scholarly journals Genomics and Therapeutic Vulnerabilities of Primary Bone Tumors

Cells ◽  
2020 ◽  
Vol 9 (4) ◽  
pp. 968 ◽  
Author(s):  
Katia Scotlandi ◽  
Claudia Maria Hattinger ◽  
Evelin Pellegrini ◽  
Marco Gambarotti ◽  
Massimo Serra
Keyword(s):  
Therapeutic Strategies ◽  
Common Variants ◽  
Primary Tumors ◽  
Primary Bone Tumors ◽  
Road Map ◽  
Tumors Of Bone ◽  
Primary Bone ◽  
Novel Therapeutic Strategies ◽  
Treatment Responsiveness ◽  
Selection Of

Osteosarcoma, Ewing sarcoma and chondrosarcoma are rare diseases but the most common primary tumors of bone. The genes directly involved in the sarcomagenesis, tumor progression and treatment responsiveness are not completely defined for these tumors, and the powerful discovery of genetic analysis is highly warranted in the view of improving the therapy and cure of patients. The review summarizes recent advances concerning the molecular and genetic background of these three neoplasms and, of their most common variants, highlights the putative therapeutic targets and the clinical trials that are presently active, and notes the fundamental issues that remain unanswered. In the era of personalized medicine, the rarity of sarcomas may not be the major obstacle, provided that each patient is studied extensively according to a road map that combines emerging genomic and functional approaches toward the selection of novel therapeutic strategies.

Nonneoplastic Lesions That Simulate Primary Tumors of Bone

2012 ◽  
Vol 136 (7) ◽  
pp. 772-788 ◽  
Author(s):  
Fabrizio Remotti ◽  
Frieda Feldman
Keyword(s):  
Clinical Information ◽  
Diagnostic Process ◽  
Bone Lesions ◽  
Primary Tumors ◽  
Primary Bone Tumors ◽  
Aneurysmal Bone Cysts ◽  
Tumors Of Bone ◽  
Primary Neoplasms ◽  
Primary Bone ◽  
The Many

Context.—The skeletal system may be affected by a variety of nonneoplastic lesions, which may potentially be confused with primary bone tumors on clinical, radiologic, and pathologic grounds. These conditions include fractures, infections, and reactive and degenerative processes, as well as an array of quasineoplastic entities, such as intramedullary cystic lesions like unicameral and aneurysmal bone cysts; fibro-osseous lesions, such as fibrous dysplasia; and exophytic entities, like osteochondromas. Objective.—To review clinical, radiographic, and histologic features of nonneoplastic lesions of bone, discussing the difficulties in diagnosis and the differential diagnosis with primary neoplasms of bone. Data Sources.—The sources include the more relevant medical literature on the different subjects and case-derived material. Conclusions.—Because many nonneoplastic bone lesions may require biopsy or resection, the general surgical pathologist must be familiar with these lesions and be aware that review of hematoxylin-eosin slides is only one of the many steps in the diagnostic process, which also includes review of imaging studies and all available clinical information. Morphologic analysis disconnected from the clinical and radiographic context may lead to misinterpretation.

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 Bone Microenvironment and Osteosarcoma Metastasis

2020 ◽  
Vol 21 (19) ◽  
pp. 6985 ◽  
Author(s):  
Chaofei Yang ◽  
Ye Tian ◽  
Fan Zhao ◽  
Zhihao Chen ◽  
Peihong Su ◽  
...  
Keyword(s):  
Bone Tumor ◽  
Poor Prognosis ◽  
Therapeutic Strategies ◽  
Bone Microenvironment ◽  
Primary Bone Tumor ◽  
Secondary Tumors ◽  
Primary Bone ◽  
Novel Therapeutic Strategies ◽  
The Relationship ◽  
Novel Therapeutic

The bone microenvironment is an ideal fertile soil for both primary and secondary tumors to seed. The occurrence and development of osteosarcoma, as a primary bone tumor, is closely related to the bone microenvironment. Especially, the metastasis of osteosarcoma is the remaining challenge of therapy and poor prognosis. Increasing evidence focuses on the relationship between the bone microenvironment and osteosarcoma metastasis. Many elements exist in the bone microenvironment, such as acids, hypoxia, and chemokines, which have been verified to affect the progression and malignance of osteosarcoma through various signaling pathways. We thoroughly summarized all these regulators in the bone microenvironment and the transmission cascades, accordingly, attempting to furnish hints for inhibiting osteosarcoma metastasis via the amelioration of the bone microenvironment. In addition, analysis of the cross-talk between the bone microenvironment and osteosarcoma will help us to deeply understand the development of osteosarcoma. The cellular and molecular protagonists presented in the bone microenvironment promoting osteosarcoma metastasis will accelerate the exploration of novel therapeutic strategies towards osteosarcoma.

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.

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