Imaging of Pediatric Bone Tumors: Osteosarcoma and Ewing Sarcoma

2009 ◽  
pp. 259-273 ◽  
Author(s):  
Geetika Khanna
Keyword(s):  
Ewing Sarcoma ◽  
Bone Tumors

scholarly journals The predictive role of Ki-67 protein in determining the aggressiveness of primary malignant bone tumors. A retrospective study

2019 ◽  
Vol 2 (2) ◽  
pp. 91-95
Author(s):  
Ioan-Mihai Japie ◽  
Dragoș Rădulescu ◽  
Adrian Bădilă ◽  
Alexandru Papuc ◽  
Traian Ciobanu ◽  
...  
Keyword(s):  
High Risk ◽  
Ewing Sarcoma ◽  
Bone Tumors ◽  
Risk Group ◽  
High Risk Group ◽  
Low Risk ◽  
The Other ◽  
Malignant Bone Tumors ◽  
Ki 67

AbstractIntroduction: In order to diagnose and stage malignant bone tumors, the pathologic examination of harvested pieces with immunohistochemistry test is necessary; they also provide information regarding the prognosis on a medium to long term. Among tissular biomarkers with potential predictive value, a raised Ki-67 protein level is used to determine the risk of local recurrence or metastasis.Material and method: This study was performed on 50 patients with primary malignant bone tumors admitted in the Traumatology and Orthopedy Department of University Emergency Hospital, Bucharest. Patients repartition according to diagnosis was the following: 21 patients with osteosarcoma, 18 patients with chondrosarcoma, 6 patients with Ewing sarcoma, 3 patients with malignant fibrous histiocytoma, and 2 with fibrosarcoma. The follow-up period was between 12 and 72 months with a mean of 26 months.Results: Patients were aged between 18 and 77 years old, with a mean age of 41,36. There were 22 women and 28 men. No sex or age difference was notable for the tumor outcome. After calculating the Ki-67 LI, 36 patients were included in the high-risk group (Ki-67 LI > 25%), while 14 had a low risk for metastasis and local relapse (Ki-67 < 25%). The low-risk patients had chondrosarcoma (8 patients), osteosarcoma (5 patients), and fibrosarcoma (1 patient). During the follow-up, 8 patients, all belonging to the high risk group, developed metastasis, while 5 patients developed local recurrences; 4 patients who relapsed belonged to the high risk group and 1 to the low risk group. Metastases developed in 3 patients with osteosarcoma, 2 with Ewing sarcoma, 2 with chondrosarcoma and 1 patient with fibrosarcoma. Most metastases occurred within one year after surgery. The other fibrosarcoma patient developed local recurrence after 6 months, while the other local recurrences occurred in osteosarcoma patients (2 cases) and 1 in a Ewing sarcoma patient and chondrosarcoma patient.Conclusions: Our study concluded that while Ki-67 LI values are useful in determining the aggressivity of primary malignant bone tumors, it should always be used in conjunction with the clinical, imaging and anatomopathological diagnosis methods in order to accurately predict the patients’ outcome.

scholarly journals SHH Signaling Pathway Drives Pediatric Bone Sarcoma Progression

Cells ◽  
2020 ◽  
Vol 9 (3) ◽  
pp. 536 ◽  
Author(s):  
Frédéric Lézot ◽  
Isabelle Corre ◽  
Sarah Morice ◽  
Françoise Rédini ◽  
Franck Verrecchia
Keyword(s):  
Signaling Pathway ◽  
Ewing Sarcoma ◽  
Bone Tumors ◽  
Current Knowledge ◽  
Bone Sarcoma ◽  
Bone Homeostasis ◽  
Shh Signaling ◽  
Primary Bone Tumors ◽  
Shh Pathway ◽  
Primary Bone

Primary bone tumors can be divided into two classes, benign and malignant. Among the latter group, osteosarcoma and Ewing sarcoma are the most prevalent malignant primary bone tumors in children and adolescents. Despite intensive efforts to improve treatments, almost 40% of patients succumb to the disease. Specifically, the clinical outcome for metastatic osteosarcoma or Ewing sarcoma remains poor; less than 30% of patients who present metastases will survive 5 years after initial diagnosis. One common and specific point of these bone tumors is their ability to deregulate bone homeostasis and remodeling and divert them to their benefit. Over the past years, considerable interest in the Sonic Hedgehog (SHH) pathway has taken place within the cancer research community. The activation of this SHH cascade can be done through different ways and, schematically, two pathways can be described, the canonical and the non-canonical. This review discusses the current knowledge about the involvement of the SHH signaling pathway in skeletal development, pediatric bone sarcoma progression and the related therapeutic options that may be possible for these tumors.

scholarly journals Rare Primary Malignant Bone Sarcomas

Cancers ◽  
2020 ◽  
Vol 12 (11) ◽  
pp. 3092
Author(s):  
Emanuela Palmerini ◽  
Alberto Righi ◽  
Eric L. Staals
Keyword(s):  
Ewing Sarcoma ◽  
Bone Tumors ◽  
Spindle Cell ◽  
Clinical Aspects ◽  
Vascular Tumors ◽  
Nerve Sheath Tumor ◽  
Round Cell ◽  
Primary Bone Tumors ◽  
Bone Sarcomas ◽  
Primary Bone

Rare primary malignant bone sarcomas (RPMBS), other than osteosarcoma, chondrosarcoma, chordoma, and Ewing sarcoma, account for about 5–10% of primary bone tumors and represent a major diagnostic challenge. These tumors include spindle cell and round cell sarcoma entities, hemangiopericytoma-like and vascular tumors. Additionally, several histotypes, traditionally described in the soft tissues, such as myxofibrosarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumor of bone, have been reported in patients with primary bone tumors. While wide surgical resection is the mainstay of local treatment, systemic therapy of these rare entities is controversial. Patients with undifferentiated spindle cell or pleomorphic high-grade tumors of bone, are usually treated with osteosarcoma-like chemotherapy, while patients with round cell and undifferentiated round cell tumors (URCTs), may respond to sarcoma treatment regimens for Ewing sarcoma patients. Studies on analogies and differences among these ultra-rare tumors have seldom been reported. This review describes relevance, clinical aspects, diagnostic procedures, staging, treatment recommendations, and current research in this composite tumor group.

scholarly journals Association between TP53 rs1042522 gene polymorphism and the risk of malignant bone tumors: a meta-analysis

2019 ◽  
Vol 39 (3) ◽  
Author(s):  
Xin Huang ◽  
Fashuai Wu ◽  
Zhicai Zhang ◽  
Zengwu Shao
Keyword(s):  
Ewing Sarcoma ◽  
Bone Tumors ◽  
Meta Analysis ◽  
Suppressor Gene ◽  
Tumor Formation ◽  
Cochrane Library ◽  
Nucleotide Polymorphisms ◽  
Malignant Bone Tumors ◽  
Increased Risk ◽  
The Cochrane Library

AbstractTP53 is a tumor suppressor gene which is essential for regulating cell division and preventing tumor formation. Several studies have assessed the associations of TP53 single-nucleotide polymorphisms (SNP) with susceptibility of malignant bone tumors, including osteosarcoma and Ewing sarcoma, but the results are inconsistent. In the present meta-analysis, we aimed to elucidate the associations of TP53 rs1042522 genetic polymorphism with the risk of osteosarcoma or Ewing sarcoma. We systematically searched Medline, PubMed, Web of Science, Embase, and the Cochrane Library databases. Eligible studies assessing the polymorphisms in the TP53 rs1042522 gene and risk of malignant bone tumors were incorporated. The pooled odds ratio (OR) with its 95% confidence intervals (95% CIs) were used to assess these possible associations. Five studies with a total of 567 cases and 935 controls were finally included the meta-analysis. Meta-analysis of TP53 rs1042522 polymorphism was significantly associated with an increased risk of malignant bone tumors (G versus C: OR = 1.27, 95% CI 1.08–1.50, P=0.005; GG versus GC/CC: OR = 1.55, 95% CI 1.21–2.00, P=0.001). Moreover, in a stratified analysis, a statistically significant correlation between this SNP and osteosarcoma risk was also observed. Our results suggest that there are significant associations of TP53 rs1042522 polymorphism with malignant bone tumors risk. More studies based on larger sample sizes and homogeneous samples are warranted to confirm these findings.

Gap junction in bone remodeling and in primary bone tumors: osteosarcoma and Ewing sarcoma

Bone Cancer ◽  
2015 ◽  
pp. 83-89
Author(s):  
Julie Talbot ◽  
Audrey Lamora ◽  
Verena Stresing ◽  
Franck Verrecchia
Keyword(s):  
Gap Junction ◽  
Bone Remodeling ◽  
Ewing Sarcoma ◽  
Bone Tumors ◽  
Primary Bone Tumors ◽  
Primary Bone

scholarly journals Effective factors on clinical outcomes of children with osteosarcoma and Ewing bone tumors

2020 ◽  
Vol 6 (2) ◽  
pp. e26-e26
Author(s):  
Sasan Hejazi ◽  
Mehran Noroozi ◽  
Mohammad Amin Kuhestani ◽  
Farid Ghazizadeh ◽  
Mohammad Karmiyar ◽  
...  
Keyword(s):  
Survival Rate ◽  
Incidence Rate ◽  
Bone Tumor ◽  
Ewing Sarcoma ◽  
Bone Tumors ◽  
Correct Diagnosis ◽  
Survival Rates ◽  
Bone Cancer ◽  
Malignant Bone Tumors ◽  
Childhood Malignancies

Introduction: Malignant bone tumors account for 6% of childhood malignancies. The peak incidence rate for bone cancer is in adolescents. Geographic and race differences in incidence rate of bone tumors are important for correct diagnosis and treatment. Objectives: This study aimed to investigate the demographic features of patients with malignant bone tumors and prognostic factors. Patients and Methods: In a retrospective study, records of all patients diagnosed and treated for malignant bone tumor from March 1996 to February 2014 were investigated. Data were collected using checklists and analyzed using SPSS software. Results: The most common type of bone tumor was Ewing sarcoma (73.7%) in patients who were over 10 years old (87.5%). Primary site of disease in most cases of osteosarcoma and Ewing sarcoma was distal femur (50% and 27.3%, respectively). Most common clinical manifestation was local pain and mass (88.5%). Regarding metastasis, 72.7% of osteosarcoma patients and 32.4% of Ewing sarcoma patients had metastasis. There was not statistically relationship between survival rate and gender, age group and stage of disease at diagnosis time. One, three and five-year survival rate was lower than the previous studies. Conclusion: The incidence of Ewing sarcoma is more then osteosarcoma in West Azerbaijan. In our study there were no statistically important differences in survival rates of patients with metastasis at diagnosis time and those without metastasis.

scholarly journals New Insights about the Wnt/β-Catenin Signaling Pathway in Primary Bone Tumors and Their Microenvironment: A Promising Target to Develop Therapeutic Strategies?

2019 ◽  
Vol 20 (15) ◽  
pp. 3751 ◽  
Author(s):  
Danieau ◽  
Morice ◽  
Rédini ◽  
Verrecchia ◽  
Royer
Keyword(s):  
Signaling Pathway ◽  
Ewing Sarcoma ◽  
Bone Tumors ◽  
Tumor Resection ◽  
Therapeutic Strategies ◽  
Bone Homeostasis ◽  
Current Standard ◽  
Primary Bone Tumors ◽  
Promising Target ◽  
Primary Bone

Osteosarcoma and Ewing sarcoma are the most common malignant primary bone tumors mainly occurring in children, adolescents and young adults. Current standard therapy includes multidrug chemotherapy and/or radiation specifically for Ewing sarcoma, associated with tumor resection. However, patient survival has not evolved for the past decade and remains closely related to the response of tumor cells to chemotherapy, reaching around 75% at 5 years for patients with localized forms of osteosarcoma or Ewing sarcoma but less than 30% in metastatic diseases and patients resistant to initial chemotherapy. Despite Ewing sarcoma being characterized by specific EWSR1-ETS gene fusions resulting in oncogenic transcription factors, currently, no targeted therapy could be implemented. It seems even more difficult to develop a targeted therapeutic strategy in osteosarcoma which is characterized by high complexity and heterogeneity in genomic alterations. Nevertheless, the common point between these different bone tumors is their ability to deregulate bone homeostasis and remodeling and divert them to their benefit. Therefore, targeting different actors of the bone tumor microenvironment has been hypothesized to develop new therapeutic strategies. In this context, it is well known that the Wnt/β-catenin signaling pathway plays a key role in cancer development, including osteosarcoma and Ewing sarcoma as well as in bone remodeling. Moreover, recent studies highlight the implication of the Wnt/β-catenin pathway in angiogenesis and immuno-surveillance, two key mechanisms involved in metastatic dissemination. This review focuses on the role played by this signaling pathway in the development of primary bone tumors and the modulation of their specific microenvironment.

scholarly journals Successful Spontaneous Pregnancy after Treatment for Ewing Sarcoma including Sacrectomy

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
T. Hockertz ◽  
M. Velickovic
Keyword(s):  
Radiation Therapy ◽  
Survival Rate ◽  
Ewing Sarcoma ◽  
Pediatric Cancer ◽  
Bone Tumors ◽  
Reproductive Capacity ◽  
Affected Patient ◽  
Malignant Bone Tumors ◽  
Spontaneous Pregnancy ◽  
Surgical Methods

Ewing sarcomas are highly malignant bone tumors and usually occur in childhood. Radiation therapy, chemotherapy, and surgical methods increase the survival rate of the affected patient, but infertility and reduced reproductive capacity are common late effects of pediatric cancer treatment.

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