Search Results (6)

Background: Giant cell tumor of bone (GCTB) is a locally aggressive tumor. It accounts for only 5% of all bony tumors. Early diagnosis, and follow-up for recurrence is often difficult due to a lack of biogenetic markers. Giant cells are multinucleated epithelioid cells derived from macrophages. Histologically, giant cells are also present in other pathologies of bone, e.g., aneurysmal bone cyst, chondroblastoma, giant cell granuloma, and malignant giant cell tumor, etc. Similarly, radiographic findings overlap with other osteolytic lesions, making the diagnosis and prognosis of giant cell tumor very challenging. Aims and Objectives: The purpose of this study was to explore biological and genetic markers which can be used for detection, differentiation, recurrence, and prognosis of GCTB. This will help to better understand the clinical outcome of GCTB and minimize the need for interventions. Methods: We conducted a literature search using Google, Google Scholar, PubMed, Wiley Library, Medline, Clinical trials.org, and Web of Science. Our search strategy included MeSH terms and key words for giant cell tumor and biogenetic markers from date of inception to September 2020. After excluding review articles, 246 duplicates, and non-relevant articles, we included 24 articles out of 1568 articles, summarizing the role of biogenetic markers in the prognosis of GCT. Results: P63 is 98.6% sensitive and relatively specific for GCT as compared to other multinucleated giant cells containing neoplasms. MDM2 (mouse double minute 2 homolog), IGF1 (insulin-like growth factor 1), STAT1 (signal transducer and activator of transcription 1), and RAC1 (Ras-related C3 botulinum toxin substrate 1) are associated with GCTB recurrence, and might serve as biomarkers for it. Increased expression of the proteins STAT5B, GRB2, and OXSR1 was related to a higher probability of metastasis. H3F3A and H3F3B mutation analysis appears to be a highly specific, although less sensitive, diagnostic tool for the distinction of giant cell tumor of bone (GCTB) and chondroblastoma from other giant cell-containing tumors. A neutrophil to lymphocyte ratio (NLR) > 2.70, platelet to lymphocyte ratio (PLR) > 215.80, lymphocyte to monocyte ratio (LMR) ≤ 2.80, and albumin to globulin ratio (AGR) < 1.50 were significantly associated with decreased disease-free survival (DFS) (p < 0.05). Large amounts of osteoclast-related mRNA (cathepsin K, tartrate-resistant acid phosphatase, and matrix metalloproteinase9) in GCTs (p < 0.05) are associated with the grade of bone resorption. We propose that subarticular primary malignant bone sarcomas with H3.3 mutations represent true malignant GCTB, even in the absence of a benign GCTB component. IMP3 and IGF2 might be potential biomarkers for GCT of the spine in regulating the angiogenesis of giant cell tumor of bone and predicting patients’ prognosis. Conclusions: This review study shows serological markers, genetic factors, cell membrane receptor markers, predictive markers for malignancy, and prognostic protein markers which are highly sensitive for GCT and relatively specific for giant cell tumor. MDM2, IGF1, STAT1, RAC1 are important makers in determining recurrence, while P63 and H3F3A differentiate GCT from other giant cell-containing tumors. STAT5B, GRB2, and OXSR1 are significant in determining the prognosis of GCT. Apart from using radiological and histological parameters, we can add them to tumor work-up for definitive diagnosis and prognosis. Full article

Chondroblastoma metastasis, though rare, represents a clinically significant and notably important aspect of bone tumors. Understanding its epidemiological characteristics, pathological features, and treatment modalities, despite its infrequency, is imperative for comprehensive patient management. This review aims to elucidate the epidemiology, molecular mechanisms, diagnostic challenges, and therapeutic strategies associated with chondroblastoma metastasis. The patterns, prognostic factors, and treatment outcomes were explored through an analysis of case studies and clinical reports. Notably, we highlighted emerging therapeutic perspectives aimed at improving patient outcomes. To the best of our knowledge, there has been no previous review addressing these matters cumulatively, highlighting a significant gap in the existing scholarly literature. By shedding light on the nuances of chondroblastoma metastasis, this review contributes to the advancement of knowledge in this field and informs clinical decision-making for improved patient care. Full article

Background: Chondroblastoma (CB), a rare benign bone tumor that produces chondrocytes, often develops in the epiphysis or apophysis of children and young adults. The treatment of these rare tumors is complex. The standard treatment protocol involves curettage with local adjuvants and bone graft or cement application. The authors examined 38 CBs to determine risk factors for local recurrence, complications, and functional outcomes following epiphyseal curettage. Methods: Twenty-two girls and sixteen boys aged 10 to 17 years with histologically confirmed chondroblastoma who arrived at our hospital between January 2000 and June 2021 were reviewed retrospectively. Clinical data, radiographic images, histological results, treatment, functional outcomes, and the local recurrence rate were examined—surgical treatment involved total tumor curettage, followed by bone grafting and adjuvant techniques. Local recurrences have also been reported. Results: The most frequently affected site was the proximal femur. Sites of involvement included the proximal femur in 10 (26.3%) cases, the proximal tibia in 8 (20.8%), the humerus in 5 cases (13.2%), the distal tibia in 4 cases (10.5%), the distal femur in 3 cases (7.9%), the supracetabular region in 3 cases (7.9%), the talus in 1 case (2.6%), the calcaneus in 1 case (2.6%), the scapula in 1 case (2.6%), the lumbar spine in 1 case (2.6%), and the iliac bone in 1 (2.6%) patient. The mean follow-up was 144.2 months (24 to 276). The local recurrence rate was 7.9%. The mean Musculoskeletal Tumor Society (MSTS) score was 28.3 points (17 to 30). The mean duration of symptoms at presentation was 5.8 (range, 1 to 28) months. Conclusion: Aggressive curettage and bone grafting resulted in local control and good outcomes in most pediatric patients. In a relatively small proportion of cases, long-term complications and recurrence can occur due to growth plate damage and late diagnosis. In patients admitted to the pediatric clinic with pain, which is often accompanied by localized edema and joint effusion, early detection via advanced radiological scans (X-ray, CT, or MRI) may prevent delays in diagnosis. Full article

Giant cells (GCs) are thought to originate from the fusion of monocytic lineage cells and arise amid multiple backgrounds. To compare GCs of different origins, we immunohistochemically characterised the GCs of reactive and neoplastic lesions (n = 47). We studied the expression of 15 molecules including HLA class II molecules those relevant to the cell cycle, bone metabolism and lineage affiliation. HLA-DR was detectable in the GCs of sarcoidosis, sarcoid-like lesions, tuberculosis, and foreign body granuloma. Cyclin D1 was expressed by the GCs of neoplastic lesions as well as the GCs of bony callus, fibroid epulis, and brown tumours. While cyclin E was detected in the GCs of all lesions, p16 and p21 showed a heterogeneous expression pattern. RANK was expressed by the GCs of all lesions except sarcoid-like lesions and xanthogranuloma. All GCs were RANK-L-negative, and the GCs of all lesions were osteoprotegerin-positive. Osteonectin was limited to the GCs of chondroblastoma. Osteopontin and TRAP were detected in the GCs of all lesions except xanthogranuloma. RUNX2 was heterogeneously expressed in the reactive and neoplastic cohort. The GCs of all lesions except foreign body granuloma expressed CD68, and all GCs were CD163- and langerin-negative. This profiling points to a functional diversity of GCs despite their similar morphology. Full article

Benign tumours comprise the majority of primary vertebral tumours, and these are often found incidentally on imaging. Nonetheless, accurate diagnosis of these benign lesions is crucial, in order to avoid misdiagnosis as more ominous malignant lesions or infection. Furthermore, some of these tumours, despite their benign nature, can have localised effects on the spine including neural compromise, or can be locally aggressive, thus necessitating active management. Haemangiomas and osteomas (enostosis) are the commonest benign tumours encountered. Others include osteoid osteoma, osteoblastoma, fibrous dysplasia, osteochondroma, chondroblastoma, haemangioma, simple bone cysts, aneurysmal bone cysts, giant cell tumours, eosinophilic granuloma and notochordal rests. The majority of lesions are asymptomatic; however, locally aggressive lesions (such as aneurysmal bone cysts or giant cell tumours) can present with nonspecific symptoms, such as back pain, neurological deficits and spinal instability, which may be indistinguishable from more commonly encountered mechanical back pain or malignant lesions including metastases. Hence, imaging, including radiography, computed tomography (CT) and magnetic resonance imaging (MRI), plays a critical role in diagnosis. Generally, most incidental or asymptomatic regions are conservatively managed or may not require any follow-up, while symptomatic or locally aggressive lesions warrant active interventions, which include surgical resection or percutaneous treatment techniques. Due to advances in interventional radiology techniques in recent years, percutaneous minimally invasive techniques such as radiofrequency ablation, sclerotherapy and cryoablation have played an increasing role in the management of these tumours with favourable outcomes. The different types of primary benign vertebral tumours will be discussed in this article with an emphasis on pertinent imaging features. Full article

In this review, we describe the attributes of histone H3 mutants identified in cancer. H3 mutants were first identified in genes encoding H3.3, in pediatric high-grade glioma, and subsequently in chondrosarcomas and giant cell tumors of bone (GCTB) in adolescents. The most heavily studied are the lysine to methionine mutants K27M and K36M, which perturb the target site for specific lysine methyltransferases and dominantly perturb methylation of corresponding lysines in other histone H3 proteins. We discuss recent progress in defining the consequences of these mutations on chromatin, including a newly emerging view of the central importance of the disruption of H3K36 modification in many distinct K to M histone mutant cancers. We also review new work exploring the role of H3.3 G34 mutants identified in pediatric glioma and GCTB. G34 is not itself post-translationally modified, but G34 mutation impinges on the modification of H3K36. Here, we ask if G34R mutation generates a new site for methylation on the histone tail. Finally, we consider evidence indicating that histone mutations might be more widespread in cancer than previously thought, and if the perceived bias towards mutation of H3.3 is real or reflects the biology of tumors in which the histone mutants were first identified. Full article