Bone Cancers
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Primary bone tumours are tumours that start in the bone. In contrast, secondary bone cancer is where the cancer started in another part of the body but has then spread to the bones. The most common types of primary bone tumour are osteosarcoma and Ewing's sarcoma, both of which are most frequently diagnosed in children and young adults. Other less common types of bone cancer include: Chondrosarcoma (a cancer arising in cartilage cells, usually found in adults between ages 50-75, though the less common mesenchymal-chondrosarcoma is more frequent in younger patients), Malignant Fibrous Histiocytoma of bone (MFH), Chondoma (a rare low grade malignancy occuring mostly between ages 30 -70), and other rare tumours.

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Chondrosarcoma
Ewing's Sarcoma
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Information Patients and the Public (9 links)


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Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Jeong JY, Kim SY, Jeong DC, Kim KJ
A small Askin's tumor presenting with early onset of chest pain.
World J Surg Oncol. 2015; 13:112 [PubMed] Free Access to Full Article Related Publications
Most primitive neuroectodermal tumor of the chest wall destroy the rib, chest wall muscles, diaphragm, and lung or extend into the spinal compartment, resulting in a large-sized tumor and symptoms. In contrast, we recently encountered a rare case of Askin's tumor presenting with early-onset chest pain despite the small size. After resection of the tumor and adjuvant chemotherapy, the patient remains disease-free over 3 years of follow-up.

Related: Ewing's Sarcoma


Humm JL, Sartor O, Parker C, et al.
Radium-223 in the treatment of osteoblastic metastases: a critical clinical review.
Int J Radiat Oncol Biol Phys. 2015; 91(5):898-906 [PubMed] Related Publications
The element radium (Ra) was discovered by the Curies in 1898 and within a decade was in broad scientific testing for the management of several forms of cancer. The compound was known to give rise to a series of both high-energy particulate and penetrating γ-emissions. The latter found an important role in early 20th century brachytherapy applications, but the short-range α-particles seemed much less useful. Although highly cytotoxic when released within a few cell diameters of critical cell nuclei, the dense double-strand break damage was poorly repaired, and concerns regarding treatment-related toxicities and secondary malignancies halted clinical development. Moreover, the most common isotope of Ra has an exceptionally long half-life (>1600 years for (226)Ra) that proved daunting when aiming for a systemic cancer therapy. Fortunately, other radium isotopes have more convenient half-lives while still producing cytotoxic α particles. Radium-223 dichloride has a half-life of 11.4 days, and this isotope was identified as an excellent candidate for radionuclide therapy of cancers metastatic to bone. The calcium-mimetic chemical properties of the radium allowed intravenous infusion with rapid uptake to sites of new bone formation. The highly efficient bone localization suggested a potential therapeutic role for osteoblastic bone metastases, and a series of phase 1, 2, and 3 clinical trials was undertaken to explore this possibility. This series of clinical explorations culminated in the ALSYMPCA trial, an international, placebo-controlled, phase 3 study that accrued 921 symptomatic men with bone-metastatic, castrate-resistant prostate cancer. Results of this trial demonstrated a prolongation of overall survival, and regulatory agencies around the world have now approved this product as a treatment for advanced prostate cancer.

Related: Breast Cancer Myeloma Myeloma - Molecular Biology Osteosarcoma


Imtiaz S, Kazmi A
Patterns of care and outcomes of adult osteosarcoma in a tertiary care cancer centre in Pakistan.
J Pak Med Assoc. 2014; 64(10):1166-70 [PubMed] Related Publications
OBJECTIVE: To present our experience of treatment outcomes in adult osteosarcoma patients.
METHODS: The retrospective study was conducted at the Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, Pakistan, and comprised data related to 74 adult patients with osteosarcoma from 1995 to 2009. The treatment plan consisted of surgery preceded by neo-adjuvant chemotherapy followed by adjuvant chemotherapy. SPSS 16 was used for statistical analysis.
RESULTS: Of the 74 patients in the study, 58 (78%) were in the 18-29 age group with an overall male-to-female ratio of 3:1. The commonest site of disease was femur, 30 (43%). Of the 66 (89%) patients undergoing definitive surgery, 59 (89.4%) had amputation. The remaining 7 (10.6%) limb salvage operations were in the neo-adjuvant chemotherapy group. Good histopathological response rates in high-dose methotrexate containing regimens and other regimens were similar with an overall good response rate of 18/51 (35%). The commonest site of relapse was lung.Twelve out of 27 (44%) patients with lung-only metastases underwent successful metastatectomy. For patients with localised disease at presentation 3-year event-free survival was 30%, and 3-year overall survival was 71%. For patients with metastases at presentation 3-year overall survival was 45%. Median overall survival for patients receiving high-dose methotrexate and other regimens was 1.7 years vs 2.9 years.
CONCLUSION: Adult osteosarcoma treated with cisplatin/doxorubicin based chemotherapy and surgery had good outcomes. The role of high-dose methotrexate in adult osteosarcoma remains uncertain.

Related: Osteosarcoma


Tang C, Zhao Y, Huang S, et al.
Influence of Artemisia annua extract derivatives on proliferation, apoptosis and metastasis of osteosarcoma cells.
Pak J Pharm Sci. 2015; 28(2 Suppl):773-9 [PubMed] Related Publications
Regarding the Artemisia annua extract derivatives called dihydroarteminin (DHA) as the object, we studied about its influence to the proliferation, apoptosis and metastasis of human osteosarcoma cells. First, we cultured in vitro the osteosarcoma cell strain and divided them into groups, then detected the cell proliferation, apoptosis and cell metastasis, etc by multiple measurement technique. Finally, we observed the influence of DHA to human osteosarcoma cells. Osteosarcoma cells were all sensitive to DHA, and the appropriate concentration range was 10~40μM. DHA could effectively restrain its protein expression, and there was a significant difference between experimental group and control group. These finding suggest that, the Artemisia annua extract derivatives (DHA) has a biological effect of observably restraining the proliferation and metastasis of human osteosarcoma cells and promoting the tumour cell apoptosis.

Related: Apoptosis MMP9: matrix metallopeptidase 9 VEGFA


Meyliker RG, Strauss RA
Periosteal osteosarcoma of the mandible: a case report.
J Oral Maxillofac Surg. 2015; 73(4):787.e1-4 [PubMed] Related Publications
This report describes a rare case of a periosteal osteosarcoma of the mandible in a 50-year-old African-American woman who showed no underlying bony changes at panoramic radiography or computed tomography. This report describes the diagnostic workup used to obtain the definitive diagnosis and the surgical treatment and recommended method for subsequent tumor surveillance. Emphasis is placed on distinguishing periosteal osteosarcomas as a separate entity from conventional intraosseous osteosarcomas in the head and neck region.

Related: Osteosarcoma


Zhu J, Gu J, Ma J, et al.
Histone deacetylase inhibitors repress chondrosarcoma cell proliferation.
J BUON. 2015 Jan-Feb; 20(1):269-74 [PubMed] Related Publications
PURPOSE: Due to the high resistance to conventional therapy, there is still no convincingly effective treatment for chondrosarcoma. As a promising new treatment strategy, histone deacetylase inhibitors (HDACIs) have been reported to induce cell arrest, apoptosis and differentiation in some kinds of malignancies, but how HDACi exert their effects on chondrosarcoma is not well understood yet.
METHODS: We investigated the effects of HDACIs trichostatin A (TSA) and sodium valproate (VPA) on chondrosarcoma cells in vitro and in vivo. The cell proliferation and cell cycle were examined in two chondrosarcoma cell lines, SW1353 and JJ012, by MTS and flow cytometry assays, respectively. The in vivo effects of HDACIs were investigated by assessing the chondrosarcoma growth in a mouse xenograft model.
RESULTS: Our results showed that TSA and VPA significantly repressed the proliferation of chondrosarcoma cells in a concentration-dependent manner. Flow cytometry indicated that TSA arrested the cell cycle in G2/M phase and VPA arrested the cell cycle in G1 phase. The tumor growth was markedly suppressed in mice treated with TSA and VPA.
CONCLUSIONS: HDACIs significantly repress the proliferation of chondrosarcoma cells in vitro and in vivo. Our findings imply that HDACIs may provide a novel therapeutic target for the treatment of chondrosarcoma.

Related: Chondrosarcoma Signal Transduction


Wodajo FM
Top five lesions that do not need referral to orthopedic oncology.
Orthop Clin North Am. 2015; 46(2):303-14 [PubMed] Related Publications
Patients with potential bone and soft tissue tumors can be challenging for orthopedic surgeons. Lesions that appear benign can still create anxiety for the clinician and patient. However, attention to a few key imaging and clinical findings is enough to correctly diagnose five of the most common bone and soft tissue lesions: lipoma, enchondroma, osteochondroma, nonossifying fibroma, and Paget disease. Accurate identification of these lesions should be within the scope of most orthopedic surgeons and, because most of these patients will not need surgical treatment, referral to orthopedic oncology will not typically be required.


Righi A, Gambarotti M, Longo S, et al.
Small cell osteosarcoma: clinicopathologic, immunohistochemical, and molecular analysis of 36 cases.
Am J Surg Pathol. 2015; 39(5):691-9 [PubMed] Related Publications
Small round cell osteosarcoma is a very rare type of osteosarcoma, histologically mimicking other small round cell malignancies of bone, most notably Ewing sarcoma. To distinguish small cell osteosarcoma from other primary small cell malignancies of bone, we evaluated the immunohistochemical (IHC) expression of CD99 and SATB2, a marker of osteoblastic differentiation. Second, we analyzed EWSR1 and FUS gene aberrations using fluorescence in situ hybridization and/or reverse transcription-polymerase chain reaction (RT-PCR) techniques to assess whether small cell osteosarcoma and Ewing sarcoma share the same genetic alteration analysis. Thirty-six cases of primitive small cell osteosarcoma of bone were included in this study. All the cases of small cell osteosarcoma showed strong nuclear expression of SATB2 associated with negativity for CD99 antibody or weak, cytoplasmic staining in few neoplastic cells. Reverse transcription-polymerase chain reaction was negative for EWS-FLI1 type 1-2, EWS-ERG type 1, and CIC-DUX4 in the 10 available cases of small cell osteosarcoma analyzed. Fluorescence in situ hybridization analysis was feasible with a readable signal in 13 cases of small cell osteosarcoma, and none of these cases showed any EWSR1 and FUS gene rearrangements. In conclusion, it appears extremely useful to combine IHC analysis of SATB2 and CD99 with molecular analysis of Ewing sarcoma-associated genetic aberrations, to differentiate small cell osteosarcoma from other small round cell malignancies of bone. The strong IHC expression of SATB2 associated with CD99 immunonegativity and the absence of EWSR1 and FUS gene rearrangements in small cell osteosarcoma argues against the existence of a morphologic/genetic continuum with Ewing sarcoma.

Related: FISH Osteosarcoma


Ferrari S, Meazza C, Palmerini E, et al.
Nonmetastatic osteosarcoma of the extremity. Neoadjuvant chemotherapy with methotrexate, cisplatin, doxorubicin and ifosfamide. An Italian Sarcoma Group study (ISG/OS-Oss).
Tumori. 2014 Nov-Dec; 100(6):612-9 [PubMed] Related Publications
BACKGROUND: Based on the results of the ISG/OS-1 study, the MAP regimen (methotrexate [MTX], doxorubicin [ADM] and cisplatin [CDP] with the addition of ifosfamide [IFO] in poor-responder patients) was investigated in patients with nonmetastatic osteosarcoma of the extremity (ISG/OS-Oss study).
PATIENTS AND METHODS: Compared with the ISG/OS-1 study (cumulative doses: ADM 420 mg/m(2), MTX 120 g/m(2), CDP 600 mg/m(2), IFO 30 g/m(2)), the ISG/OS-Oss study reduced the number of MTX cycles from 10 to 5 (cumulative MTX dose: 60 g/m(2)) in order to diminish treatment duration and toxicity.
RESULTS: From January 2007 to June 2011, 171 patients (median age 16 years, 60% males) were registered. The limb salvage rate was 94% and the good pathologic response rate 51% (these figures were 92% and 48%, respectively, in the ISG/OS-1 study). At a median follow-up of 39 months (range, 4-80), the 5-year overall survival rate was 80% (95% CI, 73%-87%) and the event-free survival was 50% (95% CI, 39%-59%). For comparison, the 5-year overall and event-free survival rates in ISG/OS-1 were 73% (95% CI, 65%-81%) and 64% (95% CI, 56%-73%), respectively.
CONCLUSIONS: This study confirms that in nonmetastatic osteosarcoma of the extremity, conservative surgery in more than 90% and a good pathologic response rate of 50% can be expected with primary chemotherapy based on the MAP regimen. The response and resection rates in the ISG/OS-Oss study are in the same range as those of the previous study, whereas the event-free survival is lower than that previously achieved. Since the only difference between the two studies was the cumulative dose of postoperatively given MTX, our data support the importance of the cumulative dose of MTX in the MAP regimen.

Related: Cisplatin Doxorubicin Ifosfamide Methotrexate Osteosarcoma


Righi A, Gambarotti M, Benini S, et al.
MDM2 and CDK4 expression in periosteal osteosarcoma.
Hum Pathol. 2015; 46(4):549-53 [PubMed] Related Publications
Periosteal osteosarcoma is defined by the World Health Organization as an intermediate-grade, malignant, cartilaginous, and bone-forming neoplasm arising on the surface of bone. Unlike other subtypes of osteosarcoma, no data have been published about mouse double minute 2 (MDM2) and cyclin-dependent kinase 4 (CDK4) expression. For this reason, we evaluated the molecular and immunohistochemical features of MDM2 and CDK4 in 27 cases relative to 20 patients with a diagnosis of periosteal osteosarcoma, surgically treated at the Rizzoli Institute between 1981 and 2014. When possible, these results were compared with the MDM2 amplification status as determined by fluorescence in situ hybridization (FISH). All but 1 case (26/27, 96.3%) were negative for MDM2 protein using immunohistochemistry both in primary and in recurrent periosteal osteosarcoma, whereas gene amplification of MDM2 was not detected in any tumor analyzed (10 cases). The positive immunohistochemical case shows a weak/moderate focal nuclear expression of MDM2 antibody in the prevalent cartilaginous component and in the spindle cells of peripheral fibroblastic areas associated with osteoid production in a primary periosteal osteosarcoma. CDK4 immunohistochemical expression was negative in all 27 cases. This retrospective analysis has demonstrated that MDM2 and CDK4 are very rarely expressed in primary and recurrent periosteal osteosarcomas and therefore do not appear to be molecules central to the control of cancer development, growth, and progression in periosteal osteosarcoma. Therefore, when compared with low-grade central and parosteal osteosarcomas, MDM2 and CDK4 markers cannot be used diagnostically to differentiate this subtype of osteosarcoma.

Related: CDK4 Osteosarcoma MDM2 gene


Mühlhofer HM, Lenze U, Lenze F, et al.
Inter- and intra-observer variability in biopsy of bone and soft tissue sarcomas.
Anticancer Res. 2015; 35(2):961-6 [PubMed] Related Publications
BACKGROUND/AIM: The aim of this study was to analyze the inter- and intra-observer variability regarding biopsy technique in bone and soft tissue sarcoma based on magnetic resonance imaging (MRI).
PATIENTS AND METHODS: Thirty-seven MRI scans of bone and soft tissue sarcomas treated in our clinic were randomly selected. Six observers with three different expertise levels were assigned to analyze the scans for suspected entity and preferred biopsy technique at 2 time points with a delay of 8 weeks.
RESULTS: The differentiation between bone and soft tissue sarcomas in MRI seemed closely related to the observer's level of experience. Regarding biopsy technique, no inter-observer accordance could be identified in either group.
CONCLUSION: We observed an association of inter- and intra-observer agreement regarding suspected tumor entity and the observer's level of experience. The decision for either biopsy technique showed a low inter-observer but high intra-observer variability. These findings suggest that the decision for incisional or core needle biopsy is, even in the expert group, frequently based on personal predilection.

Related: Osteosarcoma Soft Tissue Sarcomas


Li S, Sun W, Wang H, et al.
Research progress on the multidrug resistance mechanisms of osteosarcoma chemotherapy and reversal.
Tumour Biol. 2015; 36(3):1329-38 [PubMed] Related Publications
Osteosarcoma (OS) is the most common and aggressive primary malignant type of bone cancer in children and adolescents. Chemotherapy is one of the most important treatments for OS. Although cancer therapy has improved over the past few decades, survival outcomes for OS patients remain unsatisfactory. One of the primary reasons for the failure of current treatments is that patients with stage IV cancer often develop resistance to anticancer agents. This article will review multidrug resistance (MDR) mechanisms of OS and strategies for overcoming resistance.

Related: Osteosarcoma


Liu F, Zhang Q
Questions about XY Wen et al. entitled "Matrix metalloproteinase 2 expression and survival of patients with osteosarcoma: a meta-analysis".
Tumour Biol. 2015; 36(2):557-8 [PubMed] Related Publications
XY Wen et al. published an article entitled "Matrix metalloproteinase 2 expression and survival of patients with osteosarcoma: a meta-analysis" in Tumor Biology last year. The investigators demonstrated that high MMP2 expression was associated with poor prognosis in osteosarcoma. After reading these articles, we have some questions about the data extraction and processing in the meta-analysis.

Related: MMP2 Osteosarcoma


McDonald MW, Linton OR, Calley CS
Dose-volume relationships associated with temporal lobe radiation necrosis after skull base proton beam therapy.
Int J Radiat Oncol Biol Phys. 2015; 91(2):261-7 [PubMed] Related Publications
PURPOSE: We evaluated patient and treatment parameters correlated with development of temporal lobe radiation necrosis.
METHODS AND MATERIALS: This was a retrospective analysis of a cohort of 66 patients treated for skull base chordoma, chondrosarcoma, adenoid cystic carcinoma, or sinonasal malignancies between 2005 and 2012, who had at least 6 months of clinical and radiographic follow-up. The median radiation dose was 75.6 Gy (relative biological effectiveness [RBE]). Analyzed factors included gender, age, hypertension, diabetes, smoking status, use of chemotherapy, and the absolute dose:volume data for both the right and left temporal lobes, considered separately. A generalized estimating equation (GEE) regression analysis evaluated potential predictors of radiation necrosis, and the median effective concentration (EC50) model estimated dose-volume parameters associated with radiation necrosis.
RESULTS: Median follow-up time was 31 months (range 6-96 months) and was 34 months in patients who were alive. The Kaplan-Meier estimate of overall survival at 3 years was 84.9%. The 3-year estimate of any grade temporal lobe radiation necrosis was 12.4%, and for grade 2 or higher radiation necrosis was 5.7%. On multivariate GEE, only dose-volume relationships were associated with the risk of radiation necrosis. In the EC50 model, all dose levels from 10 to 70 Gy (RBE) were highly correlated with radiation necrosis, with a 15% 3-year risk of any-grade temporal lobe radiation necrosis when the absolute volume of a temporal lobe receiving 60 Gy (RBE) (aV60) exceeded 5.5 cm(3), or aV70 > 1.7 cm(3).
CONCLUSIONS: Dose-volume parameters are highly correlated with the risk of developing temporal lobe radiation necrosis. In this study the risk of radiation necrosis increased sharply when the temporal lobe aV60 exceeded 5.5 cm(3) or aV70 > 1.7 cm(3). Treatment planning goals should include constraints on the volume of temporal lobes receiving higher dose. The EC50 model provides suggested dose-volume temporal lobe constraints for conventionally fractionated high-dose skull base radiation therapy.


Coathup MJ, Sanghrajka A, Aston WJ, et al.
Hydroxyapatite-coated collars reduce radiolucent line progression in cemented distal femoral bone tumor implants.
Clin Orthop Relat Res. 2015; 473(4):1505-14 [PubMed] Article available free on PMC after 01/04/2016 Related Publications
BACKGROUND: Aseptic loosening of massive bone tumor implants is a major cause of prosthesis failure. Evidence suggests that an osteointegrated hydroxyapatite (HA)-coated collar would reduce the incidence of aseptic loosening around the cemented intramedullary stem in distal femoral bone tumor prostheses. Because these implants often are used in young patients with a tumor, such treatment might extend the longevity of tumor implants. Questions/purposes We asked whether (1) HA-coated collars were more likely to osteointegrate; (2) HA collars were associated with fewer progressive radiolucent lines around the stem-cement interface; and (3) HA-coated collars were associated with less bone loss at the bone-shoulder implant junction?
METHODS: Twenty-two patients were pair-matched to one of two groups--either (1) implants with a HA-coated ingrowth collar (HA Collar Group); or (2) implants without an ingrowth collar (Noncollar Group). Age, sex, and length of followup were similar in both groups. HA-coated collars were developed and used at our institution from 1992 to address the high failure rate attributable to aseptic loosening in patients with massive bone tumor implants. Before this, smooth titanium shafts were used routinely adjacent to bone at the transection site. The minimum followup was 2 years (mean, 7 years; range, 2-12 years). Radiographs obtained throughout the followup period were analyzed and osteointegration at the shaft of the implant quantified. Radiolucent line progression around the cemented stem was semi-quantitatively assessed and cortical bone loss at the bone-shoulder implant junction was measured during the followup period.
RESULTS: Comparison of the most recent radiographs showed nine of 11 patients had osteointegrated HA collars, whereas only one patient in the Noncollar Group had osteointegration (p > 0.001). The radiolucent line score quantified around the cemented stem was lower in the HA Collar Group when compared with the Noncollar Group (p = 0.001). Results showed an increase in cortical bone loss at the bone-shoulder implant junction in the Noncollar Group when compared with the HA Collar Group (p < 0.001).
CONCLUSIONS: Osteointegration at the implant collar resulted in fewer radiolucent lines adjacent to the intramedullary cemented stem and decreased cortical bone loss immediately adjacent to the transection site. These results suggest that the HA collar may help reduce the risk of aseptic loosening in patients with this type of implant, but longer followup and a larger prospective comparison series are necessary to prove this more definitively.

Related: Chondrosarcoma Osteosarcoma


Magnan H, Goodbody CM, Riedel E, et al.
Ifosfamide dose-intensification for patients with metastatic Ewing sarcoma.
Pediatr Blood Cancer. 2015; 62(4):594-7 [PubMed] Related Publications
BACKGROUND: Outcomes for patients with metastatic Ewing sarcoma (ES) remain poor. We investigated whether the intensification of ifosfamide improved survival for patients with metastatic ES.
PROCEDURE: We conducted a retrospective chart review of 30 patients with metastatic ES treated with the MSKCC "EFT regimen." The regimen included an intensification of ifosfamide dosing from 1,800 mg/m(2) /day × 5 days per cycle to 2,800 mg/m(2) /day × 5 days per cycle.
RESULTS: Twenty six of the 30 patients completed planned chemotherapy. Two patients experienced disease progression during therapy. There were no toxic deaths. One patient developed secondary leukemia. The 4-year event free survival (EFS) was 27% and the overall survival (OS) was 39%.
CONCLUSIONS: Intensification of ifosfamide was tolerated and did not increase toxicity in patients with metastatic ES. The intensification did not improve outcomes for these patients with metastatic disease.

Related: Ifosfamide Ewing's Sarcoma


Young PS, Bell SW, Mahendra A
The evolving role of computer-assisted navigation in musculoskeletal oncology.
Bone Joint J. 2015; 97-B(2):258-64 [PubMed] Related Publications
We report our experience of using a computer navigation system to aid resection of malignant musculoskeletal tumours of the pelvis and limbs and, where appropriate, their subsequent reconstruction. We also highlight circumstances in which navigation should be used with caution. We resected a musculoskeletal tumour from 18 patients (15 male, three female, mean age of 30 years (13 to 75) using commercially available computer navigation software (Orthomap 3D) and assessed its impact on the accuracy of our surgery. Of nine pelvic tumours, three had a biological reconstruction with extracorporeal irradiation, four underwent endoprosthetic replacement (EPR) and two required no bony reconstruction. There were eight tumours of the bones of the limbs. Four diaphyseal tumours underwent biological reconstruction. Two patients with a sarcoma of the proximal femur and two with a sarcoma of the proximal humerus underwent extra-articular resection and, where appropriate, EPR. One soft-tissue sarcoma of the adductor compartment which involved the femur was resected and reconstructed using an EPR. Computer navigation was used to aid reconstruction in eight patients. Histological examination of the resected specimens revealed tumour-free margins in all patients. Post-operative radiographs and CT showed that the resection and reconstruction had been carried out as planned in all patients where navigation was used. In two patients, computer navigation had to be abandoned and the operation was completed under CT and radiological control. The use of computer navigation in musculoskeletal oncology allows accurate identification of the local anatomy and can define the extent of the tumour and proposed resection margins. Furthermore, it helps in reconstruction of limb length, rotation and overall alignment after resection of an appendicular tumour.

Related: Chondrosarcoma Osteosarcoma Soft Tissue Sarcomas Ewing's Sarcoma


Qu N, Yao W, Cui X, Zhang H
Malignant transformation in monostotic fibrous dysplasia: clinical features, imaging features, outcomes in 10 patients, and review.
Medicine (Baltimore). 2015; 94(3):e369 [PubMed] Related Publications
Malignant transformation in fibrous dysplasia (FD) is uncommon. The purpose of this study was to investigate clinical and imaging features, and outcomes of malignant transformation in monostotic FD.Data for 10 pathologically confirmed malignant transformations in monostotic FD from January 2005 to December 2013 were retrospectively reviewed. Patient data were recorded, and radiographs (n = 10), computed tomography (CT) (n = 5), magnetic resonance (MR) (n = 4), and bone scintigrams (n = 10) were evaluated for lesion location, margin, cortical destruction, marrow involvement, periosteal reaction, and soft tissue mass by 2 musculoskeletal radiologists with agreement by consensus. Clinical features, management, and prognosis were also analyzed for each of the 10 cases.There were 8 male and 2 female patients (mean age 46.5 ± 15.9 years). The affected sites were the femur (n = 4), humerus (n = 2), tibia (n = 3), and ilium (n = 1). Five cases had received previous surgery and 5 cases had no history of surgery. No patients had been given prior irradiation treatment. For the 5 cases with surgery, radiographs and CT showed purely osteolytic lesions with poor margination in the curettage area (n = 5), cortical destruction (n = 5), obvious soft tissue mass (n = 1), and mineralization (n = 2). For the 5 cases without surgery, radiographs and CT identified poorly marginated, osteolytic lesions within or near the area with "ground-glass" opacity (n = 4), cortical erosion (n = 4), and mineralization (n = 2). Magnetic resonance imaging (MRI) also identified lesions with heterogeneous signal intensity and pronounced enhancement. Bone scintigraphy revealed eccentric increased uptake of radionuclide in monostotic lesion (n = 10). Pathology reports revealed osteosarcoma (n = 7), fibrosarcoma (n = 2), and malignant fibrous histiocytoma (MFH) (n = 1). At the end of the study, 1 patient died from tumors, 1 patient was alive with lung metastasis, 1 patient experienced recurrence, and 7 patients were alive without recurrence.Patients with FD and a history of surgery should be followed up, for the osteolytic lesions in the operative areas strongly indicate the malignant transformation. The radiographic feature of FD-related malignancies is poorly marginated, mineralized, and osteolytic lesions with cortical destruction. Further investigations are needed to explore the pathogenesis of malignancies in FD and to establish optimal therapeutic strategies.

Related: Osteosarcoma


Tamamyan G, Dominkus M, Lang S, et al.
Multiple relapses in high-grade osteosarcoma: when to stop aggressive therapy?
Pediatr Blood Cancer. 2015; 62(3):529-30 [PubMed] Related Publications
The prognosis after relapse of high-grade osteosarcoma is poor and complete resection of all tumors is essential for survival. A 6-year old was diagnosed with high-grade osteosarcoma and treated according to the COSS-96 protocol. Within 5 years from initial diagnosis, five osteosarcoma relapses occurred and every time it was possible to achieve complete surgical remission. Additional treatments included chemotherapy and dendritic cell-based cancer immune therapy. Since the end of therapy of the 5th relapse, he is alive for 11½ years. Our experience further supports that aggressive surgery can help to achieve long-term survival even in patients with multiple osteosarcoma relapses.

Related: Osteosarcoma


Barbato S, Sgarbi G, Gorini G, et al.
The inhibitor protein (IF1) of the F1F0-ATPase modulates human osteosarcoma cell bioenergetics.
J Biol Chem. 2015; 290(10):6338-48 [PubMed] Article available free on PMC after 06/03/2016 Related Publications
The bioenergetics of IF1 transiently silenced cancer cells has been extensively investigated, but the role of IF1 (the natural inhibitor protein of F1F0-ATPase) in cancer cell metabolism is still uncertain. To shed light on this issue, we established a method to prepare stably IF1-silenced human osteosarcoma clones and explored the bioenergetics of IF1 null cancer cells. We showed that IF1-silenced cells proliferate normally, consume glucose, and release lactate as controls do, and contain a normal steady-state ATP level. However, IF1-silenced cells displayed an enhanced steady-state mitochondrial membrane potential and consistently showed a reduced ADP-stimulated respiration rate. In the parental cells (i.e. control cells containing IF1) the inhibitor protein was found to be associated with the dimeric form of the ATP synthase complex, therefore we propose that the interaction of IF1 with the complex either directly, by increasing the catalytic activity of the enzyme, or indirectly, by improving the structure of mitochondrial cristae, can increase the oxidative phosphorylation rate in osteosarcoma cells grown under normoxic conditions.

Related: Osteosarcoma


Tang X, Guo W, Yang R, et al.
Synthetic mesh improves shoulder function after intraarticular resection and prosthetic replacement of proximal humerus.
Clin Orthop Relat Res. 2015; 473(4):1464-71 [PubMed] Article available free on PMC after 01/04/2016 Related Publications
BACKGROUND: Shoulder function often is limited after tumor resection and endoprosthetic replacement of the proximal humerus. This is partly attributable to the inability to reliably reattach rotator cuff tendons to the prosthesis and achieve adequate shoulder capsule repair with a metallic prosthesis. An option to attain these goals is to use synthetic mesh for the reconstruction, although the value of this method has not been well documented in the literature.
QUESTIONS/PURPOSES: We asked whether patients who had shoulder reconstruction using synthetic mesh had (1) better shoulder function; (2) improved ROM compared with shoulder reconstructions without mesh; and (3) more stable joints compared with those in patients with similar resections who had reconstructions without synthetic mesh.
METHODS: During a 5-year period, we performed 41 intraarticular resections with endoprosthetic reconstructions for malignancies in the proximal humerus meeting specified criteria to generate similarity in the study groups. Twelve patients (29%) were lost to followup before 24 months, leaving 29 patients available for review at a mean of 45 months (range, 24-70 months). This retrospective study compared 14 patients with soft tissue reconstruction that included synthetic mesh with 15 patients with soft tissue reconstruction without the use of synthetic mesh. The choice was made during consultation between the patient and surgeon, after reviewing the perceived advantages and disadvantages of each approach. A tumor band (ligament advanced reinforcement system) was used as synthetic mesh and wrapped around the prosthesis of the proximal humerus for soft tissue reconstruction in the reconstruction-with-mesh group. Study endpoints included the Musculoskeletal Tumor Society (MSTS) function scores, American Shoulder and Elbow Surgeons (ASES) score, shoulder ROM, and proximal migration of the humeral prosthesis.
RESULTS: The mean MSTS score for patients without synthetic mesh reconstruction was 20 ± 3 points (66%), whereas for patients with synthetic mesh reconstruction, the mean score was 24 ± 2 points (79%; p = 0.001). Patients with synthetic mesh reconstruction had a higher mean total ASES score (85 ± 1.1 points versus 72 ± 1.7 points; p = 0.025), and better function for activities of daily living. They also had better ROM on mean active forward flexion (p = 0.020), abduction (p < 0.001), and external rotation (p < 0.001) than patients without synthetic mesh reconstruction. Proximal migration of the prosthesis was observed in five of 15 of patients in the group without synthetic mesh reconstruction and in none of those treated with synthetic mesh (p = 0.042).
CONCLUSIONS: Patients with intraarticular resection and endoprosthetic replacement of the proximal humerus with reconstruction that included synthetic mesh had better shoulder function and ROM, and more stable joints than patients who had reconstruction without synthetic mesh. This result supports prior observations by others and it remains to be shown whether use of the ligament advanced reconstruction system is superior to other types of mesh or other types of reconstructions. Further investigation is needed but our results indicate that using mesh should be considered for patients with tumor resection and endoprosthetic replacement of the proximal humerus.

Related: Chondrosarcoma Osteosarcoma


Jonckheere J, Shahabpour M, Willekens I, et al.
Rapid malignant transformation of primary synovial chondromatosis into chondrosarcoma.
JBR-BTR. 2014 Sep-Oct; 97(5):303-7 [PubMed] Related Publications
Chondrosarcoma of the synovium is rare. It may arise de novo from the synovium or pre-existing synovial chondro- matosis may undergo malignant transformation into chondrosarcoma. Diagnosing a malignant transformation of the synovium remains a big challenge. It is based on the correlation of clinical findings, imaging and histology, as illustrated in this case report.

Related: Chondrosarcoma


Moussazadeh N, Kulwin C, Anand VK, et al.
Endoscopic endonasal resection of skull base chondrosarcomas: technique and early results.
J Neurosurg. 2015; 122(4):735-42 [PubMed] Related Publications
OBJECT: The authors of this study sought to report the technique and early clinical outcomes of a purely endonasal endoscopic approach for resection of petroclival chondrosarcomas.
METHODS: Between 2010 and 2014, 8 patients (4 men and 4 women) underwent endonasal endoscopic operations to resect petroclival chondrosarcomas at 2 institutions. The patients' mean age was 44.8 years (range 30-64 years). One of the patients had previously undergone radiation therapy and another a staged craniotomy. Using volumetric software, an independent neuroradiologist assessed the extent of the resections on MRI scans taken immediately after surgery and at the 3-month follow-up. Immediate complications and control of symptoms were also recorded. In addition, the authors reviewed the current literature on surgical treatment of chondrosarcoma.
RESULTS: The mean preoperative tumor diameter and volume were 3.4 cm and 9.8 cm(3), respectively. Six patients presented with cranial neuropathies. Endonasal endoscopic surgery achieved > 95% resection in 5 of the 8 patients and < 95% resection in the remaining 3 patients. One of the 6 neuropathies resolved, and the remaining 5 partially improved. One instance of postoperative CSF leakage required a reoperation for repair; no other complications associated with these operations were observed. All of the patients underwent adjuvant radiotherapy.
CONCLUSIONS: According to the authors' experience, the endoscopic endonasal route is a safe and effective approach for the resection of appropriately selected petroclival chondrosarcomas.

Related: Chondrosarcoma


Fang D, Yang H, Lin J, et al.
17β-estradiol regulates cell proliferation, colony formation, migration, invasion and promotes apoptosis by upregulating miR-9 and thus degrades MALAT-1 in osteosarcoma cell MG-63 in an estrogen receptor-independent manner.
Biochem Biophys Res Commun. 2015; 457(4):500-6 [PubMed] Related Publications
In bone, different concentration of estrogen leads to various of physiological processes in osteoblast, such as the proliferation, migration, and apoptosis in an estrogen receptor-dependent manner. But little was known about the estrogen effects on osteosarcoma (OS). In this study, OS cell MG-63 was treated with low (1 nM) or high (100 nM) dose of 17β-Estradiol (E2) with the presence or absence of estrogen receptor α (ERα), for evaluating the E2 effects on proliferation, migration, invasion, colony formation and apoptosis. Consistent with a previous study, high dose of E2 treatment dramatically downregulated expressing level of long non-coding RNA metastasis associated lung adenocarcinoma transcript 1 (MALAT-1). The observation of upregulation of miR-9 after a high dose of E2 treatment indicated the cause of MALAT-1 reduction. Downregulation of MALAT-1 promoted the combination of SFPQ/PTBP2 complex. It was also observed that the proliferation, migration, invasion, colony formation and apoptosis of OS cells were remarkably affected by high dose of E2 treatment, but not by low dose, in an ERα independent manner. Furthermore, the abolishment of the effects on these physiological processes caused by ectopic expression of miR-9 ASOs suggested the necessity of miR-9 in MALAT-1 regulation. Here we found that the high dose of E2 treatment upregulated miR-9 thus posttranscriptionally regulated MALAT-1 RNA level in OS cells, and then the downregulation of MALAT-1 inhibited cell proliferation, migration, invasion and epithelial-mesenchymal transition (EMT) processes in the E2-dose dependent and ER-independent ways.

Related: Apoptosis MicroRNAs Osteosarcoma MALAT1


Velivela K, Rajesh A, Uppin MS, Purohit AK
Primary intracranial peripheral PNET"--a case report and review.
Neurol India. 2014 Nov-Dec; 62(6):669-73 [PubMed] Related Publications
The site of origin of primitive neuroectodermal tumors (PNETs) is quite varied and has significant influence on the prognosis. We report a case of intracranial peripheral PNET/Ewing's sarcoma arising from the superior tentorial surface in a 13-year-old girl. Gross total excision of the tumor was done. We have discussed the distinction between central nervous system PNET (CNS PNET) and Intracranial Peripheral PNET (pPNET/ES) as their treatment and prognosis varies radically. A review of literature shows that prognosis is better in intracranial pPNET/ES.

Related: Ewing's Sarcoma


Zheng S, Qiao G, Min D, et al.
Heterogeneous expression and biological function of ubiquitin carboxy-terminal hydrolase-L1 in osteosarcoma.
Cancer Lett. 2015; 359(1):36-46 [PubMed] Related Publications
Ubiquitin carboxyl terminal hydrolase 1 (UCHL1), a member of the UCH class of DUBs, has been reported as either an oncogene or a tumor suppressor. However, the molecular mechanism underlying the biological function of UCHL1 in osteosarcoma is still unclear. This study was aimed at elucidating the roles of UCHL1 in regulating the biological behavior of osteosarcoma cells. In this study, we found that UCHL1 was elevated in osteosarcoma compared with normal bone tissue. Moreover, UCHL1 expression level was correlated with tumor maximum diameter, high rate of lung metastases and short survival time. Then, we found that knockdown of UCHL1 in osteosarcoma cell MG63 inhibited cell proliferation and significantly increased cell population in the G1 phase. Several cyclins promoting G1/S phase transition were reduced after UCHL1 knockdown, including cell cycle regulator cyclin D1, cyclin E1 and CDK6. Moreover, inhibition of UCHL1 in MG63 cells dramatically induced cell apoptosis. We also found that down-regulation of UCHL1 in MG63 significantly inhibited cell invasion. Then, we found that there was a positive correlation between UCHL1 expression level and the Akt and ERK phosphorylation status. Finally, in vivo data showed that knockdown of UCHL1 inhibited osteosarcoma growth in nude mice. These results indicate that UCHL1 could work as an oncogene and may serve as a promising therapeutic strategy for osteosarcoma.

Related: Apoptosis CDK6 AKT1 Signal Transduction BCL1 Gene (CCND1) CCNE1


Kang S, Han I, Hong SH, et al.
The MRI appearances of cancellous allograft bone chips after the excision of bone tumours.
Bone Joint J. 2015; 97-B(1):121-8 [PubMed] Related Publications
Cancellous allograft bone chips are commonly used in the reconstruction of defects in bone after removal of benign tumours. We investigated the MRI features of grafted bone chips and their change over time, and compared them with those with recurrent tumour. We retrospectively reviewed 66 post-operative MRIs from 34 patients who had undergone curettage and grafting with cancellous bone chips to fill the defect after excision of a tumour. All grafts showed consistent features at least six months after grafting: homogeneous intermediate or low signal intensities with or without scattered hyperintense foci (speckled hyperintensities) on T1 images; high signal intensities with scattered hypointense foci (speckled hypointensities) on T2 images, and peripheral rim enhancement with or without central heterogeneous enhancements on enhanced images. Incorporation of the graft occurred from the periphery to the centre, and was completed within three years. Recurrent lesions consistently showed the same signal intensities as those of pre-operative MRIs of the primary lesions. There were four misdiagnoses, three of which were chondroid tumours. We identified typical MRI features and clarified the incorporation process of grafted cancellous allograft bone chips. The most important characteristics of recurrent tumours were that they showed the same signal intensities as the primary tumours. It might sometimes be difficult to differentiate grafted cancellous allograft bone chips from a recurrent chondroid tumour.

Related: Chondrosarcoma


Reddy KI, Wafa H, Gaston CL, et al.
Does amputation offer any survival benefit over limb salvage in osteosarcoma patients with poor chemonecrosis and close margins?
Bone Joint J. 2015; 97-B(1):115-20 [PubMed] Related Publications
A poor response to chemotherapy (≤ 90% necrosis) for osteosarcomas leads to poorer survival and an increased risk of local recurrence, particularly if there is a close margin of excision. We evaluated whether amputation confers any survival benefit over limb salvage surgery (LSS) with narrow margins in patients who respond poorly to chemotherapy. We only analysed patients with an osteosarcoma of the limb, a poor response to chemotherapy and close margins on LSS (marginal/intralesional) or primary amputation: 360 patients (36 LSS (intralesional margins), 197 LSS (marginal margins) and 127 amputations) were included. Local recurrence developed in 13 (36%) following LSS with intralesional margins, and 39 (20%) following LSS with marginal margins. There was no local recurrence in patients who underwent amputation. The five-year survival for all patients was 41% (95% confidence interval (CI) 35 to 46), but for those treated by LSS with marginal margins was 46.2% (95% CI 38 to 53), 36.3% (95% CI 27 to 45) for those treated by amputation, and 28% (95 CI 14 to 44) for those treated by LSS with intralesional margins. Patients who had LSS and then developed local recurrence as a first event had the same survival as those who had primary amputation without local recurrence. Prophylactic adjuvant radiotherapy was used in 40 patients but had no discernible effect in preventing local recurrence. Although amputation offered better local control, it conferred no clear survival benefit over LSS with marginal margins in these patients with a poor overall prognosis.

Related: Osteosarcoma


Jamshidi K, Shiradi MR
Unusual form and location for a tumor: multiosseous Ewing sarcoma in the foot.
Am J Orthop (Belle Mead NJ). 2015; 44(1):E32-5 [PubMed] Related Publications
Ewing sarcoma, as the prototype of a small round blue cell tumor of bone, typically affects adolescents and young adults. The most commonly involved sites include the diaphyses of long bones, ribs, and flat bones, such as the pelvis and scapula. We report a case of multifocal Ewing sarcoma involving multiple bones in the foot. Given the multifocal nature of the disease confined to the foot, the initial impression was that of osteomyelitis. We describe the histologic, radiologic, and diagnostic features of the tumor and outline treatment and prognosis. To our knowledge, this is the first report of multifocal Ewing sarcoma involving multiple bones in the foot.

Related: Ewing's Sarcoma


Bhat KP, Bétous R, Cortez D
High-affinity DNA-binding domains of replication protein A (RPA) direct SMARCAL1-dependent replication fork remodeling.
J Biol Chem. 2015; 290(7):4110-7 [PubMed] Article available free on PMC after 13/02/2016 Related Publications
SMARCAL1 catalyzes replication fork remodeling to maintain genome stability. It is recruited to replication forks via an interaction with replication protein A (RPA), the major ssDNA-binding protein in eukaryotic cells. In addition to directing its localization, RPA also activates SMARCAL1 on some fork substrates but inhibits it on others, thereby conferring substrate specificity to SMARCAL1 fork-remodeling reactions. We investigated the mechanism by which RPA regulates SMARCAL1. Our results indicate that although an interaction between SMARCAL1 and RPA is essential for SMARCAL1 activation, the location of the interacting surface on RPA is not. Counterintuitively, high-affinity DNA binding of RPA DNA-binding domain (DBD) A and DBD-B near the fork junction makes it easier for SMARCAL1 to remodel the fork, which requires removing RPA. We also found that RPA DBD-C and DBD-D are not required for SMARCAL1 regulation. Thus, the orientation of the high-affinity RPA DBDs at forks dictates SMARCAL1 substrate specificity.

Related: Osteosarcoma


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