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Background: The purpose of this study was to evaluate the implant survival, functional score and complications of intercalary endoprostheses implanted for metastatic involvement of the femoral and humeral diaphysis. Methods: The selected group covered patients with bone metastasis who were surgically treated with an intercalary endoprosthesis between 2012 and 2021. The functional outcome was evaluated with the Musculoskeletal Tumor Society (MSTS) scoring system, and complications were evaluated by using the failure classification for prosthetics designed by Henderson. Results: The mean follow-up was 29.8 months. In our group of 25 patients with 27 intercalary endoprostheses (18 femurs, 9 humeri), there were 7 implant-related complications (25.9%), which were more common on the humerus (4 cases, 44.4%) than on the femur (3 cases, 16.7%). Only type II failure—aseptic loosening (5 cases, 18.5%)—and type III failure—structural failure (2 cases, 7.4%)—occurred. There was a significantly higher risk of aseptic loosening of the endoprosthesis in the humerus compared with that in the femur (odds ratio 13.79, 95% confidence interval 1.22–151.05, p = 0.0297). The overall cumulative implant survival was 92% 1 year after surgery and 72% 5 years after surgery. The average MSTS score was 82%. The MSTS score was significantly lower (p = 0.008) in the humerus (75.9%) than in the femur (84.8%). Conclusions: The resection of bone metastases and replacement with intercalary endoprosthesis has excellent immediate functional results with an acceptable level of complications in prognostically favourable patients.

INTRODUCTION We aimed to identify imaging-based findings that can differentiate between spinal subchondral bone metastasis with focal pathologic endplate fracture and oedematous Schmorl's nodes that have been histopathologically confirmed. METHODS Between March 2010 and April 2016, 11 patients who had undergone spinal magnetic resonance (MR) imaging or computed tomography (CT) with final radiologic reports that included 'subchondral bone metastasis with focal pathologic endplate fracture' or 'edematous Schmorl's node' and had also undergone percutaneous imaging-guided spinal biopsies were included. Two radiologists retrospectively evaluated the following imaging features in consensus: size, location, presence of sclerotic margin, presence of intralesional or perilesional enhancement and opposite endplate enhancement of the involved disc, presence of disc height loss and presence of metabolic uptake at a corresponding lesion on nuclear medicine imaging. RESULTS A total of 11 patients, including six patients with spinal subchondral bone metastasis with focal pathologic endplate fracture and five patients with oedematous Schmorl's nodes, were included in this study (median age, 58 years; range, 50-63 years; six men). Sclerotic margin (P = 0.002) and enhancement on the opposite endplate of the involved disc (P = 0.047) were significantly different between oedematous Schmorl's node and subchondral bone metastasis with focal pathologic endplate fracture. CONCLUSION Sclerotic margin and enhancement on the opposite endplate of the involved disc suggest oedematous Schmorl's node rather than subchondral bone metastasis with focal pathologic endplate fracture. Decreased disc height is likely to be an oedematous Schmorl's node rather than subchondral bone metastasis with focal pathologic endplate fracture.

A. Kawai43, K. Kopeckova44, D. A. Krakorova45, A. Le Cesne46, F. Le Grange1, E. Legius47, A. Leithner48, A. Lopez Pousa49, J. Martin-Broto36, O. Merimsky50, C. Messiou51, A. B. Miah52, O. Mir53, M. Montemurro54, B. Morland55, C. Morosi56, E. Palmerini57, M. A. Pantaleo58, R. Piana59, S. Piperno-Neumann60, P. Reichardt61, P. Rutkowski62, A. A. Safwat63, C. Sangalli64, M. Sbaraglia19, S. Scheipl48, P. Schoffski65, S. Sleijfer66, D. Strauss67, K. Sundby Hall13, A. Trama68, M. Unk69, M. A. J. van de Sande70, W. T. A. van der Graaf66,71, W. J. van Houdt72, T. Frebourg73x, R. Ladenstein41z, P. G. Casali2,74z &

Introduction Bone metastases are frequent in patients with cancer. Electrochemotherapy (ECT) is a minimally invasive treatment. Preclinical and clinical studies supported the use of ECT in patients with metastatic bone disease (MBD). The purposes of this multicentre study are to confirm the safety and efficacy of ECT, and to identify appropriate operating procedures in different MBD conditions. Materials and methods 102 patients were treated in 11 Centres and recorded in the REINBONE registry (a shared database protected by security passwords): clinical and radiological information, ECT session, adverse events, response, quality of life indicators and duration of follow-up were registered. Results 105 ECT sessions were performed (one ECT session in 99 patients, two ECT sessions in 3 patients). 24 patients (23.5%) received a programmed intramedullary nail after ECT, during the same surgical procedure. Mean follow-up was 5.9 ± 5.1 months (range 1.5–52). The response to treatment by RECIST criteria was 40.4% objective responses, 50.6% stable disease and 9% progressive disease. According to PERCIST criteria the response was: 31.4% OR; 51.7% SD, 16.9% PD with no significant differences between the 2 criteria. Diagnosis of breast cancer and ECOG values 0–1 were significantly associated to objective response. A significant decrease in pain intensity and significant better quality of life was observed after ECT session at follow-up. Conclusion The results are encouraging on pain and tumour local control. ECT proved to be an effective and safe treatment for MBD and it should be considered as an alternative treatment as well as in combination with radiation therapy.

Background: Osteosarcoma (OS) is the basic bone neoplasm with lower survival and poor prognosis. It is distinguished by its offensive nature and metastatic potential. The fundamental death source in OS patients is lung metastasis. In addition, the proliferation and cell migration are thus essential for cancer progression, especially for intrusion and transformation. Several studies have illustrated that 1,25-Dihydroxyvitamin D (1,25(OH)2D) has a critical role in the growth and differentiation of bone. However, knowledge of the outcome of 1,25(OH)2D on the progression and incursion of osteosarcoma cells is minimal. Objective: The present study aimed to analyze the effect of different concentrations of 1,25(OH)2D on the multiplication, progression, and intrusion of OS cells and verify the effective doses of 1,25(OH)2D that can decrease the intensity of the disease and improving the prognosis in OS patients. Methods: Saos-2 cells were treated with 1,25(OH)2D (0, 50, 100, and 200 nM) for 48, 72, and 96 hours. Proliferation, invasion, and migration were determined by MTT assay, Transwell assay, and Scratch test, respectively. The levels of c-Myc and FOXO1 proteins were determined by Western blotting. Results: The proliferation, invasiveness, and migration of Saos-2 cells that were treated with 1,25(OH)2D were significantly decreased compared with untreated cells. Although 1,25(OH)2D notably decreased c-Myc protein levels (after 48 and 72 hours), FOXO1 protein levels have been significantly increased after 48 and 72 hours. 1,25(OH)2D and the vitamin D receptor (VDR) suppress c-Myc function through regulating the c-Myc/MXD1 network and thus, providing a molecular basis of 1,25(OH)2D related to the cancer-preventive actions. Conclusion: Based on the present results, 1,25(OH)2D by targeting c-Myc and FOXO1 expression displays anti-invasive, anti-migration and anti-proliferative effects on OS cells in vitro. Our findings suggest that effective doses of the 1,25(OH)2D may reduce the aggressive potential of the OS cell line. However, further investigation and clinical trials are needed.

Osteosarcoma (OS) is a foremost mesenchymal bone neoplasm and it can occur at any age with survival rate is nearly 2–8 times lesser in elders than in teenagers. The clinical therapies for cancer treatment have gradually becoming outdated because of the developments of nano-medicine and multi-targeted drug-delivery. In this work, we green synthesized the zinc oxide nanoparticles from the Cassia auriculata flower (AS-ZnONPs) extract and evaluated its antimicrobial and in vitro anticancer potential against the OS MG-63 cells. The synthesized AS-ZnONPs were confirmed and characterized by using UV–vis spectroscopy, XRD, FE-SEM, and photoluminescence techniques. The antimicrobial activity of AS-ZnONPs was studied by disc diffusion technique. The viability of AS-ZnONPs treated MG-63 cells were examined by MTT assay. The apoptotic cells in the AS-ZnONPs treated MG-63 cells were assayed by dual staining. The MMP status of AS-ZnONPs treated cells were tested by Rh-123 staining. The cell adhesion assay was performed to detect the anticancer effects of AS-ZnONPs against MG-63 cells. The results of UV–vis spectroscopy, XRD, FE-SEM, and photoluminescence techniques proved the formation of AS-ZnONPs and it has the hexagonal wurtzite structures. AS-ZnONPs displayed the potent antimicrobial activity against the tested microbial strains. The AS-ZnONPs were appreciably inhibited the cell viability of MG-63 cells. The outcomes of fluorescence staining proved that AS-ZnONPs reduced the MMP and prompted the apoptosis in MG-63 cells. In conclusion, our discoveries demonstrated that the formulated AS-ZnONPs has the potent antimicrobial and in vitro anticancer activity against the MG-63 cells. The AS-ZnONPs could be potent chemotherapeutic agent in the future to treat the OS.

Aims Chondrosarcoma is the second most common primary sarcoma of bone: conventional chondrosarcoma accounts for 85% of all cases. Conventional chondrosarcoma may be central or peripheral. Most studies group central and peripheral chondrosarcomas together, although there is growing evidence that their clinical behaviour and prognosis differ. The aims of this study were to analyze any differences in characteristics between central and peripheral chondrosarcomas and to investigate the incidence and role of different syndromes. Methods Data from two international tertiary referral sarcoma centres between January 1995 and December 2018 were retrospectively reviewed. The study population consisted of 714 patients with surgically treated conventional chondrosarcoma of the pelvis and limbs. Results In patients with Ollier’s disease and Mafucci’s syndrome, 12/20 (60%) and 2/5 (60%) of malignancies, respectively, were in the limbs, most frequently in the proximal humerus, proximal tibia, and in the hands and feet. In patients with hereditary multiple exostosis (HME), 20/29 (69.0%) of chondrosarcomas were in the pelvis and scapula, specifically in the ilium in 13/29 (44.8%) and the scapula in 3/29 (10.3%). In central chondrosarcoma, survival of patients with Ollier’s disease and non-syndromic patients was the same (p = 0.805). In peripheral chondrosarcoma, survival among HME patients was similar (p = 0.676) in patients with tumours of the pelvis and limbs. Conclusion Both central and peripheral chondrosarcoma have specific characteristics. HME is frequently seen in patients with a peripheral chondrosarcoma, in whom tumours are commonly located in the ilium and scapula. The incidence of Ollier’s disease is uncommon in patients with a central chondrosarcoma. Disease-specific survival is equal in different subtypes after adjustment for histological grade. The local recurrence-free survival is the same for different locations and subtypes after adjustment for surgical margin. Cite this article: Bone Joint J 2021;103-B(5):984–990.

PURPOSE: A T1 sequence on routine baseline staging rectal magnetic resonance imaging (MRI) is thought to help detect bone lesions. Our primary aim was to evaluate the incidence of bone lesions encountered on baseline staging rectal MRI, particularly the prevalence of bone metastases. METHODS: This retrospective study included patients with rectal adenocarcinoma who underwent baseline rectal MRI at our institution between January 2010 and December 2017. The MRI report was reviewed for presence of bone lesions. When found, lesion type, presence of axial T1 non-fat-suppressed sequence, primary tumor T-stage, and presence of other organ metastases were recorded. In the absence of bone biopsy, the reference standard was follow-up imaging via computed tomography (CT), MRI, and/or positron emission tomography/CT (PET/CT) ≥ 1 year after the baseline MRI. The Wilcoxon rank-sum test and Fisher’s exact test were used to compare clinicopathologic data of patients with malignant or benign bone lesions. RESULTS: A total of 1,197 patients were included. 62/1197 patients (mean age 56.8 years (SD: 13.8), with 39 men) had bone lesions on baseline imaging, with 6 being bone metastases (0.5%, 95% CI: 0.2%–1.1%). Of the 6 patients with bone metastases, 5/6 had other metastases (i.e. liver, lung) at baseline. CONCLUSION: Bone metastases on baseline rectal MRI performed for rectal adenocarcinoma are extremely rare. Furthermore, bone metastases without other organ (i.e. liver, lung) involvement is extremely rare.

Aim This study aimed to identify the risk of pathological fractures after bone tumour biopsy. Materials and methods Fifty rabbit femurs were divided into groups according to defect size: Control (no defect), type 1 (10% width), type 2 (20% width), type 3 (30% width), and type 4 (40% width). Another 20 were also divided into control, type A (27% length), type B (40% length), and type C (53% length) groups. Performing femoral head compression testing allowed each parameter (maximum load, displacement, elastic modulus, and fracture energy) to be calculated individually. Results Compressive maximum load was significantly higher for type 1 than for the other types when testing rectangular defects of different widths, while there were no significant differences between the three types when testing by defect length. Conclusion It may be useful for orthopaedic oncologists to make a rectangular biopsy hole with a width measuring less than 10% of the circumference and to enlarge the hole longitudinally to avoid pathological fracture.

Background: Trans-arterial embolization (TAE) is a prom-ising less aggressive modality in either primary treatment or adjuvant to surgical management of bone tumors. The main aim is to induce necrosis and decrease bleeding and therefore facilitate resection. Palliation of compression manifestation of inoperable bone neoplasm is another indication of TAE. Frequent tumors selected to this modality include vascular tumors (particularly metastases), aneurysmal bone cysts (ABC), giant cell tumor (GCT), and osteosarcoma. Aim of Study: Our aim is to assess the role of embolization in management of bone tumors. Patients and Methods: The current study prospectively evaluated 33 patients (23 males and 10 females). Their age ranged from 9 to 69 years (mean age of 43.246±19.65). Extremities, hip, vertebrae, and skull were involved by variety of bony tumors. Sixteen patients were affected by primary osseous tumors, while 17 patients had metastases. A transar-terial embolization was done in all patients as a preoperative procedure in 15 patients, definite treatmentof primary benign inaccessible tumors in 6 patients, prebiopsy embolization in 6 patients, and as a palliative treatment in 6 patients. Results: Technical success was obtained in 30 out of 33 cases with percentage of 90.9%. All benign and primary malignant bony tumors showed technical success while, 82.4% of metastatic lesions did. Clinical success was achieved in 73.3% cases of first group, 83.3% of second group, 100% of third group and 66.7% of fourth group. Four patients (12.1%) showed no clinical improvement with 9% lost follow-up. Five out of 6 benign tumors showed clinical success (83.3%) as the sixth case lost follow-up (16.7%). Embolization of 9 of primary malignant lesions was clinically successful (90%), whereas 12 out of 17 metastatic lesions were improved clinically (70.6 %), 3 cases showed no clinical success (17.6%), and 2 cases were lost at follow-up (11.8%).Conclusion: A high consideration should be given totran-sarterialembolization in the algorithmic treatment strategy of bone tumors as it is much safer and effective tool when diminution of bleeding and compression manifestations is to be specifically aimed.

Cancer causes inflammation as it progresses through healthy tissue. The differentiation of tumoral growth from the surrounding inflammatory change is paramount in planning surgeries seeking to preserve function. This retrospective study aims at illustrating how a careful use of imaging (computed tomography (CT)/magnetic resonance imaging (MRI)) can help to draw the line between infiltration and inflammation. Out of 72 cases of parosteal osteosarcoma in our institution we selected 22 which had pretreatment imaging, and out of those, 14 that had both MRI and CT. Using Fisher’s exact test, we evaluated the performance of each technique on accurately diagnosing medullary tumor infiltration, using histological analysis as a gold standard. All cases (14/14) demonstrated medullary abnormality on MRI, but only 6/14 (42.9%) demonstrated abnormality on CT. The 8/14 cases with MRI abnormality but no CT abnormality (57.1%) showed inflammation with no tumoral cells present on histological analysis. In the cases where the two examinations showed medullary abnormality (6/14) histology demonstrated tumoral infiltration. MRI demonstrated high sensitivity and negative predictive value, but low specificity and low positive predictive value and accuracy (P=1). CT demonstrated high sensitivity, specificity, high positive and negative predictive values and accuracy (P = 0.000333). MRI is highly sensitive for the detection of medullary abnormality but lacks specificity for tumor invasion. Correlation with CT is recommended in all cases of positive MR to add specificity for tumors. The adequate use of the two imaging methods allows to differentiate between inflammatory change and tumoral infiltration in POS, relevant for surgical planning.

Background There is no standard treatment for giant cell tumors of the sacrum. We compared the outcomes and complications in patients with sacral giant cell tumors who underwent intralesional nerve-sparing surgery with or without (neo-) adjuvant therapies versus those who underwent non-surgical treatment (denosumab therapy and/or embolization). Methods We retrospectively investigated 15 cases of sacral giant cell tumors treated at two institutions between 2005 and 2020. Nine patients underwent intralesional nerve-sparing surgery with or without (neo-) adjuvant therapies, and six patients received non-surgical treatment. The mean follow-up period was 85 months for the surgical group (range, 25–154 months) and 59 months (range, 17–94 months) for the non-surgical group. Results The local recurrence rate was 44% in the surgical group, and the tumor progression rate was 0% in the non-surgical group. There were two surgery-related complications (infection and bladder laceration) and three denosumab-related complications (apical granuloma of the tooth, stress fracture of the sacroiliac joint, and osteonecrosis of the jaw). In the surgical group, the mean modified Biagini score (bowel, bladder, and motor function) was 0.9; in the non-surgical group, it was 0.5. None of the 11 female patients became pregnant or delivered a baby after developing a sacral giant cell tumor. Conclusions The cure rate of intralesional nerve-sparing surgery is over 50%. Non-surgical treatment has a similar risk of complications to intralesional nerve-sparing surgery and has better functional outcomes than intralesional nerve-sparing surgery, but patients must remain on therapy over time. Based on our results, the decision on the choice of treatment for sacral giant cell tumors could be discussed between the surgeon and the patient based on the tumor size and location.

Cisplatin (CDDP) is commonly used to treat a multitude of tumors including sarcomas, ovarian and cervical cancers. Despite recent investigations allowed to improve chemotherapy effectiveness, the molecular mechanisms underlying the development of CDDP resistance remain a major goal in cancer research. Here, we show that mitochondrial morphology and autophagy are altered in different CDDP resistant cancer cell lines. In CDDP resistant osteosarcoma and ovarian carcinoma, mitochondria are fragmented and closely juxtaposed to the endoplasmic reticulum; rates of mitophagy are also increased. Specifically, levels of the mitophagy receptor BNIP3 are higher both in resistant cells and in ovarian cancer patient samples resistant to platinum-based treatments. Genetic BNIP3 silencing or pharmacological inhibition of autophagosome formation re-sensitizes these cells to CDDP. Our study identifies inhibition of BNIP3-driven mitophagy as a potential therapeutic strategy to counteract CDDP resistance in ovarian carcinoma and osteosarcoma.

OBJECTIVE To determine an optimal time interval between amputation and initiation of adjuvant chemotherapy (TIamp-chemo) in dogs with appendicular osteosarcoma without distant metastases and whether TIamp-chemo was associated with outcome. ANIMALS 168 client-owned dogs treated at 9 veterinary oncology centers. PROCEDURES Data were collected from the dogs’ medical records concerning potential prognostic variables and outcomes. Dogs were grouped as to whether they received chemotherapy within 3, 5, 7, 10, 15, 20, 30, or > 30 days after amputation of the affected limb. Analyses were performed to identify variables associated with time to tumor progression and survival time after limb amputation and to determine an optimal TIamp-chemo. RESULTS Median TIamp-chemo was 14 days (range, 1 to 210 days). Median time to tumor progression for dogs with a TIamp-chemo ≤ 5 days (375 days; 95% CI, 162 to 588 days) was significantly longer than that for dogs with a TIamp-chemo > 5 days (202 days; 95% CI, 146 to 257 days). Median overall survival time for dogs with a TIamp-chemo ≤ 5 days (445 days; 95% CI, 345 to 545 days) was significantly longer than that for dogs with a TIamp-chemo > 5 days (239 days; 95% CI, 186 to 291 days). CONCLUSIONS AND CLINICAL RELEVANCE Findings indicated that early (within 5 days) initiation of adjuvant chemotherapy after limb amputation was associated with a significant and clinically relevant survival benefit for dogs with appendicular osteosarcoma without distant metastases. These results suggested that the timing of chemotherapy may be an important prognostic variable.

An imbalance between protein aggregation and protein degradation may induce ‘stress’ in the functionality of the endoplasmic reticulum (ER). There are quality control (QC) mechanisms to minimize misfolding and to eliminate misfolded proteins before aggregation becomes lethal for the cell. Proper protein folding and maturation is one of the crucial functions of the ER. Chaperones of the ER and folding enzymes guarantee correct conformational maturation of emerging secretory proteins. Histone deacetylase (HDAC) 6 (HDAC6) is a masterpiece coordinating the cell response to protein aggregate formation. The balance between HDAC6 and its partner Valosin-containing protein/p97 determines the fate of polyubiquitinated misfolded proteins. WT161 is a terrific, selective, and bioavailable HDAC6 inhibitor. WT161 selectively inhibits HDAC6 and adequately increases levels of acetylated α-tubulin. This compound induces accumulation of acetylated tubulin and cytotoxicity in multiple myeloma (MM) cells. In this journal, Sun et al. (Biosci. Rep.41, DOI: 10.1042/BSR20203905) identified that WT161 suppresses the cell growth of osteosarcoma cells. This discovery opens the door to future chemotherapeutic regimens of this bone neoplasm.

Multilobular tumor of bone (MLTB) is an infrequent, slow-growing, bone neoplasm formed predominantly on the head. These tumors can behave as malignant neoplasms clinically and pathologically and can metastasize occasionally. No cases of MLTB in rodents have been reported, to our knowledge. We describe a novel case of an MLTB in a guinea pig. An adult guinea pig had an exophytic mass fixed on the frontal bone, maxilla, and nasal bone. On radiography, the mass had a spherical contour and variable density and was formed on the surface of the cranial bones. The mass was excised surgically. The cut surface was light-yellow to milky-white and had a granular texture with fine fibrous septa. Histologically, the neoplasm had a multilobular pattern, which consisted of many islands of bone and/or cartilage matrix surrounded by small cells and separated by fibrous septa, which closely resembles the equivalent neoplasm in dogs.

Recently, specific driver mutations were identified in chondroblastoma, giant cell tumour of bone and central cartilaginous tumours (specifically enchondroma and central chondrosarcoma), sharing the ability to induce genome-wide epigenetic alterations. In chondroblastoma and giant cell tumour of bone, the neoplastic mononuclear stromal-like cells frequently harbour specific point mutations in the genes encoding for histone H3.3 (H3F3A and H3F3B). The identification of these driver mutations has led to development of novel diagnostic tools to distinguish between chondroblastoma, giant cell tumour of bone and other giant cell containing tumours. From a biological perspective, these mutations induce several global and local alterations of the histone modification marks. Similar observations are made for central cartilaginous tumours, which frequently harbour specific point mutations in the metabolic enzymes IDH1 or IDH2. Besides an altered methylation pattern on histones, IDH mutations also induce a global DNA hypermethylation phenotype. In all of these tumour types, the mutation-driven epigenetic alterations lead to a highly altered transcriptome, resulting for instance in alterations in differentiation. These genomic alterations have diagnostic impact. Further research is needed to identify the genes and signalling pathways that are affected by the epigenetic alterations, which will hopefully lead to a better understanding of the biological mechanism underlying tumourigenesis.

Introduction: Studies focusing on enchondroma and atypical cartilaginous tumour (ACT) of the pelvis are lacking. The purpose of this study was to verify possible clinical and radiological findings with regard to distinguishing enchondromas from ACT of the pelvis. In addition, this study analysed functional and oncological outcomes in patients with enchondromas or ACT of the pelvis treated with curettage or resection. Materials and methods: We retrospectively reviewed the medical records of 21 patients with confirmed enchondroma or ACT of the pelvis treated by curettage or resection from 1985 to 2018. The minimum follow-up was 18months. The relationship between clinical and radiological factors and tumour type or local recurrence was assessed using Fisher exact test and Mann–Whitney U test. Results: Endosteal scalloping (p = 0.039), tumour size (0.005) and age (0.006) were shown to statistically favour ACT over enchondroma; by contrast, enchondroma and ACT patients had no difference in pain frequency (p = 0.5528). All patients with enchondroma had no local recurrence; in contrast, local recurrence occurred in one patient with ACT, initially treated with resection. The patient with local recurrence had a disease progression with a higher histological grade than the original tumour. Patients treated with curettage had better functional outcomes than patients treated with resection (p = 0.001). Discussion: Endosteal scalloping, tumour size and age could be helpful in the differential diagnosis between enchondroma and ACT of the pelvis. In addition, our study showed that ACT of the pelvis can be safely treated with curettage due to a low risk of local recurrence and better functional results compared with resection. In case of recurrence, we suggest to treat these patients with resection for the risk of disease progression.

Bone and soft tissue sarcomas are uncommon tumors that can occur within the upper extremity as well as elsewhere within the body. However, certain histopathological subtypes have increased affinity for the upper limb and even certain sites within the arm and hand. Other benign masses and tumor mimics, such as infection and traumatic lesions, are more common and imaging appearances can sometimes overlap with malignant lesions making diagnosis difficult. In this article, we explore the current options for imaging of these lesions as well as typical imaging appearances of the more common upper limb tumors.

Technical advances in the last two decades have allowed rapid, high-resolution whole-body magnetic resonance imaging (WBMRI). MRI allows unparalleled visualization and detail in the imaging of bone marrow and surrounding soft tissues. The properties of nuclear magnetic resonance allow superb characterization of bone marrow constituents. WBMRI allows superb characterization of the different types of bone marrow and their natural evolution during the life cycle. Diffusion-weighted WBMRI is an exciting development that adds functional information to anatomical detail. The mainstay of WBMRI for bone marrow abnormalities to date has centered upon staging multiple myeloma and other hematologic bone marrow conditions, and as a valuable tool in quantifying skeletal metastases. Increasingly, WBMRI is being utilized in a variety of additional malignant and nonmalignant conditions. Due to the absence of ionizing radiation, WBMRI represents a valuable screening tool in areas such as malignant transformation in hereditary multifocal exostoses or for the development of ischemic marrow insult in at-risk patients. An additional novel area of use includes postmortem WBMRI for characterization of skeletal injuries. This article provides a state-of-the-art and current review of WBMRI of the bone marrow and highlights potential future areas of development. Level of Evidence: 5 Technical Efficacy Stage: 3 J. MAGN. RESON. IMAGING 2019. J. Magn. Reson. Imaging 2019;50:1687-1701.

How disseminated tumour cells engage specific stromal components in distant organs for survival and outgrowth is a critical but poorly understood step of the metastatic cascade. Previous studies have demonstrated the importance of the epithelial–mesenchymal transition in promoting the cancer stem cell properties needed for metastasis initiation, whereas the reverse process of mesenchymal–epithelial transition is required for metastatic outgrowth. Here we report that this paradoxical requirement for the simultaneous induction of both mesenchymal–epithelial transition and cancer stem cell traits in disseminated tumour cells is provided by bone vascular niche E-selectin, whose direct binding to cancer cells promotes bone metastasis by inducing mesenchymal–epithelial transition and activating Wnt signalling. E-selectin binding activity mediated by the α1-3 fucosyltransferases Fut3/Fut6 and Glg1 are instrumental to the formation of bone metastasis. These findings provide unique insights into the functional role of E-selectin as a component of the vascular niche critical for metastatic colonization in bone.

ResumoOsteoma osteoide é um tumor ósseo benigno, mais frequente dos 10 aos 25 anos de idade e, em cerca de 80% dos pacientes, está associado a dor forte. O presente artigo descreve um paciente masculino apresentando claudicação, encurtamento do membro inferior direito e aumento de volume indolor da perna direita desde os 11 meses de idade. Os exames de imagem demonstraram lesão osteolítica contendo pequenas ossificações de permeio, envolvidas por espessamento cortical da diáfise da tíbia direita. As hipóteses diagnósticas de osteoma osteoide, de osteomielite crônica (abscesso de Brodie), de sarcoma de Ewing e de histiocitose de células de Langerhans foram levantadas. As culturas para microrganismos foram negativas e o exame histopatológico demonstrou osteoma osteoide. O presente relato expande o conhecimento sobre osteoma osteoide como causa de claudicação e discrepância de membros inferiores indolor em lactente. O diagnóstico diferencial precoce é importante, pois a exérese da lesão é curativa e previne sequelas futuras.

Primary aneurysmal bone cyst (ABC) is a cystic bone neoplasm characterized by disease-defining gene fusions involving the USP6/Tre2 gene. The literature describing gnathic ABC is limited. This case report describes a 27-year-old man presenting with a long-standing left-sided facial asymmetry. Multi-detector computed tomography imaging demonstrated a large expansile lesion positioned within the left condylar head. The lesion was biopsied and resected. The specimen showed a giant cell-rich cystic neoplasm, with fibrous tissue lined by multinucleated giant cells. Next-generation sequencing confirmed the presence of a USP6-CDH11 fusion gene, consistent with classification as a primary ABC, the first reported to be translocation-positive in the head of the mandibular condyle.

Chordoma is an uncommon bone neoplasm. Despite being considered a low-grade or benign bone tumor because of its histology, it is aggressive and locally invasive. It has a poor prognosis because it has a high rate of recurrence and is likely to be malignant. Chordoma is usually recurrent. Although its histology is considered a low-grade tumor or benign tumor, its behaviour of recurrence leads to a poor prognosis and higher likelihood of malignant tumors occurring. Chordoma is believed to emerge from transformed remnants of notochord and appears to favor the axial bone. The most commonly affected parts of chordoma are at the sacrum, vertebra, and skull base. The present gold standard for management of chordoma is a wide surgical resection. This article highlights recent practices and future directions for treatment. Keywords : chordoma, recurrent chordoma, treatment of chordoma DOI: 10.31524/bkkmedj.2019.02.019

Osteossarcoma (OSA) é o tumor ósseo primário mais observado em cães. Trata-se de uma neoplasia maligna que afeta a formação do osso, com comportamento clínico-biológico agressivo, crescimento rápido e altamente metastático. É mais observado em cães de meia idade, com porte grande ou gigante e, em geral, restringe-se à metáfise dos ossos longos do esqueleto apendicular. Foi atendido um cão Scottish Terrier macho de dez anos de idade que chegou com claudicação intermitente do membro torácico esquerdo (MTE), sensibilidade à palpação e edema de consistência firme na região cervicotorácica, sem nenhum histórico prévio de fraturas ou traumas. O diagnóstico definitivo de OSA fibroblástico escapular foi realizado com base na associação entre os achados clínicos, radiográficos, citológicos e histopatológicos. O tratamento utilizado foi a amputação do MTE, promovendo alívio da dor com pouca ou nenhuma perda da qualidade de vida.

Purpose: To evaluate the correlation of maximum standardized uptake values (SUVmax) of 18F-Fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) with the apparent diffusion coefficient (ADC) of diffusion weighted imaging (DWI) in musculoskeletal malignancies.Methods: Institutional ethics committee approved this retrospective study. Twenty-seven patients (mean age: 44.85 ± 24.07; 17 men and 10 women) with a total of 29 musculoskeletal tumors underwent both FDG-PET/CT and DWI between January 2017 and March 2020. Region-of-interest (ROI)-based maximal standardized uptake values (SUVmax) of the tumors were measured on FDG-PET/CT images. Two radiologists measured lesions’ mean and minimum apparent diffusion coefficient (ADCmean and ADCmin) using five distinct ROIs on DWI images. Pearson correlation analysis was used to assess the correlation between SUVmax and ADC values.Results: There were 18 soft tissue tumors (62.1%) and 11 bone tumors (37.9%) with a mean maximum diameter of 9.4 ± 6.2 cm. The mean SUVmax, ADCmean and ADCmin of the whole lesions were 12.93 ± 9.63, 0.85 ± 0.28 × 10–3mm2/s and 0.61 ± 0.27 × 10–3mm2/s, respectively. SUVmax had a weak correlation with tumor maximum diameter (r = 0.378, p = 0.043), whereas ADCmean and ADCmin had none. There was strong inverse correlation between SUVmax and both ADCmean (r = –0.616, p < 0.001) and ADCmin (r = –0.638, p < 0.001).Conclusion: In musculoskeletal tumors, quantitative markers of FDG uptake and diffusion restriction strongly correlate.

Myxoid chondrosarcoma is a rare malignant neoplasm of bone or soft tissue origin, characterised by the presence of chondroid and myxoid matrix. These are traditionally radio resistant tumours, so wide surgical resection remains the mainstay of treatment. However, radiotherapy is generally advised for high-grade lesions with poor prognostic factors with adjuvant chemotherapy. The treatment decisions are primarily guided by the histological grade, extent of surgical resection, and site of the neoplasm. A case of a 28-year-old male who presented with recurrent painful swelling in the mandibular region is discussed here. To date, very few cases of Extraskeletal Myxoid Chondrosarcoma (EMC) occurring in the mandible region have been reported. So, due to the paucity of available literature, controversies still exist regarding effective treatment approaches in newly diagnosed and recurrent cases. This case reports the clinical presentation, histopathology, radiological characteristics, immune profile, and treatment modalities used in EMC with a comprehensive and relevant review of the literature.

Osteoid osteoma is a benign osteoblastic tumour constituting 10-20% of all benign tumours. It is rarely seen in the foot and accounts for approximately 4% of all cases. The present case was of a 23-years old male with an osteoid osteoma in the lateral cortex of his medial cuneiform at the articulating surface. He was misdiagnosed for nine-months with initial radiographs and Magnetic Resonance Imaging (MRI) failing to diagnosis the tumour. Extensive conservative treatment failed to resolve his symptoms, although Non-Steroidal Anti-Inflammatory Drug (NSAID) provided relief. Treatment involved excising the osteoid osteoma and filling the void left from the tumour with a synthetic bone graft.

GCTB is an osteolytic, locally-aggressive, rarely-metastasizing tumour, characterized by abundance of osteoclastlike giant cells, induced by neoplastic mononuclear cells expressing high-levels of the receptor activator of nuclear factor Kappa-B ligand (RANKL), a mediator of osteoclast activation. Although the mainstay of treatment is complete tumour removal with preservation of bone, therapy with denosumab, an inhibitor of RANKL, has been introduced for selected cases. Objectives: Denosumab-treated GCTB (DT-GCTB) was reported to show a wide spectrum of histological changes such as depletion of osteoclast-like giant cells and intralesional bone deposition, which may lead to diagnostic difficulties. We investigated clinicopathologic and molecular features of DT-GCTB, matched with pre-therapy samples. Participants: 21 cases were included (13 females, 8 males), aged 15 to 64 (median, 30 years). Results: DT-GCTB showed development of sclerotic neocortex and varying degrees of osteosclerosis radiographically. Marked depletion of giant cells, different degree of ossification, fibrosis, and proliferation of mononuclear cells was observed. Staining for H3.3G34W was positive in mononuclear cells in 19 cases (90.5%), while one negative case was positive for H3.3G34V. H3F3A G34W mutation was confirmed in 17 of 19 cases (89.5%), corresponding to nuclear staining with H3.3 G34W antibody. G34L mutation was detected in one G34W negative case, in which H3.3 G34V nuclear positive staining was observed, possibly due to cross-reaction. Conclusions: Post-therapy tumours still exhibit a similar mutation profile, while significantly differing from classic GCTB morphologically. Correlation with history of denosumab administration, awareness of features of DT-GCTB, IHC and molecular studies for histone H3 mutations are important in its assessment.

Background Osteosarcoma is an aggressive and painful bone neoplasm in dogs. Previous studies have reported epidemiological associations suggesting that large body mass, long bone length and the genetics of certain breeds including the Rottweiler are associated with elevated osteosarcoma risk. However, these studies were often limited by selection bias and confounding factors, and have rarely offered insights into breed-associated protection for osteosarcoma. The current study includes 1756 appendicular and axial osteosarcoma cases presenting to VPG Histology (Bristol, UK) compared against a control population of 905,211 dogs without osteosarcoma from primary care electronic patient records in the VetCompass™ dataset. Methods and study design Retrospective, case-control study. Multivariable logistic regression analysis explored associations between demographic risk factors (including breed, chondrodystrophy, age, sex/neuter status, skull-shape, and body mass) and osteosarcoma of all anatomical sites. Results We identified several breeds with increased and reduced odds of osteosarcoma. At highest risk were the Rottweiler and Great Dane, with > 10 times the odds of osteosarcoma compared with crossbreds, and the Rhodesian Ridgeback, which has not featured in previous lists of at-risk breeds for osteosarcoma, and had an odds ratio of 11.31 (95% confidence interval 7.37–17.35). Breeds at lowest risk of osteosarcoma (protected breeds) included the Bichon Frise, the French Bulldog and the Cavalier King Charles Spaniel, all with odd ratios of less than 0.30 compared with crossbreds. Body mass was strongly associated with osteosarcoma risk; dogs over 40 kg exhibited osteosarcoma odds of 45.44 (95% confidence interval 33.74–61.20) compared with dogs less than 10 kg. Chondrodystrophic breeds had an osteosarcoma odds ratio of 0.13 (95% confidence interval 0.11–0.16) compared with non-chondrodystrophic breeds. Conclusions This study provides evidence of strong breed-associated osteosarcoma risk and protection, suggesting a genetic basis for osteosarcoma pathogenesis. It highlights that breeds selected for long legs/large body mass are generally overrepresented amongst at-risk breeds, whilst those selected for short leg length/small body mass are generally protected. These findings could inform genetic studies to identify osteosarcoma risk alleles in canines and humans; as well as increasing awareness amongst veterinarians and owners, resulting in improved breeding practices and clinical management of osteosarcoma in dogs.

Aims Bone tumours in children/young people are often associated with significant diagnostic delay, increasing the risk of amputation. The total diagnostic interval is a sum total of the patient and system interval. The HeadSmart campaign showed a national awareness campaign based on high quality evidenced based guidance halved time to diagnosis. We conducted a systematic review and meta-analysis to identify the core signs/symptoms and a public survey to understand public awareness of these symptoms. Methods Medline, Pubmed and Embase databases were searched from January 2005 – August 2015. Key words included ‘bone tumour(s)’, ‘bone neoplasm(s)’, ‘diagnosis’, ‘signs(s)’, ‘symptom(s)’, ‘presentation(s)’. All papers discussing bone tumour presentation were included. Pooled proportions (%) of children with each sign/symptom at diagnosis were estimated. A face-to-face opinion survey (n=1000; 475 males) was conducted by Ipsos MORI. Questions included perception of cancer risk, confidence in recognising signs/symptoms and urgency of seeking medical advice. Results The search strategy identified 15477 papers. 713 papers were reviewed in full; 11 met the inclusion criteria, describing the symptoms/signs at diagnosis in 1246 children. 29 symptoms/signs were recorded but only those that occurred in 2% or more of patients are reported. These were pain (76%), swelling (21%), fever (4%), history of trauma (3%), functional limitation (3%), palpable mass (3%), pain and swelling (2%), volume increase (2%), limp (2%) and pathological fracture (2%). Public awareness of the top 4 presenting symptoms of bone tumours were low. 23% of the public identified recurrent or persistent bone pain as a symptom of cancer; 27% identified swelling of bone or joint; 21% identified fever and 14% thought a slow recovery after injury or trauma could be a sign of cancer. Conclusions This is the first systematic review on paediatric bone tumours to our knowledge. It provides strong evidence of the common presentations and will form the basis of a new RCPCH endorsed clinical guideline on the assessment/investigation of suspected bone tumours. The results of the public awareness survey highlight low awareness which need to be tackled in order to drive earlier diagnosis. This will form key messaging in our new national awareness campaign called ChildCancerSmart.

Primary sclerosing epithelioid fibrosarcoma (SEF) of bone is a rare and scarcely reported neoplasm. We document clinicopathological and molecular features of 9 additional cases. Five males and 4 females had a mean age of 39 years (14-71 years). Most tumors affected flat/irregular bones; only 3 cases involved a long bone. By radiology, it has characteristic radiographic features of a predominantly lytic expansile lesion with a sclerotic rim. Referring diagnoses were SEF (n = 2), low-grade osteosarcoma (n = 2), chondrosarcoma (n = 1), and chondromyxoid fibroma (n = 1). Histologically, five cases revealed classical morphology of SEF of soft tissue. Remaining cases were classified as hybrid SEF/low-grade fibromyxoid sarcoma, characterized by spindle or stellate cells, prominent stroma, and giant hyalinized areas. Various morphological deviations such as prominent vasculature (n = 3), osteoid-like material (n = 4), or parallel bone trabeculae (n = 2) were observed. Immunohistochemically, all cases showed diffuse and strong MUC4 expression. SATB2 was observed in 5/8 cases. Using FISH, EWSR1, and FUS rearrangements were detected in 4 cases and 1 case, respectively. EWSR1-CREB3L1 fusion was identified in 1 additional case by next-generation sequencing. Recurrence and metastasis were observed in 1 case and 2 cases, respectively. All but one patient were alive with disease for a mean interval of 31 months. SEF of bone is a relatively indolent sarcoma of adults, most commonly located in the flat/irregular bones. Due to overlapping histological features, it is often misdiagnosed as osteosarcoma or a chondroid tumor. Most SEF of bone exhibit EWSR1 rearrangements, but rare cases may harbor a FUS gene fusion.

Background Diffusion-weighted imaging (DWI) has been described to correlate with tumoural necrosis in response to preoperative chemotherapy for osteosarcoma. Objective To assess the accuracy of DWI in evaluating the response to neoadjuvant chemotherapy at the mid-course treatment of long-bone osteosarcoma and in predicting survival. Materials and methods We conducted a prospective single-centre study over a continuous period of 11 years. Consecutive patients younger than 20 years treated with a neoadjuvant regimen for peripheral conventional osteosarcoma were eligible for inclusion. Magnetic resonance imaging (MRI) with DWI was performed at diagnosis, and mid- and end-course chemotherapy with mean apparent diffusion coefficients (ADC) calculated at each time point. A percentage less than or equal to 10% of the viable residual tissue at the histological analysis of the surgical specimen was defined as a good responder to chemotherapy. Survival comparisons were calculated using the Kaplan-Meier method. Uni- and multivariate analyses with ADC change were performed by Cox modelling. This is an expansion and update of our previous work. Results Twenty-six patients between the ages of 4.8 and 19.6 years were included, of whom 14 were good responders. At mid-course chemotherapy, good responders had significantly higher mean ADC values (P=0.046) and a higher increase in ADC (P=0.015) than poor responders. The ADC change from diagnosis to mid-course MRI did not appear to be a prognosticator of survival and did not impact survival rates of both groups. Conclusion DWI at mid-course preoperative chemotherapy for osteosarcoma should be considered to evaluate the degree of histological necrosis and to predict survival. The anticipation of a response to neoadjuvant treatment by DWI may have potential implications on preoperative management.

There are conflicting reports regarding the outcome and effect of denosumab for distal radius giant cell tumor of bone (GCTB). The authors performed this study to evaluate the behavior of distal radius GCTB in relation to the type of treatment and the administration of denosumab. The files of 72 patients with distal radius GCTB treated from 1984 to 2018 were reviewed. Fourteen patients were administered denosumab. Surgical treatment consisted of curettage (25 patients) or resection (47 patients) and allograft or vascularized fibular head graft reconstruction. Median follow-up was 63.1 months (interquartile range [IQR], 35.5–107.1 months). The authors evaluated local recurrences, metastasis, function, and complications. The local recurrence rate was 30.6% at a median of 14.0 months (IQR, 10–19 months), with no difference between curettage and resection. The local recurrence rate was significantly higher in the patients who received denosumab. The metastasis rate was 9.7% at a median of 41.0 months (IQR, 15–114 months), with no difference regarding denosumab administration. Function was significantly better in patients after curettage. The complication rate was 25%; vascularized fibular graft reconstruction was associated with fewer complications. This study found that denosumab increases the risk of local recurrence after curettage, function is better after curettage, and vascularized fibular graft is the optimal reconstruction after resection of distal radius GCTB. [ Orthopedics . 2020;43(5):284–291.]

Aims: Osteoblastoma-like osteosarcoma is a rare variant of osteosarcoma (1% of all osteosarcomas), histologically similar to osteoblastoma. In the current World Health Organisation (WHO) classification, osteoblastoma-like osteosarcoma is classified within the group of conventional (high-grade) osteosarcomas. However, several published cases have been actually regarded as low-grade malignant tumours. As strict morphological criteria to distinguish between low- and high-grade lesions are not available, we reviewed our series of osteoblastoma-like osteosarcomas in the attempt to identify clinical and morphological features predictive of aggressiveness. Methods and results: We retrieved 15 cases of osteoblastoma-like osteosarcoma from the files of the Istituto Ortopedico Rizzoli. Patients received various treatments. Five patients developed metastasis and five patients developed local recurrences (all after incomplete surgery). Eleven patients were alive without disease, while four patients died of their disease. Statistical analysis revealed a statistically significant (P=0.048) lower disease-free survival in patients with areas of conventional (high-grade) osteosarcoma. Conclusions: With the important limitation of a small cohort of patients, the presence of areas of conventional (high-grade) osteosarcoma is the only parameter to predict the aggressiveness of osteoblastoma-like osteosarcoma.

Numerous studies have suggested that microRNAs (miRNAs) are dysregulated in osteosarcoma (OS), implicating miRNAs in OS initiation and progression. Therefore, knowledge of aberrantly expressed miRNAs in OS may provide novel mechanistic insights into the tumorigenesis and tumor development of OS and facilitate therapeutic methods for patients with this aggressive bone neoplasm. In this study, data obtained from reverse transcription quantitative polymerase chain reaction (RT-qPCR) revealed that miR-935 was significantly decreased in OS tissues and cell lines. Restoration expression of miR-935 obviously restricted proliferation and invasion of OS cells. In addition, high-mobility group box 1 (HMGB1) was predicted to be a putative target of miR-935. Subsequent dual-luciferase reporter assay, RT-qPCR, and Western blot analysis confirmed that miR-935 could directly target the 3′-untranslated region of HMGB1 and negatively regulated HMGB1 expression in OS cells. Furthermore, a significant negative association was found between miR-935 and HMGB1 mRNA expression in OS tissues. Rescue experiments showed that recovery of HMGB1 expression partially rescued miR-935-induced suppression of cell proliferation and invasion in OS. These results provide the first evidence for the suppressive roles of miR-935 in OS by directly targeting HMGB1, suggesting that miR-935 may be a potential candidate for the treatment of patients with this disease.

Giant cell tumor of bone (GCTB) is an intermediate (locally aggressive) bone tumor with a recurrence rate of >30% following surgery. GCTB recurrence is ultimately due to the proliferation of neoplastic stromal (NS) cells. However, the precise mechanism underlying the regulation of NS cell proliferation remains unknown. p62 protein is a multifunctional adaptor protein that exerts a positive role in bone tumors and metabolic bone diseases. In the present study, the mRNA and protein expression levels of p62 were detected by reverse transcription-quantitative PCR and western blotting, respectively, in 8 paired fresh GCTB tumor tissues and adjacent normal cancellous bone tissues. The association between p62 expression level and patient prognosis was subsequently analyzed in 54 paraffin-embedded tumor specimens by immunohistochemistry assay. NS cells were isolated from GCTB primary cell culture, and the role of p62 was evaluated using in vitro cell proliferation, migration and invasion assays. The results revealed that p62 mRNA and protein were overexpressed in tumor tissues. High p62 expression levels were significantly associated with the recurrence of GCTB (P=0.001). The patients in the high p62 expression group had shorter 5-year recurrence-free survival rates compared with the patients in the low p62 expression group (P

Highlights • Osteochondroma is a benign cartilaginous tumour that rarely affects the scapula. • We present a rare case of osteochondroma with winging of scapula, present in his fourth decade of life. • This symptomatic scapular osteochondroma exhibited positive radiological findings. • The patient was treated surgically and recovered fully, with no recurrence. • This report increases awareness of unusual osteochondroma sites and features., Introduction Osteochondroma, a type of cartilaginous tumour, is the most common benign tumour affecting the bone. These tumours usually arise around the knee, proximal humerus, and pelvis, but very rarely occur at the scapula. Osteochondromas are usually asymptomatic and uncomplicated, but must be treated by surgical resection. Presentation of case In this report, we present a rare case of a symptomatic scapular osteochondroma associated with scapular winging in a 30-year-old man. This tumour exhibited positive radiological findings and was treated surgically, leading to a complete resolution of the patient's symptoms with no history of recurrence. Discussion This case was unique because although the patient presented in his fourth decade of life, he had not noticed any signs indicative of lesional growth during adolescence and the maturation process. Additionally, this case was symptomatic and involved an unusual site. Conclusion By reporting this rare case of a ventral-side scapular osteochondroma that presented with scapular winging, we aim to increase the awareness of the unusual manifestations of osteochondroma, particularly atypical sites, signs, and symptoms. Furthermore, we have described the surgical treatment of this case in detail to assist other surgeons who face similar cases.

Background: Dedifferentiated chondrosarcoma (DDC) accounts for a small proportion of chondrosarcomas. They demonstrate aggressive behaviour with a high rate of local recurrence and systemic progression resulting in poor long-term survival rates. Due to its relatively low incidence, previous studies have grouped different histiotypes together to achieve adequate study numbers for analysis. Methods: This retrospective study examines the clinical course and the role of chemotherapy in the subgroup of patients with DDC where osteosarcoma is the predominant dedifferentiated component. Between 2000-2010, 21 patients were identified. Results: The mean age at presentation was 64 years (range 35-80 years). 12 patients were considered unfit for chemotherapy, whilst 2 patients declined chemotherapy. 5 patients received neoadjuvant chemotherapy, with less than 90% necrosis demonstrated in all these cases. 3 patients received post-operative chemotherapy. The median survival for the entire group was 9.5 months. In the 7 patients who received chemotherapy, the median survival was 17 months, and those who had chemotherapy had a greater median time to local recurrence. Conclusion: This study demonstrates that cytotoxic chemotherapy may be offered to appropriately selected patients.

Osteochondromas are bone exostoses, with the vast majority extending from the metaphyseal region of long bones and are capped by cartilage. A review of the current literature reveals spontaneous regression of osteochondromas is a rarely documented event, with all but two of these recorded events resolving before skeletal maturity and within 6 years of identification. We present a case of trauma-induced resolution of a solitary osteochondroma after less than 3 months in a 15-month-old male, with a review of current literature. Keywords: Osteochondroma, Regression, Exostoses, Bone neoplasm, Bone

Introducción: La neoplasia ósea es una patología que altera la homeostasis del hueso dependiendo de la benignidad o malignidad del proceso. El estudio imagenológico aporta una serie de información que es fundamental para el diagnóstico y tratamiento de esta patología. El objetivo del presente reporte es establecer las características imagenológicas de los pacientes atendidos en el Servicio de Imágenes del Hospital “Juan Tanca Marengo” con tumores óseos. Métodos: Se realizó un estudio observacional, en el que se registraron todos los casos con diagnóstico de neoplasias óseas atendidos en el Hospital “Juan Tanca Marengo“, en Guayaquil entre enero del 2006 a enero del 2008. Para el análisis estadístico se emplearon medidas de tendencia central. Resultados: Se incluyeron 100 casos, la edad promedio entre los pacientes con tumores óseos primarios fue menor que entre las metástasis (25.4 vs 32.8 años). Del grupo de pacientes con lesiones primarias fueron más frecuentes los del sexo masculino (56.0 vs 37.5%). La afectación cortical fue mayor entre pacientes con tumor óseo metastásico que entre tumores primarios (62.5 vs 39.3%). En las otras variables estudiadas no se presentaron diferencias significativas. Conclusión: La mayoría de las características imagenológicas no son exclusivas del tipo de lesión ósea (primaria o metastásica). Las características de edad y género ayudan a la orientación diagnóstica de las imágenes de las lesiones tumorales óseas.

Malignant transformation of giant cell tumor of bone (GCTB) without radiotherapy exposure is exceptionally rare, occurring in less than 1% of GCTBs. The safety and efficacy of denosumab in patients with GCTB was recently reported. We herein report a case of a benign recurrent GCTB with an H3F3A mutation that underwent secondary malignant transformation during treatment with denosumab. A 29-year-old woman underwent curettage of a GCTB of the left ischium in 2005. Ten years after the first surgery, the GCTB recurred locally. We started treatment with denosumab. During the first 5 months of treatment, we observed a demarcated area of osteosclerosis in the recurrent lesion, and the patient's clinical condition improved. At 6 months, however, the patient developed pain, and a rapidly growing mass was detected by computed tomography. An incisional biopsy was performed. Histologic analysis showed a high-grade osteosarcoma. The patient developed lung metastases and died soon after beginning chemotherapy. The mechanism of sarcomatous transformation of GCTB during denosumab therapy is unclear. These findings suggest that the scientific community should be aware of the possible malignant transformation of GCTB during denosumab treatment.

The present report illustrates a very rare case report of a foreign body granuloma of the foot associated with a 2-year period of a retained spongeous rubber foreign body after a penetrating injury. Because it appeared almost 2 years after the injury, the history of trauma was minimized, the foreign body had migrated from the plantar to the dorsal side of the foot, and the radiographic appearance resembled a malignant process, it was difficult to rule out the possibility of a malignant tumor. This case shows the weakness of magnetic resonance imaging in detecting spongeous rubber in the body. Also, a bone osteoblastic reaction to the foreign body has very rarely been noted. To the best of our knowledge, just 5 cases have been previously reported. Provided suspicion exists of previous trauma with an embedded foreign body, the differential diagnosis of a bone neoplasm should consider the possibility of a foreign body reaction. Biopsy proved to be the best diagnostic tool.

Dedifferentiated chondrosarcoma is defined by the presence of a low grade malignant cartilaginous component juxtaposed to a high grade malignant non-cartilaginous sarcomatous components. Only 4 cases in which the high grade component showed epithelial differentiation have been reported in the literature; three featured a squamous and the one a glandular epithelial component. Here we describe a case of dedifferentiated chondrosarcoma exhibiting epithelial “adamantinoma-like” basaloid features. The patient underwent wide resection of the proximal tibia and post-operative chemotherapy and died 8 months after the diagnosis due to lung and bone metastases.

Introduction: The purpose of our study is to verify possible clinical and radiological findings with regard to distinguishing enchondroma from atypical cartilaginous tumour (ACT). In addition, this study determined risk factors that are associated with local recurrence of enchondroma or ACT treated with curettage. Materials and methods: We retrospectively reviewed the records of 54 patients with enchondroma and 35 patients with ACT of the long bones treated by curettage between 1986 and 2015. The minimum follow-up was 18 months. The relationship between clinical and radiological factors and the tumour type or local recurrence was assessed using Chi-square test or Fischer exact test. Results: Endosteal scalloping (p = 0.004) and soft tissue extension (p = 0.017) were shown to statistically favour ACT over enchondroma; by contrast, pain (p = 0.034) was more frequent in enchondroma compared to ACT. All patients with enchondroma had no local recurrence; in contrast, local recurrence occurred in four patients with ACT (11%). Soft tissue extension (p = 0.049) and the diagnosis of ACT (p = 0.021) were associated with an increased risk of local recurrence. We had a disease progression in three of four patients with local recurrence, and these had higher histological grade than the original tumour. Discussion: Our data show that endosteal scalloping and soft tissue extension could be helpful in the differential diagnosis between enchondroma and ACT. We suggest following only those patients with ACT after surgery to identify any possible recurrence and, in case of recurrence, treat these patients with resection for the risk of disease progression.

Donati, Davide Maria/0000-0002-9170-0576; Campanacci, Laura/0000-0002-4734-6741; Bindiganavile Srinivas, Srimanth/0000-0003-0458-7331; MANFRINI, MARCO/0000-0003-2961-0171 WOS: 000399195300009 PubMed: 27995558 Background Intercalary reconstruction of tibial sarcomas with vascularized fibula autografts and massive bone allografts is reliable with predictable long-term results. However, inadequate data exist comparing free and pedicled vascularized fibula autografts in combination with a massive bone allograft in patients undergoing intercalary tibia reconstructions. Questions/Purpose Among patients undergoing large-segment intercalary allografting, we sought to compare supplemental free vascularized fibular autografts with supplemental pedicled vascularized fibular autografts, in terms of (1) oncologic results, (2) complications associated with surgery, (3) Musculoskeletal Tumor Society (MSTS) scores, and (4) surgical time. Methods Between 1994 and 2013, we treated 320 patients, younger than 40 years, with tibial sarcomas. Thirty-five patients (11%) underwent amputations. One hundred ninety-five patients (61%) were treated with intraarticular resection of the tibia, which constituted 104 tumor endoprostheses, 63 proximal tibia allograft prosthetic composites, 21 osteoarticular allografts, and seven arthrodeses with allografts. Ninety patients (28%) underwent joint-sparing intercalary reconstruction. Forty-one (13%) of these 90 patients were treated with allografts alone, two (1%) with vascularized fibula grafts, and 47 (15%) with intercalary allografts supplemented by autografts (free fibular autografts, 22 patients, 7%; pedicled fibular autografts, 25 patients, 8%). During the study period, we used free vascularized fibular autografts in association with massive bone allograft for a resection longer than 12 cm with a very small periarticular residual segment. The choice for using a pedicled fibula harvested in the ipsilateral leg initially was for patients having only diaphyseal resections and the indication was later extended to intraepiphyseal osteotomies with a small periarticular residual segment. The goals of this study are to present the long-term results in this group of patients and compare their results based on the type of vascularized fibula harvest. There were 33 male and 14 female patients with mean age of 14 +/- 6 years. The median followup was 84 months (range, 7-231 months). No patients were lost to followup before 1 year. Four patients died and were not available for followup after 18 months. The mean tibia resection length was 15 +/- 4 cm and mean length of the harvested vascularized fibula was 18 +/- 4 cm. Results Overall 5- and 10-year oncologic survival rates in this study were 87% +/- 5% and 83% +/- 6% respectively. With the numbers available, we observed no difference in survivorship free from death from disease between the study groups (85% +/- 8% [95% CI, 174-232 months] of the free vascularized group versus 82% +/- 8% [95% CI, 148-206 months] of the pedicled fibula graft group; p = 0.741). At last followup, 40 patients had no evidence of disease and seven had died of disease. Local recurrence was observed in two patients in the supplemental free vascularized fibula group and three patients in the supplemental pedicled vascularized fibula group, whereas metastases was observed in eight patients. With the numbers available, we observed no difference in the proportion of patients experiencing surgical complications between those treated with free vascularized fibula grafts and those treated with pedicled grafts (eight of 22 [36%] versus nine of 25 [36%] respectively; p = 0.605). With the numbers available, we observed no difference in mean MSTS scores between patients treated with free vascularized fibula grafts and those treated with pedicled grafts (24 +/- 9 versus 25 +/- 8; mean difference, 0.48; 95% CI, 0.54-4.6; p = 0.858). Mean surgical time was longer in the free vascularized fibula and massive bone allograft group at 9.4 +/- 1.7 hours compared with that of the pedicled vascularized fibula and massive bone allograft group at 5.7 +/- 1.3 hours (mean difference, 3.73 hours; 95% CI, 2.8-4.6 hours; p ae 0.001). Conclusions Intercalary reconstruction of tibia sarcomas with massive bone allografts supplemented with vascularized fibula grafts provide predictable results. Complications occur as expected in a biologic reconstruction, but are salvageable, preserving the original construct. The pedicled fibula can be an alternative to a free contralateral fibula for intraepiphyseal resections. Comparative technical ease, shorter surgical time, avoidance of additional microvascular anastomosis, and avoidance of surgery on the contralateral leg are notable advantages of pedicled vascularized fibula over free fibula grafts to supplement allografts when indicated in intercalary tibia resections. Italian Ministry of HealthMinistry of Health, Italy [RF-2010-2321501] This study was supported by Italian Ministry of Health (Grant Minsal RF-2010-2321501).

Osteoid osteoma and osteoblastoma are rare benign bone-forming tumors with very similar histological features. They are nowadays considered as two distinct entities. Progression of an osteoid osteoma to osteoblastoma is considered very rare with only a few cases reported in the literature. Herein we describe a case of an osteoid osteoma of the thoracic spine in a 29-year-old woman that was initially treated conservatively and progressed to osteoblastoma 5 years following the initial diagnosis. Imaging revealed an increase in the size of the spinal lesion that was surrounded by extensive paraspinal abnormal soft tissue that raised suspicion for sarcomatous transformation. The final diagnosis was established by CT-guided biopsy of both the bone lesion and the paraspinal soft tissue, which excluded malignancy and revealed an osteoblastoma surrounded by plasma cell-rich chronic inflammation. The patient then underwent wide surgical excision of the lesion and paraspinal soft tissue component that confirmed the diagnosis. Follow-up with MRI over the next 12 months was unremarkable, with no signs of recurrence or spinal instability. This unique presentation of an osteoblastoma has not been previously described. This case also demonstrates the importance of follow-up of osteoid osteomas that are treated conservatively.

Aims The purpose of this retrospective study was to differentiate between the MRI features of normal post-operative change and those of residual or recurrent disease after intralesional treatment of an atypical cartilage tumour (ACT)/grade I chondrosarcoma. Patients and Methods We reviewed the case notes, radiology and histology of 75 patients, who had been treated for an ACT/grade I chondrosarcoma by curettage, phenolisation and bone allografting between 1994 and 2005. The first post-operative Gd-enhanced MRI scan was carried out within one year of surgery. Patients had a minimum of two scans and a mean follow-up of 72 months (13 to 169). Further surgery was undertaken in cases of suspected recurrence. Results In 14 patients (18.6%) a second procedure was undertaken after a mean period of 59 months (8 to 114). Radio frequency ablation (RFA) was used in lesions of < 10 mm and curettage, phenolisation and bone grafting for those ≥ 10 mm. Only six of these (8% of total) had a histologically-proven recurrence. No increase in tumour grade was seen at time of recurrence. Conclusion Based on this study, we have been able to classify the post-operative MRI appearances into four groups. These groups differ in follow-up, and have a different risk of recurrence of the lesion. Follow-up and treatment vary for the patients in each group. We present a flow diagram for the appropriate and safe follow-up for this specific group of patients. Cite this article: Bone Joint J 2016;98-B:1674–81.

Hodgkin lymphoma classically presents as lymphadenopathy with contiguous spread with or without associated B symptoms including night sweats, weight loss, and fever. With timely diagnosis and treatment, this is a potentially curable disease. This report describes a unique case of Hodgkin lymphoma that presented with localized back pain without overt evidence of lymph node involvement. This nonclassic presentation offers a diagnostic challenge that emphasizes the importance of keeping a broad differential in an adolescent or young adult patient with lytic bone lesions.