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Current Oncology Reports Mar 2018Mesenchymal chondrosarcoma is a rare but deadly form of chondrosarcoma that typically affects adolescents and young adults. While curative intent is possible for... (Review)
Review
Mesenchymal chondrosarcoma is a rare but deadly form of chondrosarcoma that typically affects adolescents and young adults. While curative intent is possible for patients with localized disease, few options exist for patients in the unresectable/metastatic setting. Thus, it is imperative to understand the fusion-driven biology of this rare malignant neoplasm so as to lead to the future development of better therapeutics for this disease. This manuscript will briefly review the clinical and pathologic features of mesenchymal chondrosarcoma followed by an appraisal of existing data linked to the fusions, HEY1-NCOA2 and IRF2BP2-CDX1, and the associated downstream pathways.
Topics: Bone Neoplasms; Chondrosarcoma, Mesenchymal; Humans; Oncogene Proteins, Fusion; Prognosis
PubMed: 29582189
DOI: 10.1007/s11912-018-0668-z -
Cancers Mar 2023Chondrosarcomas can be classified into various forms according to the presence or absence of a precursor lesion, location, and histological subtype. The new 2020 World... (Review)
Review
Chondrosarcomas can be classified into various forms according to the presence or absence of a precursor lesion, location, and histological subtype. The new 2020 World Health Organization (WHO) Classification of Tumors of Soft Tissue and Bone classifies chondrogenic bone tumors as benign, intermediate (locally aggressive), or malignant, and separates atypical cartilaginous tumors (ACTs) and chondrosarcoma grade 1 (CS1) as intermediate and malignant tumors. respectively. Furthermore, the classification categorizes chondrosarcomas (including ACT) into eight subtypes: central conventional (grade 1 vs. 2-3), secondary peripheral (grade 1 vs. 2-3), periosteal, dedifferentiated, mesenchymal, and clear cell chondrosarcoma. Most chondrosarcomas are the low-grade, primary central conventional type. The rarer subtypes include clear cell, mesenchymal, and dedifferentiated chondrosarcomas. Comprehensive analysis of the characteristic imaging findings can help differentiate various forms of chondrosarcomas. However, distinguishing low-grade chondrosarcomas from enchondromas or high-grade chondrosarcomas is radiologically and histopathologically challenging, even for experienced radiologists and pathologists.
PubMed: 36980590
DOI: 10.3390/cancers15061703 -
Cancer Medicine Jan 2023Mesenchymal chondrosarcoma (MCS) is an ultra-rare sarcoma that follows a more aggressive course than conventional chondrosarcoma. This study evaluates prognostic...
BACKGROUND
Mesenchymal chondrosarcoma (MCS) is an ultra-rare sarcoma that follows a more aggressive course than conventional chondrosarcoma. This study evaluates prognostic factors, treatments (surgery, chemotherapy, and radiation), and outcomes in an Australian setting.
METHODS
We collected demographics, clinicopathological variables, treatment characteristics, and survival status from patients with MCS registered on the national ACCORD sarcoma database. Outcomes include overall survival (OS) and progression-free survival (PFS).
RESULTS
We identified 22 patients with MCS between 2001-2022. Median age was 28 (range 10-59) years, 19 (86%) had localised disease at diagnosis of whom 16 had surgery (84%), 11 received radiation (58%), and 10 chemotherapy (53%). Ten (52%) developed recurrence and/or metastases on follow-up and three patients with initial metastatic disease received surgery, radiation, and chemotherapy. At a median follow-up of 50.9 (range 0.4-210) months nine patients had died. The median OS was 104.1 months (95% CI 25.8-182.3). There was improved OS for patients with localised disease who had surgical resection of the primary (p = 0.003) and those with ECOG 0-1 compared to 2-3 (p = 0.023) on univariate analysis.
CONCLUSIONS
This study demonstrates contemporary Australian treatment patterns of MCS. The role of chemotherapy for localised disease remains uncertain. Understanding treatment patterns and outcomes help support treatment decisions and design of trials for novel therapeutic strategies.
Topics: Humans; Child; Adolescent; Young Adult; Adult; Middle Aged; Chondrosarcoma, Mesenchymal; Bone Neoplasms; Australia; Soft Tissue Neoplasms; Sarcoma; Cohort Studies; Retrospective Studies
PubMed: 35603739
DOI: 10.1002/cam4.4849 -
Skeletal Radiology Mar 2023Sarcoma comprises a heterogenous entity of musculoskeletal malignancies arising from a mesenchymal origin. The diagnosis and management of pediatric sarcoma requires a... (Review)
Review
Sarcoma comprises a heterogenous entity of musculoskeletal malignancies arising from a mesenchymal origin. The diagnosis and management of pediatric sarcoma requires a multidisciplinary approach and the use of various imaging modalities including CT, MRI and FDG PET scans. FDG PET/CT (FDG PET), as a metabolic imaging, complements and provides superior diagnostic information as against other imaging modalities alone. Advantages of FDG PET in differentiating malignant sarcomatous lesions from benign lesions, and value in staging and restaging have been noted in several studies. The use of FDG PET in clinical management has increased over the years. The data on prognostication of outcomes or predicting responders to therapy with FDG PET in patients with sarcoma is somewhat limited. This review will focus on the pearls and pitfalls of FDG PET and role of FDG PET in initial extent of disease assessment, treatment response, and surveillance imaging pertaining to osteosarcoma, chondrosarcoma, Ewing's sarcoma, and rhabdomyosarcoma. We also discuss the limitations and unmet needs of FDG PET in the management of patients with sarcoma.
Topics: Child; Humans; Fluorodeoxyglucose F18; Positron Emission Tomography Computed Tomography; Bone Neoplasms; Sarcoma; Positron-Emission Tomography; Soft Tissue Neoplasms; Molecular Imaging; Neoplasm Staging; Radiopharmaceuticals
PubMed: 36173459
DOI: 10.1007/s00256-022-04182-7 -
Cancers Nov 2023Sarcomas are a heterogeneous group of malignant mesenchymal tumors, including soft tissue and bone sarcomas. Macrophages in the tumor microenvironment, involved in... (Review)
Review
Sarcomas are a heterogeneous group of malignant mesenchymal tumors, including soft tissue and bone sarcomas. Macrophages in the tumor microenvironment, involved in immunosuppression and leading to tumor development, are called tumor-associated macrophages (TAMs). TAMs are very important in modulating the microenvironment of sarcomas by expressing specific markers and secreting factors that influence immune and tumor cells. They are involved in many signaling pathways, such as p-STAT3/p-Erk1/2, PI3K/Akt, JAK/MAPK, and JAK/STAT3. TAMs also significantly impact the clinical outcomes of patients suffering from sarcomas and are mainly related to poor overall survival rates among bone and soft tissue sarcomas, for example, chondrosarcoma, osteosarcoma, liposarcoma, synovial sarcoma, and undifferentiated pleomorphic sarcoma. This review summarizes the current knowledge on TAMs in sarcomas, focusing on specific markers on sarcoma cells, cell-cell interactions, and the possibly involved molecular pathways. Furthermore, we discuss the clinical significance of macrophages in sarcomas as a potential target for new therapies, presenting clinical relevance, possible new treatment options, and ongoing clinical trials using TAMs in sarcoma treatment.
PubMed: 37958467
DOI: 10.3390/cancers15215294 -
Calcified Tissue International Feb 2018Bone sarcomas are tumours belonging to the family of mesenchymal tumours and constitute a highly heterogeneous tumour group. The three main bone sarcomas are... (Review)
Review
Bone sarcomas are tumours belonging to the family of mesenchymal tumours and constitute a highly heterogeneous tumour group. The three main bone sarcomas are osteosarcoma, Ewing sarcoma and chondrosarcoma each subdivided in diverse histological entities. They are clinically characterised by a relatively high morbidity and mortality, especially in children and adolescents. Although these tumours are histologically, molecularly and genetically heterogeneous, they share a common involvement of the local microenvironment in their pathogenesis. This review gives a brief overview of their specificities and summarises the main therapeutic advances in the field of bone sarcoma.
Topics: Bone Neoplasms; Female; Giant Cell Tumor of Bone; Humans; Male; Sarcoma; Tumor Microenvironment
PubMed: 29238848
DOI: 10.1007/s00223-017-0372-2 -
PloS One 2015Mesenchymal chondrosarcoma(MCS) is a rare high-grade variant of chondrosarcoma. Consensus has not been reached on its optimal management. Resection with wide margins is... (Review)
Review
BACKGROUND
Mesenchymal chondrosarcoma(MCS) is a rare high-grade variant of chondrosarcoma. Consensus has not been reached on its optimal management. Resection with wide margins is usually recommended, but the effect of margins has been demonstrated by little positive evidence. Moreover, the effectiveness of adjuvant chemo- and/or radiotherapy remains controversial.
OBJECTIVES
To describe the clinical characteristics and outcomes of MCS of bone and soft tissue, to assess the efficacies of surgery, chemotherapy and radiation, and finally to deliver a more appropriate therapy.
MATERIALS AND METHODS
We reviewed EMBASE-, MEDLINE-, Cochrane-, Ovid- and PubMed-based to find out all cases of MCS of bone and soft tissue described between April 1994 and April 2014. Description of treatment and regular follow-up was required for each study. Language was restricted to English and Chinese. Issues of age, gender, location, metastasis, and treatment were all evaluated for each case. Kaplan-Meier Method and Cox Proportional Hazard Regression Model were used in the survival analysis.
RESULTS
From the 630 identified publications, 18 meeting the inclusion criteria were selected, involving a total of 107 patients. Based on these data, the 5-, 10-and 20-year overall survival are 55.0%, 43.5% and 15.7% respectively. The 5-, 10-, 20- year event-free survival rates are 45.0%, 27.2% and 8.1%, respectively. Treatment without surgery is associated with poorer overall survival and event-free survival. Negative surgical margins could significantly bring down the local-recurrence rate and are associated with a higher event-free survival rate. Chemotherapy regime based on anthracyclines does not benefit the overall survival. The addition of radiation therapy is not significantly associated with the overall or event-free survival. However, we recommend radiation as the salvage therapy for patients with positive margin so as to achieve better local control.
CONCLUSIONS
This review shows that surgery is essential in the management of MCS of bone and soft tissue. Appropriate adjuvant therapy may reduce local recurrence, but cannot benefit the overall survival.
Topics: Bone Neoplasms; Chondrosarcoma, Mesenchymal; Humans; Soft Tissue Neoplasms; Treatment Outcome
PubMed: 25849226
DOI: 10.1371/journal.pone.0122216 -
Genes, Chromosomes & Cancer Nov 2022Mesenchymal chondrosarcoma (MCS) is a rare translocation-associated sarcoma, driven by a canonical HEY1::NCOA2 fusion. The tumors typically have a biphasic phenotype of...
BACKGROUND
Mesenchymal chondrosarcoma (MCS) is a rare translocation-associated sarcoma, driven by a canonical HEY1::NCOA2 fusion. The tumors typically have a biphasic phenotype of primitive small blue round cells intermixed with hyaline cartilage. The head and neck (HN) region is a common site for MCS, accounting for 12-45% of all cases reported.
AIMS
We assembled a relatively large cohort of 13 molecularly confirmed HN MCS for a detailed clinicopathologic analysis. The underlying fusion events were determined using fluorescence in situ hybridization and/or targeted RNA sequencing.
RESULTS
The median age of presentation was 19 years. Five MCSs (39%) had an intraosseous presentation (skull, maxilla, palate, and mandible), while the remaining eight cases occurred in the brain/meninges, orbit, and nasal cavity. Microscopically, HN MCSs were characterized by primitive round cells arranged in a distinctive nested architecture and a rich staghorn vasculature. A cartilaginous component of hyaline cartilage islands and/or single chondrocytes were present in 69% cases. A combined immunoprofile of CD99(+)/SATB2(+)/CD34(-)/STAT6(-) was typically noted. As this immunoprofile is non-specific, the referral diagnoses in cases lacking a cartilaginous component included Ewing sarcoma family and osteosarcoma. Among the seven patients with follow-up data, three developed distant metastasis and one died of disease.
CONCLUSION
HN MCS may arise at intra- or extra-osseous sites. The HN MCS appears to have a more prolonged survival compared other MCS sites. Testing for HEY1::NCOA2 fusion is recommended in HN tumors with nested round cell morphology and staghorn vasculature that lack a distinctive cartilaginous component.
Topics: Adult; Basic Helix-Loop-Helix Transcription Factors; Cell Cycle Proteins; Child; Chondrosarcoma, Mesenchymal; Female; Gene Fusion; Head and Neck Neoplasms; Humans; In Situ Hybridization, Fluorescence; Male; Nuclear Receptor Coactivator 2; Young Adult
PubMed: 35672279
DOI: 10.1002/gcc.23075 -
Pathology International Apr 2023
Topics: Humans; Chondrosarcoma, Mesenchymal; Oncogene Proteins, Fusion; Bone Neoplasms; Lung
PubMed: 36752330
DOI: 10.1111/pin.13313