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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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).

Kato Kaneko M, Liu X, Oki H, et al.
Isocitrate dehydrogenase mutation is frequently observed in giant cell tumor of bone.
Cancer Sci. 2014; 105(6):744-8 [PubMed] Related Publications
Giant cell tumors of bone (GCTB) are benign and locally destructive tumors that include osteoclast-type multinuclear giant cells. No available treatment is definitively effective in curing GCTB, especially in surgically unresectable cases. Isocitrate dehydrogenase (IDH) mutations have been reported not only in gliomas and acute myeloid leukemias, but also in cartilaginous tumors and osteosarcomas. However, IDH mutations in GCTB have not been investigated. The IDH mutations are remarkably specific to arginine 132 (R132) in IDH1 and arginine 172 (R172) or arginine 140 (R140) in IDH2; IDH1/2 mutations are known to convert α-ketoglutarate to oncometabolite R(-)-2-hydroxyglutarate. We recently reported that the most frequent IDH mutation in osteosarcomas is IDH2-R172S, which was detected by MsMab-1, a multispecific anti-IDH1/2 mAb. Herein, we newly report the IDH mutations in GCTB, which were stained by MsMab-1 in immunohistochemistry. DNA direct sequencing and subcloning identified IDH mutations of GCTB as IDH2-R172S (16 of 20; 80%). This is the first report to describe IDH mutations in GCTB, and MsMab-1 can be anticipated for use in immunohistochemical determination of IDH1/2 mutation-bearing GCTB.

Related: Monoclonal Antibodies Osteosarcoma

Goedhart LM, Ploegmakers JJ, Kroon HM, et al.
The presentation, treatment and outcome of periosteal chondrosarcoma in the Netherlands.
Bone Joint J. 2014; 96-B(6):823-8 [PubMed] Related Publications
In this case study, we describe the clinical presentation and treatment of 36 patients with periosteal chondrosarcoma collected over a 59-year period by the archive of the Netherlands Committee on Bone Tumours. The demographics, clinical presentation, radiological features, treatment and follow-up are presented with the size, location, the histological grading of the tumour and the survival. We found a slight predominance of men (61%), and a predilection for the distal femur (33%) and proximal humerus (33%). The metaphysis was the most common site (47%) and the most common presentation was with pain (44%). Half the tumours were classified histologically as grade 1. Pulmonary metastases were reported in one patient after an intra-lesional resection. A second patient died from local recurrence and possible pulmonary and skin metastases after an incomplete resection. It is clearly important to make the diagnosis appropriately because an incomplete resection may result in local recurrence and metastatic spread. Staging for metastatic disease is recommended in grade II or III lesions. These patients should be managed with a contrast-enhanced MRI of the tumour and histological confirmation by biopsy, followed by en-bloc excision.

Related: Chondrosarcoma

Adeniran JJ, Samuel EU, Dike OC, et al.
Bone malignancies in orthopaedic hospital Igbobi Lagos, Nigeria.
Niger Postgrad Med J. 2014; 21(1):66-7 [PubMed] Related Publications
AIMS AND OBJECTIVES: To document the pattern of bone malignancies in a highly populated orthopaedic hospital in Lagos Nigeria;
PATIENTS AND METHODS: A total of 21 cases of primary malignant bone tumours were studied. This comprised 12 cases of Osteosarcoma, 7 cases of Malignant Fibrous Histiocytoma (MFH) and 2 cases of Chondrosarcoma. Males (13) were affected more than females (8) giving a male to female ratio of 1.6 to 1. The age range was 7 to 45 years with a median age of 24 years. The diameter of the swelling ranged from 6 to 20 cm with a median of 12 cm. All patients had ablative surgery except for those with affectation of the ilium. Data was analysed using the Statistical Package for Social Sciences (SPSS 16). Enneking's classification was used to grade the tumour. The duration of symptoms of all the patients before presentation ranged from 3 weeks to 4 years with a mean of 7 months. The commonest site affected was around the knee (76.2%); distal femur had 42.9% and proximal tibia 33.3%.
RESULTS: Osteosarcoma was the most common malignant bone tumour in this series and accounted for 57.1%. The peak incidence was found in the 2nd decade of life. The youngest patient was 7 years old and the oldest 43 years. The tumour was found primarily around the knee. 7 cases were in the distal part of the femur, 4 in the proximal part of the tibia and 1 case was found in the distal radius. Out of the 12 patients with osteosarcoma, 8 had paraosteal type (5 high grade, 3 intermediate grade), the remaining 4 had periosteal (all high grade) Malignant Fibrous Histiocytoma was found in 7 patients and accounted for 33.3%. The peak incidence was found in 3rd and 4th decades. 4 out of the 7 patients were high grade pleomorphic osteosarcoma, 2 were myxoid high grade dedifferentiated and one was low grade giant cell tumour type. Chondrosarcoma was found in 2 patients, accounting for 9.5%. both cases were in the ilium
CONCLUSION: Primary malignant bone tumours occurred in children and young adult in this study. It is commoner among males and most of the patients presented late to the hospital. Osteosarcoma is the commonest followed by Malignant Fibrous Histiocytoma, both occurred commonly around the knee and chondrosarcoma on the ilium.

Related: Osteosarcoma

Biswas B, Rastogi S, Khan SA, et al.
Outcomes and prognostic factors for Ewing-family tumors of the extremities.
J Bone Joint Surg Am. 2014; 96(10):841-9 [PubMed] Related Publications
BACKGROUND: There are few published studies describing the clinical results of patients uniformly treated for a Ewing-family tumor of an extremity.
METHODS: We performed a review of patients who had received uniform treatment consisting of neoadjuvant chemotherapy, surgery and/or radiation therapy as local treatment, and then adjuvant chemotherapy from June 2003 to November 2011 at a single institution.
RESULTS: There were 158 patients included in the study. The median age was fifteen years. Sixty-nine (44%) of the patients had metastatic disease at presentation. Fifty-seven patients underwent surgery, and forty-one received radical radiation therapy following neoadjuvant chemotherapy. After a median of 24.3 months (range, 1.6 to ninety-seven months) of follow-up, the five-year event-free survival, overall survival, and local control rates (and standard error) were 24.1% ± 4.3%, 43.5% ± 6%, and 55% ± 6.8%, respectively, for the entire cohort and 36.4% ± 6.2%, 57.6% ± 7.4%, and 58.2% ± 7.9%, respectively, for patients without metastases. In the multivariate analysis, metastases predicted inferior event-free survival (p = 0.02) and overall survival (p = 0.03) rates in the entire cohort, whereas radical radiation therapy predicted an inferior local control rate in the entire cohort (p = 0.001) and in patients without metastases (p = 0.04). In the group with localized disease, there was no difference between the patients who received radical radiation therapy and those who underwent surgery with regard to tumor diameter (p = 0.8) or post-neoadjuvant chemotherapy response (p = 0.1). A white blood cell count (WBC) of >11 × 109/L predicted inferior event-free survival (p = 0.005) and local control (p = 0.02) rates for patients without metastases.
CONCLUSIONS: To our knowledge, this is the largest study on extremity Ewing-family tumors treated with uniform chemotherapy and either surgical resection or radical radiation therapy in Asia. All possible efforts should be made to resect a primary tumor after neoadjuvant chemotherapy, as radical radiation therapy alone results in a poor local control rate despite a good post-neoadjuvant chemotherapy response. Patients without metastases but with a high WBC had inferior event-free survival and local control rates and may require more aggressive therapy.
LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Related: Ewing's Sarcoma

Henderson M, Neumeister MW, Bueno RA
Hand tumors: II. Benign and malignant bone tumors of the hand.
Plast Reconstr Surg. 2014; 133(6):814e-821e [PubMed] Related Publications
The incidence of both benign and malignant bone tumors arising in the hand is relatively low in comparison with other locations. Although the overwhelming majority of these tumors are benign, even benign tumors can be locally destructive and compromise hand function. Intralesional tumor excision is the most appropriate surgical intervention for many benign bone tumors of the hand; however, destructive or malignant tumors may require wide local excision or even amputation to achieve complete tumor eradication. The purpose of this review article is to provide an overview of the pertinent benign and malignant bone tumors that may be encountered by hand surgeons. Clinical presentation, radiographic features, recommended workup, and available treatment options are all reviewed.

Related: Chondrosarcoma Ewing's Sarcoma

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

Hong JB, Cho KH, Choi JH
Periosteal osteosarcoma arising from the rib and scapula: imaging features in two cases.
Korean J Radiol. 2014; 15(3):370-5 [PubMed] Free Access to Full Article Related Publications
Periosteal osteosarcoma is an extremely rare chondroblastic osteosarcoma in the flat bone. There were authors reporting of two cases of periosteal osteosarcoma in the highly unusual sites. One of them arose from the rib, in a 17-year-old male, which appeared as a hypodense juxtacortical mass with periosteal reaction on CT. The other one arose from the scapula, in a 17-year-old female, which showed the intermediate signal intensity (SI) on T1-weighted image (WI), heterogeneous high SI on T2WI, and rim-enhancement on contrast-enhanced T1WI with cortical destruction on MRI.

Related: Osteosarcoma

Brasme JF, Chalumeau M, Oberlin O, et al.
Time to diagnosis of Ewing tumors in children and adolescents is not associated with metastasis or survival: a prospective multicenter study of 436 patients.
J Clin Oncol. 2014; 32(18):1935-40 [PubMed] Related Publications
PURPOSE: The time to diagnosis (TtD) of Ewing tumors is one of the longest among pediatric tumors. Its precise consequences, however, have not been studied well. We analyzed the distribution of TtD for Ewing tumors in children and adolescents and its association with clinical features, tumor characteristics, surgical outcome, and long-term survival.
PATIENTS AND METHODS: We analyzed prospectively collected data from two multicenter clinical trials of patients younger than 21 years old who had Ewing bone tumors treated in France between 1988 and 2000. Clinical and tumoral features, TtD, and outcome associations were studied by univariable and multivariable analyses.
RESULTS: The median TtD for the 436 patients was 70 days (interquartile range, 27 to 146 days), with no significant decrease during the study period (P > .2). The factors associated with long TtD were older age and some tumor sites (pelvis, extremities of limbs). Increased tumor volume and decreased histologic response to chemotherapy were associated with long TtD on univariable analysis (P < .05) but not after adjustment. Presence of a nerve or spinal-cord compression at diagnosis, presence or site of metastasis, surgical treatment, mutilating surgery, complete resection, or survival were not associated with TtD.
CONCLUSION: TtD of Ewing tumors was long, especially for adolescents and for certain tumor sites, and did not improve over time. But TtD was not associated with metastasis, surgical outcome, or survival. These findings could be used to comfort parents at diagnosis and in expert testimony produced for malpractice claims.

Related: France

Li X, Wang FS, Wu ZY, et al.
MicroRNA-19b targets Mfn1 to inhibit Mfn1-induced apoptosis in osteosarcoma cells.
Neoplasma. 2014; 61(3):265-73 [PubMed] Related Publications
Accumulative evidence has confirmed that, miR-17-92, a typical polycistronic mRNA cluster, was up-regulated in various solid tumors, and play an important role in the occurrence and development progress of tumors. In our study, we detected the six members of miR-17-92 cluster in osteosarcoma cell line, finding that the expression of miR-17 and miR-19b was up-regulated significantly. Further studies have found that Mfn1 was one of the target genes of miR-19b and the transcription and expression level of Mfn1 were down-regulated by miR-19b. MTS, flow cytometry, TUNEL-DAPI, Annexin V-FITC and transwell assay demonstrated that Mfn1 significantly blocked the cell cycle, promoted apoptosis and inhibited proliferation and invasion of osteosarcoma cells. Whereas, miR-19b targets 3'UTR sequences of Mfn1 genes inhibit the expression of Mfn1, thus inhibited Mfn1 triggered anti-cancer effect. Taken together, miR-19b functions by targeting Mfn1 reduce the protein expression level, thus provides a novel target to understand the molecular biology and genetics mechanisms of occurrence and development of osteosarcoma, contributing to the diagnosis and therapy of osteosarcoma.

Related: Apoptosis

Xu Z, Wang T
miR-214 promotes the proliferation and invasion of osteosarcoma cells through direct suppression of LZTS1.
Biochem Biophys Res Commun. 2014; 449(2):190-5 [PubMed] Related Publications
Previous studies have shown that miR-214 functions either as an oncogene or a tumor suppressor in various human cancer types. The role of this microRNA in osteosarcoma (OS) is presently unclear. Here, we demonstrated that miR-214 is frequently upregulated in OS specimens, compared with noncancerous bone tissues. Bioinformatics analysis further revealed leucine zipper, putative tumor suppressor 1 (LZTS1) as a potential target of miR-214. Expression patterns of miR-214 were inversely correlated with those of LZTS1 mRNA and protein in OS tissues. Data from reporter assays showed that miR-214 directly binds to the 3'-untranslated region (3'-UTR) of LZTS1 mRNA and suppresses expression at both transcriptional and translational levels. In functional assays, miR-214 promoted OS cell proliferation, invasion and tumor growth in nude mice, which could be reversed by overexpression of LZTS1. Taken together, our data provide compelling evidence that miR-214 functions as an onco-miRNA in OS, and its oncogenic effects are mediated chiefly through downregulation of LZTS1.

Related: Osteosarcoma

Zhang Y, Paz Mejia A, Temple HT, et al.
Squamous cell carcinoma arising in dedifferentiated chondrosarcoma proved by isocitrate dehydrogenase mutation analysis.
Hum Pathol. 2014; 45(7):1541-5 [PubMed] Related Publications
Dedifferentiated chondrosarcoma is a primary bone tumor characterized by the presence of both low-grade cartilaginous and high-grade malignant noncartilaginous components. The high-grade noncartilaginous component is typically a pleomorphic fibroblastic spindle cell sarcoma. Dedifferentiation into a malignant epithelial component is extremely rare. In this report, we present a 74-year-old woman who developed a metastatic squamous cell carcinoma in the right inguinal area 1 year after wide resection of her right proximal femur for a dedifferentiated chondrosarcoma. The dedifferentiated component was composed of poorly differentiated epithelioid cells with foci of squamous cell carcinoma. Mutational analysis was performed, and the isocitrate dehydrogenase 1 R132C mutation was detected in the low-grade chondrosarcoma, dedifferentiated chondrosarcoma as well as the metastatic squamous cell carcinoma. And this mutation was not detected in patient's normal tissue. Our study supports the theory that both the chondrosarcoma cells and dedifferentiated epithelioid tumor cells arose from the same clonal origin.

Related: Chondrosarcoma

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

Zhang H, Yin Z, Ning K, et al.
Prognostic value of microRNA-223/epithelial cell transforming sequence 2 signaling in patients with osteosarcoma.
Hum Pathol. 2014; 45(7):1430-6 [PubMed] Related Publications
MicroRNA-223 (miR-223) has been demonstrated to be implicated in cell proliferation and cell cycle progression of osteosarcoma cell lines by regulating its target gene epithelial cell transforming sequence 2 (ECT2). However, the clinical significance of the deregulation of the miR-223/Ect2 axis in human osteosarcoma has not been fully elucidated. To address this problem, we firstly showed that the expression levels of miR-223 and Ect2 messenger RNA were, respectively, down-regulated and up-regulated in osteosarcoma tissues compared with those in noncancerous bone tissues significantly (both P < .001), according to the results of quantitative real-time reverse transcription-polymerase chain reaction. Notably, miR-223 down-regulation was negatively correlated with Ect2 messenger RNA up-regulation in osteosarcoma tissues (r = -0.68, P = .01). Then, the combined low miR-223 expression and high Ect2 expression (miR-223-low/Ect2-high) was significantly associated with high tumor grade (P = .01), poor response to chemotherapy (P = .01), positive metastasis (P < .001), and recurrence (P < .001) of osteosarcomas. Moreover, patients with miR-223-low/Ect2-high expression had the shortest overall survival (P < .001) and disease-free survival (P < .001) compared with patients in the other 3 groups (miR-223-low/Ect2-low, miR-223-high/Ect2-high, and miR-223-high/Ect2-low). Furthermore, the multivariate analysis identified miR-223/Ect2 expression and the status of metastasis as independent prognostic factors for overall survival and disease-free survival. In conclusion, our data offer convincing evidence that the deregulation of miR-223 and its target gene ECT2 may be associated with the aggressive tumor progression of human osteosarcoma. Of note, the combined miR-223 down-regulation and Ect2 up-regulation may be a possible marker of poor prognosis in this malignancy.

Related: Osteosarcoma Signal Transduction ECT2

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.

Wan Y, Zhao W, Jiang Y, et al.
β-catenin is a valuable marker for differential diagnosis of osteoblastoma and osteosarcoma.
Hum Pathol. 2014; 45(7):1459-65 [PubMed] Related Publications
Osteoblastoma (OB) and osteosarcoma (OS) are 2 bone tumors that predominantly affect young adults. The clinical management of OS differs significantly from that of OB, and thus, accurate diagnosis of OB and OS is critical in determining appropriate treatment modality. However, in certain cases, OS significantly overlaps with OB in clinical and radiographic characteristics, and therefore, the differential diagnosis of OB and OS can be difficult, especially when biopsy material is insufficient. To date, there have been few reports on markers for differential diagnosis of OB and OS. We have previously shown that the Wnt/β-catenin pathway is inactivated in OS. In this study, we aimed to investigate whether the cellular distribution pattern of β-catenin is a potential marker for the differential diagnosis of OB and OS. Immunohistochemical staining was studied in 17 OB samples (21 biopsies; 17 primary and 4 recurrent) and 37 OS samples with complete follow-up information. Moderate-to-strong nuclear β-catenin staining was found in all OB specimens (17/17). In contrast, positive staining of β-catenin was found in the cytoplasm and/or membrane but not the nucleus in all 32 cases of nonchondroblastic OS (32/32) and the classic OS component in chondroblastic OS (5/5). The only positive nuclear β-catenin staining detected in OS biopsies was in chondroblastic OS cells (5/5). In summary, our results indicate that, in addition to conventional histopathologic evaluation, cellular distribution of β-catenin may be used as a valuable marker in the differential diagnosis of OB and OS. Nuclear β-catenin staining strongly suggests OB, whereas cytoplasmic/membranous staining of β-catenin suggests OS.

Related: Osteosarcoma

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

Rasper M, Jabar S, Ranft A, et al.
The value of high-dose chemotherapy in patients with first relapsed Ewing sarcoma.
Pediatr Blood Cancer. 2014; 61(8):1382-6 [PubMed] Related Publications
BACKGROUND: Prognosis of patients with relapsed Ewing sarcoma (ES) is poor. The 5-year overall survival (OS) is 13%. We analyzed high-dose chemotherapy (HDtx) versus conventional chemotherapy (CHtx) in patients with relapsed ES.
PROCEDURE: Data from 239 patients with first relapse, registered during 2000-2011 in the ES relapse registry of the Cooperative Ewing Sarcoma Study Group (CESS) were analyzed.
RESULTS: Of 239 patients, 200 received various non-HDtx second-line CHtx regimens. Seventy-three patients had additional HDtx followed by autologous stem cell rescue. The 2-year event-free survival (EFS) was 10% (SE = 0.02) in patients treated without HDtx and 45% (SE = 0.09) in patients treated with HDtx. In a second step, we focused on those patients who achieved complete remission (CR) or partial remission (PR) after four to six cycles of conventional second-line CHtx. Here, the 2-year EFS was 31% (SE = 0.08) without additional HDtx and 44% (SE = 0.09) with additional HDtx. In addition, multivariate regression analysis indicates absence of HDtx treatment, with a Hazard ratio (HR) of 2.90 (95% CI 1.41-6.0), and early relapse, with a HR of 4.76 (95% CI 2.31-9.78), as independent prognostic factors for EFS.
CONCLUSION: Additional HDtx may contribute to further reduce the risk of further events in patients who respond to conventional second-line CHtx.

Related: Ewing's Sarcoma

Condello M, Cosentino D, Corinti S, et al.
Voacamine modulates the sensitivity to doxorubicin of resistant osteosarcoma and melanoma cells and does not induce toxicity in normal fibroblasts.
J Nat Prod. 2014; 77(4):855-62 [PubMed] Article available free on PMC after 10/04/2015 Related Publications
In previous studies it has been demonstrated that the plant alkaloid voacamine (1), used at noncytotoxic concentrations, enhanced the cytotoxicity of doxorubicin and exerted a chemosensitizing effect on cultured multidrug-resistant (MDR) U-2 OS-DX osteosarcoma cells. The in vitro investigations reported herein gave the following results: (i) the chemosensitizing effect of 1, in terms of drug accumulation and cell survival, was confirmed using SAOS-2-DX cells, another MDR osteosarcoma cell line; (ii) compound 1 enhanced the cytotoxic effect of doxorubicin also on the melanoma cell line Me30966, intrinsically drug resistant and P-glycoprotein-negative; (iii) at the concentrations used to sensitize tumor cells, 1 was not cytotoxic to normal cells (human fibroblasts). These findings suggest possible applications of voacamine (1) in integrative oncologic therapies against resistant tumors.

Related: Apoptosis Doxorubicin Melanoma 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] Article available free on PMC after 18/04/2015 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

Hou CH, Lin FL, Tong KB, et al.
Transforming growth factor alpha promotes osteosarcoma metastasis by ICAM-1 and PI3K/Akt signaling pathway.
Biochem Pharmacol. 2014; 89(4):453-63 [PubMed] Related Publications
Osteosarcoma is the most common primary malignancy of bone and is characterized by a high malignant and metastatic potential. Transforming growth factor alpha (TGF-α) is classified as the EGF (epidermal growth factor)-like family, which is involved in cancer cellular activities such as proliferation, motility, migration, adhesion and invasion abilities. However, the effect of TGF-α on human osteosarcoma is largely unknown. We found that TGF-α increased the cell migration and expression of intercellular adhesion molecule-1 (ICAM-1) in human osteosarcoma cells. Transfection of cells with ICAM-1 siRNA reduced TGF-α-mediated cell migration. We also found that the phosphatidylinositol 3'-kinase (PI3K)/Akt/NF-κB pathway was activated after TGF-α treatment, and TGF-α-induced expression of ICAM-1 and cell migration was inhibited by the specific inhibitors and siRNAs of PI3K, Akt, and NF-κB cascades. In addition, knockdown of TGF-α expression markedly decreased cell metastasis in vitro and in vivo. Our results indicate that TGF-α/EGFR interaction elicits PI3K and Akt activation, which in turn activates NF-κB, resulting in the expression of ICAM-1 and contributing the migration of human osteosarcoma cells.


Chen J, Sun MX, Hua YQ, Cai ZD
Prognostic significance of serum lactate dehydrogenase level in osteosarcoma: a meta-analysis.
J Cancer Res Clin Oncol. 2014; 140(7):1205-10 [PubMed] Related Publications
BACKGROUND: A number of studies have investigated the role of serum lactate dehydrogenase (LDH) level in patients with osteosarcoma but have yielded inconsistent and inconclusive results. Thus, we conducted a meta-analysis to assess its prognostic value more precisely.
METHODS: Systematic computerized searches of PubMed, Embase and Web of Science databases were performed. The pooled hazard ratio (HR) with 95 % confidence intervals (95 % CI) of overall survival was used to assess the prognostic role of serum LDH level.
RESULTS: Ten studies published between 1997 and 2013 with a total of 943 osteosarcoma patients were included. Overall, the pooled HR for all ten eligible studies evaluating high LDH level on overall survival was 1.92(95 % CI 1.53-2.40). Sensitivity analysis suggested that the pooled HR was stable and omitting a single study did not change the significance of the pooled HR. Funnel plots and Egger's tests revealed there was some possibility of publication bias risk in the meta-analysis.
CONCLUSION: This meta-analysis shows that high serum LDH level is obviously associated with lower overall survival rate in patients with osteosarcoma, and it is an effective biomarker of prognosis.

Related: 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

Krych A, Odland A, Rose P, et al.
Oncologic conditions that simulate common sports injuries.
J Am Acad Orthop Surg. 2014; 22(4):223-34 [PubMed] Related Publications
Primary bone and soft-tissue tumors that mimic common sports injuries are relatively rare and are not often encountered by most orthopaedists. Prompt and accurate diagnosis of these tumors is crucial to maximize the clinical outcome. Many bone and soft-tissue tumors present disproportionately in young and active patients who are often involved in athletic activities. Thus, the clinician may misdiagnose these rare tumors as more common sports injuries. Symptoms that should raise suspicion for a neoplastic process include pain unrelated to activity and a clinical course that does not follow the typically expected recovery for a common sports injury. An awareness of the salient features of several bone and soft-tissue tumors as well as nononcologic processes that may simulate sports injuries can aid clinicians in the prompt diagnosis and clinical decision making of these rare tumors.

Related: Ewing's Sarcoma Synovial Sarcoma

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