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
Osteosarcoma
Primary Lymphoma of Bone
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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).

Shirazian S, Agha-Hosseini F
Oral osteosarcoma: a case report and analysis of previously reported cases.
N Y State Dent J. 2014; 80(2):50-4 [PubMed] Related Publications
Osteosarcoma is the most common malignancy of mesenchymal cells after hematopoietic neoplasms. Most originate within bones, but the occurrence of this malignancy in the jaw bones is rare. There is controversy about the characteristics of this tumor in the literature. The aim of this paper was to collect the previous reported data and provide a statistical analysis of them. Additionally, we have reported a case of mandibular osteosarcoma.

Related: Osteosarcoma


Xie L, X D T, Yang RL, Guo W
Interscapulothoracic resection of tumours of shoulder with a note on reconstruction.
Bone Joint J. 2014; 96-B(5):684-90 [PubMed] Related Publications
We retrospectively reviewed the outcomes of 33 consecutive patients who had undergone an extra-articular, total or partial scapulectomy for a malignant tumour of the shoulder girdle between 1 July 2001 and 30 September 2013. Of these, 26 had tumours which originated in the scapula or the adjacent soft tissue and underwent a classic Tikhoff-Linberg procedure, while seven with tumours arising from the proximal humerus were treated with a modified Tikhoff-Linberg operation. We used a Ligament Advanced Reinforcement System for soft-tissue reconstruction in nine patients, but not in the other 24. The mean Musculoskeletal Tumor Society score (MSTS) was 17.6 (95% confidence interval (CI) 15.9 to 19.4); 17.6 (95% CI 15.5 to 19.6) after the classic Tikhoff-Linberg procedure and 18.1 (95% CI 13.8 to 22.3) after the modified Tikhoff-Linberg procedure. Patients who had undergone a LARS soft-tissue reconstruction had a mean score of 18.6 (95% (CI) 13.9 to 22.4) compared with 17.2 (95% CI 15.5 to 19.0) for those who did not. The Tikhoff-Linberg procedure is a useful method for wide resection of a malignant tumour of the shoulder girdle which helps to preserve hand and elbow function. The method of soft-tissue reconstruction has no effect on functional outcome.

Related: Chondrosarcoma Soft Tissue Sarcomas


Gaston CL, Nakamura T, Reddy K, et al.
Is limb salvage surgery safe for bone sarcomas identified after a previous surgical procedure?
Bone Joint J. 2014; 96-B(5):665-72 [PubMed] Related Publications
Bone sarcomas are rare cancers and orthopaedic surgeons come across them infrequently, sometimes unexpectedly during surgical procedures. We investigated the outcomes of patients who underwent a surgical procedure where sarcomas were found unexpectedly and were subsequently referred to our unit for treatment. We identified 95 patients (44 intra-lesional excisions, 35 fracture fixations, 16 joint replacements) with mean age of 48 years (11 to 83); 60% were males (n = 57). Local recurrence arose in 40% who underwent limb salvage surgery versus 12% who had an amputation. Despite achieving local control, overall survival was worse for patients treated with amputation rather than limb salvage (54% vs 75% five-year survival). Factors that negatively influenced survival were invasive primary surgery (fracture fixation, joint replacement), a delay of greater than two months until referral to our oncology service, and high-grade tumours. Survival in these circumstances depends mostly on factors that are determined prior to definitive treatment by a tertiary orthopaedic oncology unit. Limb salvage in this group of patients is associated with a higher rate of inadequate marginal surgery and, consequently, higher local recurrence rates than amputation, but should still be attempted whenever possible, as local control is not the primary determinant of survival.

Related: Osteosarcoma


Aoki M, Nishio J, Iwasaki H, et al.
Osteosarcoma of the patella mimicking giant cell tumor: imaging features with histopathological correlation.
Anticancer Res. 2014; 34(5):2541-5 [PubMed] Related Publications
Patellar tumors represent an uncommon etiology of anterior knee pain and their diagnosis is often delayed. We present an unusual case of conventional osteosarcoma arising in the patella of a 47-year-old man. The patient presented with a 1-year history of increasing anterior knee pain and swelling. Plain radiographs revealed a multi-locular lytic lesion in the inferolateral side of the patella. Computed tomography scans demonstrated an intraosseous lytic lesion with cortical thinning/breakthrough anteriorly. On magnetic resonance imaging, the lesion exhibited low signal intensity on T1-weighted images and heterogeneous high signal intensity on T2-weighted images. Fluid-fluid levels were also observed on T2-weighted images. Contrast-enhanced fat-suppressed T1-weighted images demonstrated strong enhancement of the lesion. These imaging features were suggestive of a benign condition; however, the diagnosis of osteosarcoma was confirmed by histopathology. After neoadjuvant chemotherapy, a wide resection with a free anterolateral thigh flap was performed. The patient subsequently underwent adjuvant chemotherapy and had no evidence of local recurrence or distant metastasis six months after surgery. Our case highlights the difficulty in the diagnosis of patellar osteosarcoma and the importance of performing a biopsy before definitive treatment.

Related: Osteosarcoma


Ruivo C, Hopper MA
Spinal chondrosarcoma arising from a solitary lumbar osteochondroma.
JBR-BTR. 2014 Jan-Feb; 97(1):21-4 [PubMed] Related Publications
Chondrosarcoma is a primary malignant neoplasm of cartilage-forming cells that rarely involves the axial skeleton, typically affecting skeletally mature patients. It may arise as a primary bone tumour or as a secondary lesion from a pre-existing benign cartilaginous neoplasm such as an osteochondroma or enchondroma. We report the case of a 68-year-old female who presented with a mildly painful paraspinal mass lesion as a result of malignant degeneration of a previously unknown solitary lumbar osteochondroma into a large chondrosarcoma. The characteristic imaging findings on cross-sectional imaging techniques are reviewed and illustrated, along with an outline of relevant clinical and therapeutic aspects.


Chung SW, Han I, Oh JH, et al.
Prognostic effect of erroneous surgical procedures in patients with osteosarcoma: evaluation using propensity score matching.
J Bone Joint Surg Am. 2014; 96(8):e60 [PubMed] Related Publications
BACKGROUND: Little is known concerning erroneous surgical procedures of malignant bone tumors, and the prognostic effect of erroneous surgical procedures in osteosarcoma has not been determined.
METHODS: We retrospectively reviewed 240 patients with initially non-metastatic high-grade osteosarcoma of the pelvis and extremities and, of these, identified twenty-six who had undergone previous less appropriate surgical procedures due to misdiagnosis followed by adequate treatment at our institution. We evaluated the clinicopathologic characteristics of these twenty-six patients compared with the remaining 214 patients treated with regular protocol. Subsequently, thirty-eight patients (nineteen in the matched case group and nineteen in the matched control group) were matched for multiple different variables using propensity score matching, and the oncologic results in terms of event-free survival and overall survival were analyzed.
RESULTS: The patients undergoing erroneous surgical procedures were typically older, with small, non-osteoblastic-type tumors that were in an unusual location, showed an osteolytic pattern on radiographs, had a tendency toward marginal or intralesional excision with positive histologic margin, and had not been treated with neoadjuvant chemotherapy (all p < 0.05). After adjustment of confounding variables by propensity score matching, there was no significant difference between matched groups with regard to event-free survival (p = 0.46) and overall survival (p = 0.99).
CONCLUSIONS: Distinct differences existed in the clinicopathologic characteristics of the patients who underwent erroneous surgical procedures due to misdiagnosis. We failed to detect a prognostic relevance of the presence of previous erroneous procedures followed by adequate treatment.

Related: Osteosarcoma


Stacchiotti S, Pantaleo MA, Astolfi A, et al.
Activity of sunitinib in extraskeletal myxoid chondrosarcoma.
Eur J Cancer. 2014; 50(9):1657-64 [PubMed] Related Publications
BACKGROUND: Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma, marked by NR4A3 rearrangement. Herein we report on the activity of sunitinib in a series of 10 patients, strengthening what initially observed in two cases.
PATIENTS AND METHODS: From July 2011, 10 patients with progressive metastatic translocated EMC have been consecutively treated with sunitinib 37.5mg/day, on a named-use basis. In an attempt to interpret the activity of sunitinib in EMC, genotype/phenotype correlations were carried out by fluorescence in situ hybridization (FISH) analyses. Moreover, transcriptome, immunohistochemical and biochemical analyses of a limited set of samples were performed focusing on some putative targets of sunitinib.
RESULTS: Eight of 10 patients are still on therapy. Six patients had a Response Evaluation Criteria in Solid Tumours (RECIST) partial response (PR), two were stable, two progressed. Positron emission tomography (PET) was consistent in 6/6 evaluable cases. One patient underwent surgery after sunitinib, with evidence of a pathologic response. At a median follow-up of 8.5 months (range 2-28), no secondary resistance was detected. Median progression free survival (PFS) has not been reached. Interestingly, all responsive cases turned out to express the typical EWSR1-NR4A3 fusion, while refractory cases carried the alternative TAF15-NR4A3 fusion. Among putative sunitinib targets, only RET was expressed and activated in analysed samples.
CONCLUSIONS: This report confirms the therapeutic activity of sunitinib in EMC. Genotype/phenotype analyses support a correlation between response and EWSR1-NR4A3 fusion. Involvement of RET deserves further investigation.

Related: Angiogenesis Inhibitors EWSR1 gene TAF15 gene Sunitinib (Sutent) RET


Valenti MT, Zanatta M, Donatelli L, et al.
Ascorbic acid induces either differentiation or apoptosis in MG-63 osteosarcoma lineage.
Anticancer Res. 2014; 34(4):1617-27 [PubMed] Related Publications
BACKGROUND/AIM: Osteosarcoma originates from mesenchymal stem cells with impaired bone differentiation. In the present study we investigated the effect of ascorbic acid (AsA) on osteogenic differentiation and apoptosis of the MG-63 osteosarcoma cell line.
MATERIALS AND METHODS: We evaluated the expression of runt-related transcription factor-2 (RUNX2) and secreted phosphoprotein 1 (SPP1) genes by real-time Polymerase Chain Reaction (PCR) and of endogenous bone morphogenetic protein-2 (BMP2) and osteocalcin proteins by immunohistochemistry. We analyzed osteoblast maturation by phosphatase alkaline synthesis and calcium deposition, and apoptosis by (TUNEL) test and Annexin staining.
RESULTS: Our results showed that RUNX2 and SPP1 gene expression was increased in cells treated with low concentrations of AsA with respect to untreated cells. At higher concentrations, AsA induced apoptosis of osteosarcoma cells, possibly with the involvement of p21.
CONCLUSION: Our findings support the ability of AsA to induce both differentiation, by affecting the target involved in early and late phases of osteogenic maturation, and apoptosis in poorly-differentiated osteosarcoma cells.

Related: Apoptosis Osteosarcoma SPP1


Igarashi K, Yamamoto N, Shirai T, et al.
The long-term outcome following the use of frozen autograft treated with liquid nitrogen in the management of bone and soft-tissue sarcomas.
Bone Joint J. 2014; 96-B(4):555-61 [PubMed] Related Publications
In 1999, we developed a technique for biological reconstruction after excision of a bone tumour, which involved using autografts of the bone containing the tumour treated with liquid nitrogen. We have previously reported the use of this technique in 28 patients at a mean follow up of 27 months (10 to 54). In this study, we included 72 patients who underwent reconstruction using this technique. A total of 33 patients died and three were lost to follow-up, at a mean of 23 months (2 to 56) post-operatively, leaving 36 patients available for a assessment at a mean of 101 months 16 to 163) post-operatively. The methods of reconstruction included an osteo-articular graft in 16, an intercalary in 13 and, a composite graft with prosthesis in seven. Post-operative function was excellent in 26 patients (72.2%), good in seven (19.4%), and fair in three (8.3%) according to the functional evaluation system of Enneking. No recurrent tumour occurred within the grafts. The autografts survived in 29 patients (80.6%), and the rates of survival at five and ten years were 86.1% and 80.6 %, respectively. Seven of 16 osteo-articular grafts (44%) failed because of fracture or infection, but all the composite and intercalary grafts survived. The long-term outcomes of frozen autografting, particularly using composite and intercalary grafts, are satisfactory and thus represent a good method of treatment for patients with a sarcoma of bone or soft tissue.

Related: Osteosarcoma Soft Tissue Sarcomas Childhood Soft Tissue Sarcomas Soft Tissue Sarcoma


Lin P, Mobasher ME, Alawi F
Acute dyskerin depletion triggers cellular senescence and renders osteosarcoma cells resistant to genotoxic stress-induced apoptosis.
Biochem Biophys Res Commun. 2014; 446(4):1268-75 [PubMed] Related Publications
Dyskerin is a conserved, nucleolar RNA-binding protein implicated in an increasing array of fundamental cellular processes. Germline mutation in the dyskerin gene (DKC1) is the cause of X-linked dyskeratosis congenita (DC). Conversely, wild-type dyskerin is overexpressed in sporadic cancers, and high-levels may be associated with poor prognosis. It was previously reported that acute loss of dyskerin function via siRNA-mediated depletion slowed the proliferation of transformed cell lines. However, the mechanisms remained unclear. Using human U2OS osteosarcoma cells, we show that siRNA-mediated dyskerin depletion induced cellular senescence as evidenced by proliferative arrest, senescence-associated heterochromatinization and a senescence-associated molecular profile. Senescence can render cells resistant to apoptosis. Conversely, chromatin relaxation can reverse the repressive effects of senescence-associated heterochromatinization on apoptosis. To this end, genotoxic stress-induced apoptosis was suppressed in dyskerin-depleted cells. In contrast, agents that induce chromatin relaxation, including histone deacetylase inhibitors and the DNA intercalator chloroquine, sensitized dyskerin-depleted cells to apoptosis. Dyskerin is a core component of the telomerase complex and plays an important role in telomere homeostasis. Defective telomere maintenance resulting in premature senescence is thought to primarily underlie the pathogenesis of X-linked DC. Since U2OS cells are telomerase-negative, this leads us to conclude that loss of dyskerin function can also induce cellular senescence via mechanisms independent of telomere shortening.

Related: Apoptosis Osteosarcoma


Choi EY, Gardner JM, Lucas DR, et al.
Ewing sarcoma.
Semin Diagn Pathol. 2014; 31(1):39-47 [PubMed] Related Publications
Classification of small round cell tumors of bone is often challenging due to overlapping clinicopathologic features. The purpose of this article is to review the clinical, radiological, histologic, and molecular features of Ewing sarcoma and to provide a discussion of the differential diagnosis of small round cell tumors of bone.

Related: Ewing's Sarcoma


Green JT, Mills AM
Osteogenic tumors of bone.
Semin Diagn Pathol. 2014; 31(1):21-9 [PubMed] Related Publications
In this paper we provide an overview of benign and malignant osteogenic bone tumors. We describe the diagnostic features, radiographic findings, and pertinent ancillary studies needed to diagnose these bone-forming lesions. We begin with osteoid osteoma and osteoblastoma, which are histologically bland and eminently benign with rare possible exceptions. On the other end of the behavioral spectrum is osteosarcoma, which encompasses many subtypes ranging from high-grade osteogenic osteosarcoma to less overtly osteogenic lesions such as telangiectatic and small cell osteosarcoma. While classic osteogenic osteosarcoma can be easily recognized by its high grade morphology and formation of extracellular lace-like osteoid, its variants may pose diagnostic dilemmas as their differential diagnoses can include benign, fibrous, and vascular lesions, among others. Recognition of these variants is essential to avoid diagnostic pitfalls. In equivocal cases, some forms of osteosarcoma have shown molecular alterations that may prove diagnostically useful.

Related: Osteosarcoma


Qasem SA, DeYoung BR
Cartilage-forming tumors.
Semin Diagn Pathol. 2014; 31(1):10-20 [PubMed] Related Publications
Cartilage-forming tumors as a group are the most common primary bone tumors; this is largely due to the common occurrence of asymptomatic benign lesions such as osteochondroma and enchondroma. The common feature of these tumors is the presence of chondrocytic cells and the formation of cartilaginous tumor matrix. Some of these tumors are true neoplasms while others are hamartomas or developmental abnormalities. The morphologic heterogeneity of these tumors may be explained by a common multipotent mesenchymal cell differentiating along the lines of fetal-adult cartilage maturation. Recently mutations in IDH1 and IDH2 have been detected in a variety of benign and malignant cartilaginous tumors.(1-4.)

Related: Chondrosarcoma


Kenney LB, Duffey-Lind E, Ebb D, et al.
Impaired testicular function after an ifosfamide-containing regimen for pediatric osteosarcoma: a case series and review of the literature.
J Pediatr Hematol Oncol. 2014; 36(3):237-40 [PubMed] Related Publications
To assess testicular function after standard dose ifosfamide, we evaluated 6 young adult osteosarcoma survivors (median age at diagnosis, 16.5 y; median follow-up, 4 y) treated with ifosfamide (median dose, 45.5 g/m) as part of a chemotherapy regimen (adriamycin/cisplatin/methotrexate/ifosfamide/± muramyl-tripeptide-phosphatidyl-ethanolamine). Four of 6 survivors (67%) had abnormal semen analysis (2 oligospermic, 2 azoospermic). Of those, 1/4 had reduced testicular volume, and 2/3 elevated FSH levels. All reported adequate sexual function, 6/6 had normal testosterone levels, but 4/6 had elevated LH levels. Ifosfamide exposure in the context of this regimen was associated with a high likelihood of impaired spermatogenesis and Leydig cell insufficiency.

Related: Cisplatin Doxorubicin Ifosfamide Methotrexate Osteosarcoma


Gillmann C, Jäkel O, Schlampp I, Karger CP
Temporal lobe reactions after carbon ion radiation therapy: comparison of relative biological effectiveness-weighted tolerance doses predicted by local effect models I and IV.
Int J Radiat Oncol Biol Phys. 2014; 88(5):1136-41 [PubMed] Related Publications
PURPOSE: To compare the relative biological effectiveness (RBE)-weighted tolerance doses for temporal lobe reactions after carbon ion radiation therapy using 2 different versions of the local effect model (LEM I vs LEM IV) for the same patient collective under identical conditions.
METHODS AND MATERIALS: In a previous study, 59 patients were investigated, of whom 10 experienced temporal lobe reactions (TLR) after carbon ion radiation therapy for low-grade skull-base chordoma and chondrosarcoma at Helmholtzzentrum für Schwerionenforschung (GSI) in Darmstadt, Germany in 2002 and 2003. TLR were detected as visible contrast enhancements on T1-weighted MRI images within a median follow-up time of 2.5 years. Although the derived RBE-weighted temporal lobe doses were based on the clinically applied LEM I, we have now recalculated the RBE-weighted dose distributions using LEM IV and derived dose-response curves with Dmax,V-1 cm³ (the RBE-weighted maximum dose in the remaining temporal lobe volume, excluding the volume of 1 cm³ with the highest dose) as an independent dosimetric variable. The resulting RBE-weighted tolerance doses were compared with those of the previous study to assess the clinical impact of LEM IV relative to LEM I.
RESULTS: The dose-response curve of LEM IV is shifted toward higher values compared to that of LEM I. The RBE-weighted tolerance dose for a 5% complication probability (TD5) increases from 68.8 ± 3.3 to 78.3 ± 4.3 Gy (RBE) for LEM IV as compared to LEM I.
CONCLUSIONS: LEM IV predicts a clinically significant increase of the RBE-weighted tolerance doses for the temporal lobe as compared to the currently applied LEM I. The limited available photon data do not allow a final conclusion as to whether RBE predictions of LEM I or LEM IV better fit better clinical experience in photon therapy. The decision about a future clinical application of LEM IV therefore requires additional analysis of temporal lobe reactions in a comparable photon-treated collective using the same dosimetric variable as in the present study.

Related: Chondrosarcoma


Ulaner GA, Magnan H, Healey JH, et al.
Is methylene diphosphonate bone scan necessary for initial staging of Ewing sarcoma if 18F-FDG PET/CT is performed?
AJR Am J Roentgenol. 2014; 202(4):859-67 [PubMed] Related Publications
OBJECTIVE: The purpose of this study was to determine whether methylene diphosphonate (MDP) bone scans are necessary during initial staging in patients with Ewing sarcoma (ES) in whom (18)F-FDG PET/CT is performed.
MATERIALS AND METHODS: A retrospective review was performed of patients who underwent FDG PET/CT and MDP bone scan before treatment of newly diagnosed ES from January 2004 to November 2012. Studies were reviewed to document suspected primary and metastatic malignancy. Pathology and imaging follow-up were used to determine the presence or absence of disease at suspected sites.
RESULTS: Sixty patients were identified in whom FDG PET/CT and MDP bone scans were performed before treatment of newly diagnosed ES. Forty-four primary malignancies had a lytic CT appearance, three were sclerotic, and 13 involved only soft tissue. In 11 of 12 patients with osseous metastases, these were detected on PET/CT, with the one false-negative occurring in a sclerotic primary tumor; in nine of 12 patients with osseous metastases, these were detected on MDP bone scan, with the three false-negatives occurring in patients with lytic primary tumors. Only one of 13 patients with a soft-tissue primary malignancy had bone metastases on both bone scan and PET/CT. PET/CT also showed that eight patients had lung metastases and three patients had lymph node metastases, which were not evident on MDP bone scan.
CONCLUSION: When ES is lytic, MDP bone scan does not add to staging performed by FDG PET/CT; thus, MDP bone scanning may be omitted. However, when ES is sclerotic, MDP bone scan may detect osseous metastases not detected by FDG PET/CT.

Related: Bisphosphonates Ewing's Sarcoma


Sikri V, Sobti S
Askin tumour: a rare thoracopulmonary tumour in adults.
Indian J Chest Dis Allied Sci. 2013 Oct-Dec; 55(4):233-5 [PubMed] Related Publications
Askin tumour, a primitive neuroectodermal tumour of the thoracopulmonary region, is a rare tumour presenting in childhood. Its presentation in adults is rare. We report a case of an Askin tumour in an adult patient who presented to us with worsening breathlessness and vague chest pain. Investigations including immunohistochemistry confirmed the diagnosis of Askin tumour.

Related: Lung Cancer Ewing's Sarcoma


Babkina IV, Kuznetsov IN, Soloviev YN, et al.
Angiogenic factors in blood serum of bone tumor patients.
Klin Lab Diagn. 2013; (10):63-5, 31-3 [PubMed] Related Publications
It is proven that angiogenesis and cartilage resorption processes involving vascular endothelial growth factor (VEGF) and its receptors play an important role in bone tissue regeneration and bone tumor development. A comparative analysis of VEGE VEGF-R1, VEGF-R2 and angiogenin content in blood serum of healthy persons, and patients with primary malignant, benign and borderline bone tumors was performed. 366 bone tumor patients aged 14-76 years were examined; 223 (60,9%) male and 143 (39,1%) -female. Control group included 48 practically healthy subjects aged 15-69 years; 25 (52,1%) male and 23 (47,9%) female. Serum VEGE VEGF-R1, VEGF-R2 and angiogenin concentrations measured using standard ELISA kits produced by "R&D Systems Inc. ", USA. It was shown that long-term treatment outcome (3-years overall survival) of typical osteosarcoma patients depended on tumor malignancy grade (determined by the criterion G). Baseline serum VEGF, its soluble receptors and angiogenin concentrations had no predictive value.

Related: Osteosarcoma VEGFA


Boulytcheva IV, Kushlinskii NE, Soloviev YN, Aliev MD
Expression of molecular markers in low-grade chondrosarcomas and cartilaginous tumors with uncertain differentiation.
Klin Lab Diagn. 2013; (10):61-3, 25-30 [PubMed] Related Publications
Among the wide array of human neoplasms, primary tumors of bone are relatively uncommon and sundry group of solid tumors traditionally categorized according to their presumed mesenchymal differentiation. A locally aggressive or malignant group of cartilaginous matrix-producing neoplasms with diverse morphological features and clinical behavior require additional ancillary studies for prompt diagnosis and appropriate surgical treatment. They are histologically, behaviorally and genetically diverse, their pathogenesis is poorly understood Moreover treatment options are limited with surgical resection continuing to provide the only possibility of cure in many cases. However, there has been tremendous progress in the last decade in understanding the molecular pathogenesis of sarcoma, which may ultimately lead to more effective therapy and prognostification for these rare malignancies (1. Atypical cartilaginous tumor/grade1chondrosarcomas behave as locally aggressive lesions, and only metastasize in exceptional cases. Only a small percentage of the IDH1mutations can be identified using the specific IDHIRI32H antibody Histologic grade is the most important predictor of local recurrence and metastasis in chondrosarcoma , commonly patents die from locally recurrent tumor ofpelvis or scull, that is difficult to manage surgically Association between ratio of matrix metalloproteinaise- 1 and tissue inhibitor ofmetalloproteinase- 1, expression of metalloproteinase-1, -2 and-9, Col-IV, Cox-2, Bcl-2, Bax in context with histological and clinical data could play a significant role in determining prognosis in patients with borderline cartilaginous tumors. The mandatory application of multidisciplinary care in management of atypical cartilaginous tumor/grade 1 chondrosarcomas with integration of histologic, molecular, radiographic and clinical data is difficult to overestimate.

Related: Chondrosarcoma PTGS2 IDH1 gene


Yu D, Fu S, Cao Z, et al.
Unraveling the novel anti-osteosarcoma function of coptisine and its mechanisms.
Toxicol Lett. 2014; 226(3):328-36 [PubMed] Related Publications
Uncontrolled cell proliferation and robust angiogenesis play critical roles in osteosarcoma growth and metastasis. In this study we explored novel agents derived from traditional Chinese medicinal herbs that potently inhibit osteosarcoma growth and metastasis. Coptisine, an active component of the herb Coptidis rhizoma, markedly inhibited aggressive osteosarcoma cell proliferation. Coptisine induced cell cycle arrest at the G0/G1 phase through downregulation of CDK4 and cyclin D1 expression and effectively suppressed tumor growth in a xenografted mouse model. Coptisine significantly impeded osteosarcoma cell migration, invasion, and capillary-like network formation by decreasing the expression of VE-cadherin and integrin ß3, and diminishing STAT3 phosphorylation. Coptisine significantly elevated blood erythrocyte and hemoglobin levels while still remaining within the normal range. It also moderately increased white blood cell and platelet counts. These data suggest that coptisine exerts a strong anti-osteosarcoma effect with very low toxicity and is a potential anti-osteosarcoma drug candidate.

Related: Angiogenesis and Cancer Osteosarcoma


Sbaihat A, Bacciu A, Pasanisi E, Sanna M
Skull base chondrosarcomas: surgical treatment and results.
Ann Otol Rhinol Laryngol. 2013; 122(12):763-70 [PubMed] Related Publications
OBJECTIVES: We describe our experience in the management of patients with skull base chondrosarcoma, an uncommon neoplasm of the skull base.
METHODS: Thirteen cases of surgically treated skull base chondrosarcomas were identified. The patients' follow-ups ranged from 7 to 86 months (mean, 47 months).
RESULTS: The most common tumor locations were the jugular foramen (5 cases), the petrous apex (3 cases), and the petroclival region (3 cases). An infratemporal fossa type A approach was performed in 2 cases, and 2 patients underwent an infratemporal fossa type B approach. Two patients underwent a transotic approach, 1 patient underwent a petro-occipital transsigmoid approach, and a petro-occipital transsigmoid approach combined with a transotic approach was chosen in 1 case. One patient underwent an infratemporal fossa type C approach combined with a transotic approach, and 2 patients underwent an infratemporal fossa type B approach combined with a transotic approach. One patient underwent an infratemporal fossa type B approach combined with a transzygomatic approach, and the last patient underwent a transmastoid approach. Gross total tumor removal was achieved in all patients. Postoperative radiotherapy was performed in 7 cases. The most common complications were lower cranial nerve deficits. Two patients experienced recurrences, 36 months and 6 years after surgical removal.
CONCLUSIONS: We believe that the primary treatment for chondrosarcomas of the skull base is gross total surgical resection. We usually do not recommend radiotherapy as the primary treatment for patients with skull base chondrosarcomas; however, radiotherapy may be considered as an alternative primary treatment in selected cases in which there are serious medical contraindications to surgery, as well as in elderly patients. We reserve postoperative radiotherapy for patients with histologically aggressive tumors (grade II or III), as well as for cases of subtotal resection or recurrent tumors.

Related: Chondrosarcoma


Wang D, Bi Z
Bufalin inhibited the growth of human osteosarcoma MG-63 cells via down-regulation of Bcl-2/Bax and triggering of the mitochondrial pathway.
Tumour Biol. 2014; 35(5):4885-90 [PubMed] Related Publications
Cinobufacini (Huachansu), a Chinese medicine prepared from the skin of Bufo bufo gargarizans Cantor (Bufonidae), has potent anti-tumor activity in vitro and in vivo. However, the molecular mechanism of cell apoptosis induced by Bufalin remains elusive. Here, we investigated the apoptosis in Bufalin-treated human osteosarcoma MG-63 cells. The results showed that Bufalin could inhibit cell proliferation and induce apoptosis in a dose- and time-dependent manner. Further investigation revealed that a disruption of mitochondrial transmembrane potential (MMP) and an up-regulation of reactive oxygen species (ROS) in Bufalin-treated cells. By western blot analysis, we found that the up-regulation of Apaf-1, cleaved PARP, cleaved caspase-3, cleaved caspase-9, and Bax/Bcl-2, varies with different concentration of Bufalin. These protein interactions may play a pivotal role in the regulation of apoptosis. Taken together, these results overall indicate that Bufalin could be used as an effective anti-tumor agent in therapy of osteosarcoma targets the mitochondrial-dependent signaling pathway.

Related: Apoptosis Mitochondrial Mutations in Cancer Osteosarcoma


Yu W, Zhang Z, Min D, et al.
Mediator of RNA polymerase II transcription subunit 19 promotes osteosarcoma growth and metastasis and associates with prognosis.
Eur J Cancer. 2014; 50(6):1125-36 [PubMed] Related Publications
Osteosarcoma (OS) is the most common primary malignant tumour of bone. Nearly 30-40% of OS patients have a poor prognosis despite multimodal treatments. Because the carcinogenesis of OS remains unclear, the identification of new oncogenes that control the tumourigenesis and progression of OS is crucial for developing new therapies. Here, we found that the expression of Mediator of RNA polymerase II transcription subunit 19 (Med19) was increased in OS samples from patients compared to normal bone tissues. Cyclin D1 and cyclin B1 are upregulated in Med19 positive OS tissues. Importantly, among 97 OS patients of Enneking stage IIB or IIIB, Med19 expression was correlated with metastasis (P<0.05) and poor prognosis (P<0.01). Med19 knockdown significantly induced growth inhibition, reduced colony-forming ability and suppressed migration in the OS cell lines Saos-2 and U2OS, along with the downregulated expression of cyclin D1 and cyclin B1. Med19 knockdown also induced apoptosis in Saos-2 cells via induction of caspase-3 and poly ADP-ribose polymerase (PARP). In addition, Med19 knockdown significantly suppressed tumour growth in an OS xenograft nude mouse model via suppression of cyclin D1 and cyclin B1. Simultaneously, Med19 downregulation decreased the expression of Ki67 and proliferating cell nuclear antigen (PCNA) in tumour samples from OS xenograft nude mice. Med19 depletion remarkably reduced tumour metastasis in a model of OS metastatic spreading. Taken together, our data suggest that Med19 acts as an oncogene in OS via a possible cyclin D1/cyclin B1 modulation pathway.

Related: Apoptosis Osteosarcoma


Bus MP, Dijkstra PD, van de Sande MA, et al.
Intercalary allograft reconstructions following resection of primary bone tumors: a nationwide multicenter study.
J Bone Joint Surg Am. 2014; 96(4):e26 [PubMed] Related Publications
BACKGROUND: Favorable reports on the use of massive allografts to reconstruct intercalary defects underline their place in limb-salvage surgery. However, little is known about optimal indications as reports on failure and complication rates in larger populations remain scarce. We evaluated the incidence of and risk factors for failure and complications, time to full weight-bearing, and optimal fixation methods for intercalary allografts after tumor resection.
METHODS: A retrospective study was performed in all four centers of orthopaedic oncology in the Netherlands. All consecutive patients reconstructed with intercalary (whole-circumference) allografts after tumor resection in the long bones during 1989 to 2009 were evaluated. The minimum follow-up was twenty-four months. Eighty-seven patients with a median age of seventeen years (range, 1.5 to 77.5 years) matched inclusion criteria. The most common diagnoses were osteosarcoma, Ewing sarcoma, adamantinoma, and chondrosarcoma. The median follow-up period was eighty-four months (range, twenty-five to 262 months). Ninety percent of tumors were localized in the femur or the tibia.
RESULTS: Fifteen percent of our patients experienced a graft-related failure. The major complications were nonunion (40%), fracture (29%), and infection (14%). Complications occurred in 76% of patients and reoperations were necessary in 70% of patients. The median time to the latest complication was thirty-two months (range, zero to 200 months). The median time to full weight-bearing was nine months (range, one to eighty months). Fifteen grafts failed, twelve of which failed in the first four years. None of the thirty-four tibial reconstructions failed. Reconstruction site, patient age, allograft length, nail-only fixation, and non-bridging osteosynthesis were the most important risk factors for complications. Adjuvant chemotherapy and irradiation had no effects on complication rates.
CONCLUSIONS: We report high complication rates and considerable failure rates for the use of intercalary allografts; complications primarily occurred in the first years after surgery, but some occurred much later after surgery. To reduce the number of failures, we recommend reconsidering the use of allografts for reconstructions of defects that are ≥15 cm, especially in older patients, and applying bridging osteosynthesis with use of plate fixation.

Related: Osteosarcoma


Carter JM, Inwards CY, Jin L, et al.
Activating GNAS mutations in parosteal osteosarcoma.
Am J Surg Pathol. 2014; 38(3):402-9 [PubMed] Related Publications
Parosteal osteosarcoma is a surface-based osteosarcoma that often exhibits deceptively bland cytologic features, hindering diagnosis in small biopsies or when correlative radiologic imaging is not readily available. A number of benign and malignant fibro-osseous lesions, including fibrous dysplasia (FD) and low-grade central osteosarcoma, fall within the morphologic differential diagnosis of parosteal osteosarcoma. Somatic mutations in GNAS, encoding the α-subunit of the heterotrimeric G protein complex (Gsα), occur in FD and McCune-Albright syndrome but have not been reported in parosteal osteosarcoma. We evaluated GNAS mutational status in parosteal osteosarcoma and several of its histologic mimics to determine its utility in differentiating these entities. Eleven of 14 (79%) FD cases had GNAS mutations within codon 201 (5 R201C and 6 R201H mutations). GNAS mutations were not detected in any cases of adamantinoma or osteofibrous dysplasia. Direct sequencing of 9 parosteal osteosarcomas, including 3 of low grade and 6 with dedifferentiation, revealed activating GNAS mutations in 5 cases (55%), distributed as 4 R201C-mutated tumors and 1 tumor with an R201H mutation. GNAS codon 227 mutations were not detected in any of the cases. There was no association between GNAS mutational status and patient demographics, histologic dedifferentiation, or clinical outcome. To our knowledge, we report the first series of parosteal osteosarcomas harboring activating GNAS mutations. Our data suggest that GNAS mutational status may have limited utility as an ancillary technique in differentiating benign and malignant fibro-osseous lesions of the bone.

Related: Osteosarcoma


Li J, You T, Jing J
MiR-125b inhibits cell biological progression of Ewing's sarcoma by suppressing the PI3K/Akt signalling pathway.
Cell Prolif. 2014; 47(2):152-60 [PubMed] Related Publications
OBJECTIVES: Increasing evidence has suggested the close relationship between microRNAs (miRNAs) dysregulation and the carcinogenesis of Ewing's sarcoma (ES), among of which miR-125b has been reported to be decreased in ES tissues recently. Strikingly, ectopic expression of miR-125b could suppress cell proliferation of ES cell line A673, suggesting the tumor suppressor role of miR-125b in ES. However, the other accurate mechanistic functions and relative molecule mechanisms are largely unknown.
MATERIALS AND METHODS: Herein, we completed a series of experiments to investigate the role of miR-125b in Ewing's sarcoma. We restored the expression of miR-125b in ES cell line A673 through transfection with miR-125b mimics. To further understand the role of miR-125b in ES, we detected the effects of miR-125b on the cell proliferation, migration and invasion, cell cycle as well as cell apoptosis.
RESULTS: We found that restored expression of miR-125b in ES cell line A673 inhibited cell proliferation, migration and invasion, arrested cell cycle progression, and induced cell apoptosis. Moreover, bioinformatic prediction suggested the oncogene, phosphoinositide-3-kinase catalytic subunit delta (PIK3CD), was a target gene of miR-125b in ES cells. Further quantitative RT-PCR and western blot assays identified over-expression of miR-125b suppressed the expression of PIK3CD mRNA and protein. PIK3CD participates in regulating the PI3K signaling pathway, which has been reported to play an important role in the development of ES. Suppression of PIK3CD down-regulated the expression of phospho-AKT and phospho-mTOR proteins and inhibited the biologic progression of A673 cells.
CONCLUSIONS: Collectively, these data suggest that miR-125b functions as a tumor suppressor by targeting the PI3K/Akt/mTOR signaling pathway, and may provide potential therapy strategy for ES patients by targeting miRNA expression.

Related: AKT1 Ewing's Sarcoma Signal Transduction


Zhuang Y, Wei M
Impact of vascular endothelial growth factor expression on overall survival in patients with osteosarcoma: a meta-analysis.
Tumour Biol. 2014; 35(3):1745-9 [PubMed] Related Publications
Osteosarcoma is the most common primary malignant bone tumor of childhood. Vascular endothelial growth factor (VEGF) expression has been implicated in tumor development and progression of osteosarcoma, but previous studies investigating the impact of VEGF expression on overall survival in patients with osteosarcoma report conflicting findings. A meta-analysis of published studies was performed. The pooled hazard ratio (HR) with its 95% confidence interval (95% CI) was used to assess the impact of VEGF expression on overall survival in patients with osteosarcoma. Nine studies with a total of 432 osteosarcoma patients were included into this meta-analysis. There was no between-study heterogeneity among those nine studies (I (2) = 0.0%). Overall, high VEGF expression was obviously associated with poorer overall survival (HR = 1.68, 95% CI 1.33-2.12, P < 0.001). Sensitivity analysis performed by excluding single study in turns showed the pooled estimate was stable. Egger's test also did not suggest evidence for publication bias (P = 0.216). Therefore, this meta-analysis suggests that VEGF expression has an important impact on overall survival in patients with osteosarcoma and high VEGF expression is associated with poorer overall survival.

Related: Osteosarcoma VEGFA


Scaglioni MF, Chang EI, Gur E, et al.
The role of the fibula head flap for joint reconstruction after osteoarticular resections.
J Plast Reconstr Aesthet Surg. 2014; 67(5):617-23 [PubMed] Related Publications
INTRODUCTION AND AIM: Endoprosthetic reconstruction is considered the mainstay of limb salvage in periarticular bone tumours. However, this procedure has limited durability especially when performed in young patients. The free fibula head flap including the proximal articular surface represents one option for hemiarthroplasty reconstruction. The aim of this study was to investigate the role of the fibula head flap for joint reconstruction after osteoarticular resections.
PATIENTS AND METHODS: All patients who underwent hemiarthroplasty procedures between 2000 and 2006 using the free fibula head flap were included in the study. Functional assessments were performed using the American Musculoskeletal Tumor Society (AMTS) classification.
RESULTS: There were five males and two females (mean age: 22.6 ± 15.9 years). Five patients underwent reconstruction following resection of malignant bone tumours and two for chronic osteomyelitis of the distal humerus. In three patients, the fibula was used for distal radius and wrist joint reconstruction, and the remaining four patients for reconstruction of the distal humerus and elbow joint. A vascularised growth plate transfer based on the lateral geniculate vessels was performed in two patients. Atechnetium-bone scan confirmed viability of all flaps 10 days after surgery, and radiographic bony union was confirmed on average 5 months following surgery. There were no complications with the recipient or donor site after a median follow-up of 71 months (range: 12 months to 10 years). All patients achieved reasonable return of function and were able to perform all activities of daily living.
CONCLUSIONS: We demonstrate that the hemiarthroplasty procedure using the free fibula flap with its proximal head is a safe procedure with good functional results. Performing autologous arthroplasty using a free fibula head flap may be a promising alternative to an endoprosthesis or alloplastic reconstruction with a low risk of complications and morbidity.

Related: Osteosarcoma Ewing's Sarcoma


Na KY, Kim HS, Jung WW, et al.
CXCL16 and CXCR6 in Ewing sarcoma family tumor.
Hum Pathol. 2014; 45(4):753-60 [PubMed] Related Publications
Chemokines are a family of peptide mediators that play an essential role in cellular migration and intracellular communication in tumor cells as well as immune cells. We hypothesized that the CXCL16-CXCR6 ligand-receptor system plays an important role in Ewing sarcoma (ES) family tumor (ESFT) progression. Using real-time quantitative reverse transcription-polymerase chain reaction, we investigated the mRNA expression of CXCL16, CXCR6, and ADAM 10 in various cell lines. We also investigated the expression of CXCL16, CXCR6, ADAM 10, and ADAM 17 in tissue samples from 61 ESFT patients using immunohistochemistry. The mRNA expression levels of CXCL16 and CXCR6 in the ES cell line were higher than those in the other cell lines. Immunohistochemical staining revealed that CXCL16 and CXCR6 were highly expressed in tumor cells of ESFT and showed a positive correlation between them. The expression of CXCL16 and CXCR6 was associated with the occurrence of lung metastasis. Univariate analysis revealed that CXCL16 or CXCR6 expression was associated with worse prognosis of ESFT patients. In addition, CXCL16 and CXCR6 expression was associated with shorter overall survival irrespective of other prognostic factors. Our results suggest that the CXCL16/CXCR6 axis appears to be important in the progression of ESFT, resulting in more aggressive clinical behavior. Furthermore, there may be a decrease in the overall survival in ESFT patients who have tumors that stain strongly for CXCL16 and CXCR6.

Related: Ewing's Sarcoma


Uhl M, Mattke M, Welzel T, et al.
High control rate in patients with chondrosarcoma of the skull base after carbon ion therapy: first report of long-term results.
Cancer. 2014; 120(10):1579-85 [PubMed] Related Publications
BACKGROUND: The current study was performed to evaluate the safety and effectiveness of irradiation with carbon ions using raster scanning as well as prognostic factors in patients with skull base chondrosarcomas.
METHODS: Between 1998 and 2008, 79 patients with chondrosarcoma of the skull base were treated using carbon ions in raster scanning. The applied median total dose was 60 gray equivalent (GyE) at 3 GyE per fraction. Local control and overall survival (OS) were evaluated using the Kaplan-Meier method. Long-term toxicity was quantitatively assessed using questionnaires.
RESULTS: The median follow-up after irradiation was 91 months (range, 3 months-175 months). Within the follow-up, 10 patients developed local disease recurrence. The 3-year, 5-year, and 10-year local control rates were 95.9%, 88%, and 88%, respectively; the corresponding OS rates were 96.1%, 96.1%, and 78.9%, respectively. With a median follow-up of 110 months after first diagnosis, the corresponding 3-year, 5-year, and 10-year OS rates were 97.5%, 97.5%, and 91.5%, respectively. Age ≤ 45 years and boost volume ≤ 55 mL were associated with significantly better local control rates. We observed a clinically relevant improvement in cranial nerve deficits 7 to 10 years after treatment (range, 45.5%-53.3%) compared with the baseline (73.4%). During follow-up, none of the patients in the current study developed a secondary malignancy.
CONCLUSIONS: Carbon ion therapy is a safe and effective treatment in patients with chondrosarcoma of the skull base. For further evaluation, a prospective randomized phase 3 trial comparing protons versus carbon ions has been recruiting patients with low-grade and intermediate-grade chondrosarcoma of the skull base since 2009.

Related: Chondrosarcoma


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