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 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.

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 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 Spindle cell sarcoma of sphenoid bone

2017 ◽  
Vol 5 (11) ◽  
pp. 5072
Author(s):  
Moorthy N. L. Nimmagadda ◽  
Vithaleswar M. Rao ◽  
Padmaja S. ◽  
Harshini Reddy
Keyword(s):  
Bone Tumors ◽  
Spindle Cell ◽  
Cell Tumor ◽  
Sphenoid Bone ◽  
Ossifying Fibroma ◽  
Spindle Cell Sarcoma ◽  
Cell Sarcoma ◽  
Primary Bone Tumors ◽  
Primary Bone ◽  
The Common

Primary bone tumors involving skull are extremely rare and they constitute 0.8% of all bone tumors. The common tumors that are seen in skull base include fibrous dysplasia, giant cell tumor, chordoma, ossifying fibroma, angiosarcoma. We report a rare case of spindle cell sarcoma arising from right sphenoid bone in a 70-year-old male which presented as unilateral defective vision with mild proptosis.

Scintigraphy with 99m Tc-Phosphonate and 67Gallium in Patients Suspected of Primary Bone Tumors

1982 ◽  
Vol 21 (04) ◽  
pp. 136-139 ◽  
Author(s):  
C.-J. Edeling
Keyword(s):  
Soft Tissue ◽  
Bone Scintigraphy ◽  
Primary Tumor ◽  
Bone Tumors ◽  
Whole Body ◽  
Skeletal Metastases ◽  
Malignant Bone Tumors ◽  
67Ga Scintigraphy ◽  
Primary Bone Tumors ◽  
Primary Bone

Whole-body scintigraphy with both 99mTc-phosphonate and 67Ga was performed on 92 patients suspected of primary bone tumors. In 46 patients with primary malignant bone tumors, scintigraphy with 99mTc-phosphonate disclosed the primary tumor in 44 cases and skeletal metastases in 11, and 67Ga scintigraphy detected the primary tumor in 43 cases, skeletal metastases in 6 cases and soft-tissue metastases in 8 cases. In 25 patients with secondary malignant bone tumors, bone scintigraphy visualized a single lesion in 10 cases and several lesions in 15 cases, and 67Ga scintigraphy detected the primary tumor in 17 cases, skeletal metastases in 17 cases and soft-tissue metastases in 9 cases. In 21 patients with benign bone disease positive uptake of 99mTc-phosphonate was recognized in 19 cases and uptake of 67Ga in 17 cases. It is concluded that bone scintigraphy should be used in patients suspected of primary bone tumors. If malignancy is suspected, 67Ga scintigraphy should be performed in addition.

CRYOSURGERY IN THE TREATMENT OF PATIENTS WITH BONE TUMORS

2019 ◽  
Vol 65 (2) ◽  
pp. 165-171
Author(s):  
Aleksey Belyaev ◽  
Georgiy Prokhorov ◽  
Anna Arkhitskaya
Keyword(s):  
Liquid Nitrogen ◽  
Bone Tumors ◽  
Cryogenic Treatment ◽  
Long Term Results ◽  
Bone Lesions ◽  
Giant Cell Tumors ◽  
Primary Bone Tumors ◽  
Circulation Circuit ◽  
Recent Emergence ◽  
Primary Bone

A review of the literature shows that surgical procedures will remain the standard treatment for primary bone tumors. Analysis of studies on the assessment of long-term results shows that additional double cryogenic treatment of the curettage cavity can improve the treatment outcomes of patients with giant cell tumors, dysplastic diseases and some forms of malignant lesions. The traditional execution of the procedure is associated with the open installation of liquid nitrogen in the bone cavity, which requires special skills in handling aggressive refrigerant from the staff and does not exclude complications. In case of multiple metastatic bone lesions, surgical treatment is not indicated. The recent emergence in clinical practice of new equipment with a closed liquid nitrogen circulation circuit inside cryoprobes resumes interest in cryoabla-tion of bone tumor lesions using modern minimally invasive puncture cryotechnology and expanding indications for its use in patients with severe comorbidities.

Wide Surgery in the Cervical Spine: Indications, Results, and Complications in a Series of 30 Patients Affected by Primary Bone Tumors

2021 ◽  
Author(s):  
Alessandro Luzzati ◽  
Gennaro Maria Scotto ◽  
Luca Cannavò ◽  
Giuseppe Orlando ◽  
Alessandra Scotto di Uccio ◽  
...  
Keyword(s):  
Cervical Spine ◽  
Bone Tumors ◽  
Primary Bone Tumors ◽  
Primary Bone

scholarly journals Primary Bone Tumors: Epidemiologic Comparison of 9200 Patients Treated at Beijing Ji Shui Tan Hospital, Beijing, China, With 10 165 Patients at Mayo Clinic, Rochester, Minnesota

2015 ◽  
Vol 139 (9) ◽  
pp. 1149-1155 ◽  
Author(s):  
Xiaohui Niu ◽  
Hairong Xu ◽  
Carrie Y. Inwards ◽  
Yuan Li ◽  
Yi Ding ◽  
...  
Keyword(s):  
Mayo Clinic ◽  
Bone Tumors ◽  
Age Distribution ◽  
Chinese Patients ◽  
Malignant Bone Tumors ◽  
Primary Bone Tumors ◽  
Male Predominance ◽  
Primary Bone ◽  
Different Populations ◽  
Epidemiologic Characteristics

Context Although primary bone tumors are extremely rare, the literature suggests that there are variations in the epidemiologic characteristics in different populations. The most frequently cited epidemiologic characteristics of primary bone tumors are derived from a large US series (Mayo Clinic), with no comparable study thus far performed in China. Objective To identify any potential epidemiologic differences between Chinese patients and a US series of patients. Design We performed a comparison study between 9200 patients treated at Beijing Ji Shui Tan Hospital (JST) and 10 165 patients treated at Mayo Clinic (MC), Rochester Minnesota. Detailed epidemiologic features were analyzed. Results We found that giant cell tumor and osteosarcoma have significantly higher incidences in the JST than the MC patients (P < .001). However, JST patients had a significantly lower incidence of Ewing sarcoma, chordoma, fibrosarcoma, myeloma, and malignant lymphoma (P < .001). For most benign and malignant bone tumors, the Chinese cohort had a more distinct male predominance than the US cohort. Malignant bone tumors had a monomodal age distribution in the JST patient group, with a bimodal age distribution in the MC cohort. Also, there were was a predilection for tumors of the femur and tibia among the JST patients (P < .001). Conclusions Our data confirm that epidemiologic variations of primary bone tumors exist in different populations. Factors that may contribute to these observed differences are proposed and discussed.

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