; benign bone lesions were more commonly found in younger males, and the distal femur and proximal tibia were the most common locations for lesions (P < .05 for all findings). Conclusion: This study demonstrates the management of a globally representative variety of benign bone lesions in a diverse suburban population of New York and should facilitate future research on how lesion type, location, management, and other factors relate to patient outcomes.

Enhancing Healthcare for Sarcoma Patients: Lessons from a Diagnostic Pathway Efficiency Analysis.

Sarcomas, rare and with lower survival rates than common tumors, offer insights into healthcare efficiency via the analysis of the total interval of the diagnostic pathway, combining the patient interval (time between the first symptom and visit with a physician) and diagnostic interval (time between first physician visit and histological diagnosis). Switzerland's healthcare system, Europe's costliest, lacks research on treating rare conditions, like mesenchymal tumors. This study examines the total interval of the diagnostic pathway for optimization strategies. Analyzing a dataset of 1028 patients presented from 2018 to 2021 to the Swiss Sarcoma Board (MDT/SB-SSN), this retrospective analysis delves into bone sarcoma (BS), soft-tissue sarcoma (STS), and their benign counterparts. Demographic and treatment data were extracted from medical records. The patient interval accounted for the largest proportion of the total interval and secondary care interval for the largest proportion of the diagnostic interval. Age, grade, and localization could be elicited as influencing factors of the length of different components of the total interval. An increasing age and tumor size, as well as the axial localization, could be elicited as factors increasing the probability of sarcoma. The patient and secondary care interval (SCI) offer the greatest potential for optimization, with SCI being the bottleneck of the diagnostic interval. New organizational structures for care work-ups are needed, such as integrated practice units (IPU) as integral part of value-based healthcare (VBHC).

Classic and rare manifestations of multiple osteoma: A case report.

INTRODUCTION AND IMPORTANCE: Osteoma is a benign tumor that can arise from compact or cancellous bone and is more commonly found in the face or skull. The incidence of osteoma believed to be underreported as most are asymptomatic. To date, the best modality to diagnose osteoma is CT scan. We report a unique case of osteoma presenting with cranial and extracranial manifestations and highlight the importance of bone survey in evaluating patients with osteoma. CASE PRESENTATION: A 26-year-old female complained of bilateral pain in the jawbone and several areas of her head. On physical examination, there were several masses in the head with the largest on the left mandible measuring 5.6 × 6.0 × 4.5 cm from MSCT examination. Hemi-mandibulectomy, histopathological and cytopathology examination were performed on the tissue obtained from the left mandible which concluded osteoma. Post-operative bone survey was performed and found osteoma on left ulna and bilateral fibula. Suspected Gardner syndrome with multiple osteoma manifestation was excluded from normal results of colon in-loop examination. We conservatively monitored the patient and most recent 6-month follow-up found no complaint nor changes in the extracranial osteoma manifestation on left ulna and both fibulas. CLINICAL DISCUSSION: The benign tumor osteoma is incredibly uncommon to present both intra and extracranially. We suggest thorough skeletal studies such as bone survey to be performed as they are crucial in the full evaluation of patients with multiple osteomas. Osteoma treatment is based on the patient's symptoms, surgery for patients with symptoms and periodic monitoring for asymptomatic patients. CONCLUSION: It is necessary to consider radiological modality for diagnosing osteoma patients. The majority of osteomas are asymptomatic and the choice of radiological examination sometimes still misses the lesion. It is important to evaluate histologically if the lesion difficult to diagnose.

Management of advanced metacarpal aneurysmal cysts: Surgical technique.

The hand is a rare site for aneurysmal bone cysts (ABCs). Depending on their degree of invasiveness, treatment of ABCs ranges from medical therapy to surgical curettage and bone filling. In the most advanced cases, bone resection and reconstruction, with or without adjuvant or neoadjuvant treatment, are indicated. We describe a technique involving segmental bone resection and structural iliac graft reconstruction with carpo-metacarpal plate arthrodesis, for the management of advanced ABC involving the base of the metacarpals. The radio-clinical results of two patients operated on using this technique are presented, at 18 and 36 months. At the last follow-up, the results showed a QDASH of 15 and 10, and a PRWE of 9 and 11. Satisfaction was 10/10 in both cases. Grasp strength was 25 kg and 42 kg versus 28 kg and 40 kg on the opposite side. Pinch strength was 6 kg and 11 kg versus 7 kg and 10 kg. Metacarpophalangeal flexion was 80° and 90°. Extension was complete in both cases. Radiographs showed good graft fusion, with no lysis or signs of ABC recurrence at the last follow-up. Segmental bone resection and iliac autograft reconstruction with carpo-metacarpal plate arthrodesis represents a therapeutic option in the management of advanced ABCs of the metacarpal base.

Reconstruction After En Bloc Resection of a Distal Radius Tumor. An Updated and Concise Review.

The distal radius is rarely affected by either primary or metastatic bone cancers. The most frequent tumors of the distal radius are giant cell tumors, which are benign tumors with the propensity to invade. En bloc excision of giant cell tumors of the distal radius achieves a low recurrence rate but compromises the wrist joint, necessitates a significant reconstruction, and has functional consequences. Reconstruction after en bloc resection of a distal radius bone tumor is challenging. Furthermore, orthopedic oncologists disagree on treating such long bone anomalies most effectively. The present article summarizes the various biological and non-biological reconstruction techniques performed after en bloc resection of a distal radius tumor, discusses the advantages and disadvantages of each reconstruction strategy, and summarizes several case studies and case reports.

Vanishing solitary osteochondroma of humerus following trauma: A case report.

Osteochondromas are the most common benign tumors of the bone. Mainly these lesions affect the long-bone metaphysis and usually are asymptomatic. When complications develop from these lesions, then they become symptomatic and surgical resection may become indicated. Spontaneous resolution of osteochondroma is rare. There have been fewer case reports about this condition. We are reporting 16 years old, male, who sustained direct trauma to his shoulder and presented with fracture at the base of a solitary osteochondroma. Complete resolution of the lesion occurred without any surgical intervention 18 months following the fracture.

Allogenic Cancellous Bone versus Injectable Bone Substitute for Endoscopic Treatment of Simple Bone Cyst and Intraosseous Lipoma of the Calcaneus and Is Intraosseous Lipoma a Developmental Stage of a Simple Bone Cyst?

Simple bone cysts (SBCs) and intraosseous lipoma (IOL) of the calcaneus are rare tumor entities that are primarily diagnosed due to unspecific heel pain, incidental findings, or rarely due to pathological fractures. Compared to traditional open tumor resections, endoscopic resection of these benign tumors aims to minimize surgical morbidity and maximize surgical efficiency without compromising safety. Grafting is regularly performed to reduce the risk of recurrence and stimulate osseous consolidation of the lytic lesion. As the incidence is low and treatment strategies are heterogeneous, there is no clear consensus for the treatment of simple cysts or intraosseous lipomas of the calcaneus. The objectives of this study are (a) to present medium to long-term results after endoscopic resection and grafting with allogenic cancellous bone or bioresorbable hydroxyapatite and calcium sulfate cement, and (b) to add further evidence to the discussion of whether calcaneal SBC and IOL are the same entity at different developmental stages. Between 2012 and 2019, a total of 25 benign bone tumors consisting of 17 SBCs and 8 IOLs were treated by A.T. with endoscopic resection and grafting, comprising the largest cohort to date. For grafting, 12 patients received allogenic cancellous bone (group A) and 13 patients received injectable bone substitute (group B). Pre- and postoperative imaging using plain X-rays and MRI was retrospectively analyzed with a mean follow-up time of 24.5 months to assess tumor size, osseous consolidation (modified Neer classification), and tumor recurrence. A retrospective chart analysis focusing on adverse intra- and perioperative events and other complications associated with the surgical procedure was performed using the modified Clavien-Dindo classification (CD1-3). A total of 12/13 cases with allogenic bone grafting showed a Neer Type 1 osseous healing of the tumorous lesion after endoscopic resection, whereas only 5/11 cases with injectable bone substitute showed sufficient healing (types 1 and 2). There were three recurrent cysts (Neer 4) and two persistent cysts (Neer 3) after using injectable bone substitute. Two CD1 complications were observed in group A (prolonged wound drainage, sural neuritis) and eight complications were observed in group B (6× CD1, 2× CD3). At least two IOLs diagnosed preoperatively using MRI were ultimately identified as SBCs upon histopathologic examination. Allogenic cancellous bone grafting after endoscopic resection of calcaneal SBC or IOL showed a very low rate of complications and no tumor recurrence in our series. On the other hand, depending on the material used, injectable bone substitute showed a high rate of "white-out" (excessive drainage), resulting in multiple complications such as prolonged wound healing, insufficient permanent defect filling, recurrence, and revision surgery. Over time, calcaneal SBC may transform into IOL, exhibiting distinct features of both entities simultaneously during ossoscopy and histopathological analysis.

The Diagnostic Challenge of Osteoid Osteoma in the Bones of the Hand-A Case Series.

Osteoid osteoma (OO) is a benign bone tumor that rarely occurs in the bones of the hand. Due to the comparatively non-specific symptoms when occurring in the hand, OO is often misdiagnosed at first presentation, posing a diagnostic challenge. In the present case study, six cases of phalangeal and carpal OO, treated surgically at our department between 2006 and 2020, were retrospectively reviewed. We compared all cases regarding demographic data, clinical presentation, imaging findings, time to diagnosis, surgical treatment, and clinical outcome in follow-up examinations. When OO occurs in the bones of the hand, it can lead to swelling and deformities, such as enlargement of the affected bone and nail hypertrophy. Initial misdiagnoses such as primary bone tumors other than OO, tendinitis, osteomyelitis, or arthritis are common. Most of the presented cases showed a prolonged time until diagnosis, whereby the primarily performed imaging modality was often not sensitive. CT proved to be the most sensitive sectional imaging modality for diagnosing OO. With adequate surgical treatment, complications and recurrence are rare.

Tenosynovial Giant Cell Tumor of Foot in a Patient with Radioiodine-Refractory Thyroid Cancer: Imaging Findings on FDG-PET/CT, MRI, and Radioiodine Scan.

We herein illustrate a case of benign tenosynovial giant cell tumor, which was incidentally detected as FDG-avid lesion on PET/CT in a patient with radioiodine refractory thyroid cancer, with predominantly non-iodine concentrating disease. The lesion was followed up clinically and with local MRI annually for subsequent 3 years. The utility of hybrid PET-CT imaging, the non-iodine concentration of the tumor along with clinical knowledge, and findings on other imaging and pathological modalities in answering and diagnosing incidental benign musculoskeletal tumors in a patient with known thyroid malignancy are presented here.

Denosumab treatment for progressive Enneking stage II cervical giant-cell tumor conservatively.

Cervical giant cell tumor of the bone (GCTB) is a rare, primary benign bone tumor in pediatric patients. Surgery remains the primary choice for treating resectable cervical GCTB. Additional adjuvant therapeutic options are available for patients with unresectable cervical GCTB, including the anti-RANKL monoclonal antibody, denosumab. We represented a case incidentally found in a 7-year-old female, who complained severe craniocervical pain, grade 2-3 dysphagia, dysphonia, hypesthesia, and extremity weakness. The patient showed an impressive clinical response to denosumab, both clinically and radiologically, without adverse events or recurrence. To date, this is the youngest patient ever reported to have a progressive Enneking stage II C3 GCTB treated with denosumab alone. Denosumab can be administered as a single and conservative therapy for pediatric patients with unresectable upper cervical GCTB, avoiding the risks and morbidity of surgical and radiative treatment.

Management of talar lesions with cement augmentation and autologous bone graft.

BACKGROUND: Treatment of bone lesions involved with the articular cartilage at the talus is challenging. We report the management of talus lesions, particularly tumors and avascular necrosis (AVN), at the articular surface through treatment with cement augmentation and autologous bone graft. METHODS: Eight benign bone tumors and three cases of AVN were reviewed retrospectively at a mean follow-up of 56 months (range, 12-162). The mean age of all patients was 36.1 years old (range, 15-73) when assessed between February 2005 and November 2021. Curettage of tumorous and necrotic lesions resulted in significant bone defects filled with bone cement augmentation. Cartilage defects of the talar dome were supported with autologous cancellous bone graft. Tolerable weight-bearing ambulation was permitted immediately after surgery. Radiological and functional evaluations were recorded. RESULTS: We observed an increase in the average The American Orthopaedic Foot and Ankle Score (AOFAS) (p = .003) and a decrease in the average Visual Analogue Scale pain score (p = .003). There was no statistically significant decrease in ROM before or after surgery (p = .114). Additionally, no talus collapse of the ankle joint occurred. Talar dome status did not aggravate before or after surgery, except for one patient. Despite no radiographic osteoarthritis exacerbation before or after surgery in six patients, five patients had osteoarthritic change. CONCLUSION: Cement implantation and autologous bone graft performed simultaneously for benign bone tumors with joint cartilage damage and AVN are technically simple, have good outcomes, and may be a suitable alternative to standard treatments.

Intra-articular Osteoid Osteoma of the Olecranon Fossa.

Osteoid osteomas are benign primary bone lesions characterized by a central nidus with surrounding reactive sclerosis, classically presenting as worsening nocturnal pain relieved by non-steroidal anti-inflammatory medications (NSAIDs). These most commonly occur in intracortical bone and the diaphysis of long bones. As a rare entity, intra-articular osteoid osteomas present unusually, often resulting in a delayed or incorrect diagnosis. We present a case of an intra-articular osteoid osteoma, emphasizing the importance of MRI in aiding diagnosis in this atypical location.

Recurrent Fusion of the Genes for High-mobility Group AT-hook 2 (HMGA2) and Nuclear Receptor Co-repressor 2 (NCOR2) in Osteoclastic Giant Cell-rich Tumors of Bone.

BACKGROUND/AIM: Chimeras involving the high-mobility group AT-hook 2 gene (HMGA2 in 12q14.3) have been found in lipomas and other benign mesenchymal tumors. We report here a fusion of HMGA2 with the nuclear receptor co-repressor 2 gene (NCOR2 in 12q24.31) repeatedly found in tumors of bone and the first cytogenetic investigation of this fusion. MATERIALS AND METHODS: Six osteoclastic giant cell-rich tumors were investigated using G-banding, RNA sequencing, reverse transcription polymerase chain reaction, Sanger sequencing, and fluorescence in situ hybridization. RESULTS: Four tumors had structural chromosomal aberrations of 12q. The pathogenic variant c.103_104GG>AT (p.Gly35Met) in the H3.3 histone A gene was found in a tumor without 12q aberration. In-frame HMGA2-NCOR2 fusion transcripts were found in all tumors. In two cases, the presence of an HMGA2-NCOR2 fusion gene was confirmed by FISH on metaphase spreads. CONCLUSION: Our results demonstrate that a subset of osteoclastic giant cell-rich tumors of bone are characterized by an HMGA2-NCOR2 fusion gene.

Posterior ankle arthroscopic treatment of a talar chondroblastoma with allograft and a platelet-rich plasma-fibrin glue: A case report and literature review.

Talar chondroblastoma, which is a benign tumor of immature cartilage cells, is an uncommon but easily missed diagnosis of foot pain. Arthroscopic treatment for this condition is a safe, powerful, and promising technique with definitive advantages of visualization and minimal invasion. Here, we report a case of a talar chondroblastoma treated by posterior ankle arthroscopic curettage, allograft bone graft, and platelet-rich plasma-fibrin glue (PRP-FG) application. Level of evidence: Case Report. Level IV.

A Case Report on Surgical Excision of Intracapsular Osteochondroma of Femur Neck using Mini-Arthrotomy without Hip Dislocation in a Young Female with Hereditary Multiple Exostoses.

Introduction: Osteochondromas are the most common benign bone tumors. They probably are developmental malformations rather than true neoplasms and are thought to originate within the periosteum as small cartilaginous nodules. The lesions consist of a bony mass produced by progressive endochondral ossification of a growing cartilaginous cap. Osteochondromas usually are found on the metaphysis of a long bone near the physis such as distal femur, proximal tibia, and proximal humerus. Surgical treatment for femur neck osteochondroma is difficult due to the high risk of avascular necrosis following excision. These lesions in femur are in close proximity to important neurovascular bundle and can cause symptoms related to their compression. Furthermore, the symptoms related to labral tear and hip impingement are common. Recurrence is rare and is caused by failure to remove the entire cartilaginous cap. Case Report: A 25-year-old female presented with the complaints of pain in the right hip and difficulty in walking and running for 1 year. On radiological examination, the right femur neck osteochondroma was diagnosed, it was located along the posteroinferior margin of the femur neck. Surgical removal of the lesion was done in lateral decubitus position using posterolateral approach to hip without dislocation of the femur. Conclusion: Osteochondroma at femur neck can be safely removed without surgical hip dislocation. It's necessary to remove it completely to avoid recurrence.