scholarly journals Notch, Wnt,andHedgehogPathways in Rhabdomyosarcoma: From Single Pathways to an Integrated Network

Sarcoma ◽  
2012 ◽  
Vol 2012 ◽  
pp. 1-7 ◽  
Author(s):  
Josep Roma ◽  
Anna Almazán-Moga ◽  
Josep Sánchez de Toledo ◽  
Soledad Gallego
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Embryonic Development ◽  
Clinical Phenotype ◽  
Common Type ◽  
Signalling Pathways ◽  
Therapeutic Approaches ◽  
Integrated Network ◽  
Molecular Features ◽  
Normal Embryonic Development

Rhabdomyosarcoma (RMS) is the most common type of soft tissue sarcoma in children. Regarding histopathological criteria, RMS can be divided into 2 main subtypes: embryonal and alveolar. These subtypes differ considerably in their clinical phenotype and molecular features. Abnormal regulation or mutation of signalling pathways that regulate normal embryonic development such asNotch, Hedgehog,andWntis a recurrent feature in tumorigenesis. Herein, the general features of each of the three pathways, their implication in cancer and particularly in RMS are reviewed. Finally, the cross-talking among these three pathways and the possibility of better understanding of the horizontal communication among them, leading to the development of more potent therapeutic approaches, are discussed.

scholarly journals Genomic landscape of gliosarcoma: distinguishing features and targetable alterations

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Mark M. Zaki ◽  
Leila A. Mashouf ◽  
Eleanor Woodward ◽  
Pinky Langat ◽  
Saksham Gupta ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Information Exchange ◽  
Predictive Biomarker ◽  
Disease Classification ◽  
Sequencing Data ◽  
Genomic Signatures ◽  
Molecular Features ◽  
Genomic Landscape ◽  
Distinguishing Features

AbstractGliosarcoma is an aggressive brain tumor with histologic features of glioblastoma (GBM) and soft tissue sarcoma. Despite its poor prognosis, its rarity has precluded analysis of its underlying biology. We used a multi-center database to characterize the genomic landscape of gliosarcoma. Sequencing data was obtained from 35 gliosarcoma patients from Genomics Evidence Neoplasia Information Exchange (GENIE) 5.0, a database curated by the American Association of Cancer Research (AACR). We analyzed genomic alterations in gliosarcomas and compared them to GBM (n = 1,449) and soft tissue sarcoma (n = 1,042). 30 samples were included (37% female, median age 59 [IQR: 49–64]). Nineteen common genes were identified in gliosarcoma, defined as those altered in > 5% of samples, including TERT Promoter (92%), PTEN (66%), and TP53 (60%). Of the 19 common genes in gliosarcoma, 6 were also common in both GBM and soft tissue sarcoma, 4 in GBM alone, 0 in soft tissue sarcoma alone, and 9 were more distinct to gliosarcoma. Of these, BRAF harbored an OncoKB level 1 designation, indicating its status as a predictive biomarker of response to an FDA-approved drug in certain cancers. EGFR, CDKN2A, NF1, and PTEN harbored level 4 designations in solid tumors, indicating biological evidence of these biomarkers predicting a drug-response. Gliosarcoma contains molecular features that overlap GBM and soft tissue sarcoma, as well as its own distinct genomic signatures. This may play a role in disease classification and inclusion criteria for clinical trials. Gliosarcoma mutations with potential therapeutic indications include BRAF, EGFR, CDKN2A, NF1, and PTEN.

scholarly journals Hedgehog Pathway Inhibition Hampers Sphere and Holoclone Formation in Rhabdomyosarcoma

2017 ◽  
Vol 2017 ◽  
pp. 1-14 ◽  
Author(s):  
Ana Almazán-Moga ◽  
Patricia Zarzosa ◽  
Isaac Vidal ◽  
Carla Molist ◽  
Irina Giralt ◽  
...  
Keyword(s):  
Stem Cells ◽  
Stem Cell ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Common Type ◽  
Hedgehog Pathway ◽  
Cellular Heterogeneity ◽  
Self Renewal ◽  
Pathway Inhibition

Rhabdomyosarcoma (RMS) is the most common type of soft tissue sarcoma in children and can be divided into two main subtypes: embryonal (eRMS) and alveolar (aRMS). Among the cellular heterogeneity of tumors, the existence of a small fraction of cells called cancer stem cells (CSC), thought to be responsible for the onset and propagation of cancer, has been demonstrated in some neoplasia. Although the existence of CSC has been reported for eRMS, their existence in aRMS, the most malignant subtype, has not been demonstrated to date. Given the lack of suitable markers to identify this subpopulation in aRMS, we used cancer stem cell-enriched supracellular structures (spheres and holoclones) to study this subpopulation. This strategy allowed us to demonstrate the capacity of both aRMS and eRMS cells to form these structures and retain self-renewal capacity. Furthermore, cells contained in spheres and holoclones showed significant Hedgehog pathway induction, the inhibition of which (pharmacologic or genetic) impairs the formation of both holoclones and spheres. Our findings point to a crucial role of this pathway in the maintenance of these structures and suggest that Hedgehog pathway targeting in CSC may have great potential in preventing local relapses and metastases.

Retroperitoneal Soft Tissue Sarcoma: Prognostic Factors and Therapeutic Approaches

2008 ◽  
Vol 94 (4) ◽  
pp. 497-504 ◽  
Author(s):  
Fabio Pacelli ◽  
Antonio Pio Tortorelli ◽  
Fausto Rosa ◽  
Valerio Papa ◽  
Maurizio Bossola ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Therapeutic Approaches ◽  
Retroperitoneal Soft Tissue Sarcoma

Morphological Examination of a Herpes-type Virus Indigenous for Tree Shrews

1970 ◽  
Vol 28 ◽  
pp. 160-161
Author(s):  
J. P. Brunschwig ◽  
R. M. McCombs ◽  
R. Mirkovic ◽  
M. Benyesh-Melnick
Keyword(s):  
Electron Microscopy ◽  
Soft Tissue ◽  
Chick Embryo ◽  
Soft Tissue Sarcoma ◽  
Cell Cultures ◽  
Tree Shrew ◽  
Type Virus ◽  
Morphological Examination ◽  
Tree Shrews ◽  
Embryo Cells

A new virus, established as a member of the herpesvirus group by electron microscopy, was isolated from spontaneously degenerating cell cultures derived from the kidneys and lungs of two normal tree shrews. The virus was found to replicate best in cells derived from the homologous species. The cells used were a tree shrew cell line, T-23, which was derived from a spontaneous soft tissue sarcoma. The virus did not multiply or did so poorly for a limited number of passages in human, monkey, rodent, rabbit or chick embryo cells. In the T-23 cells, the virus behaved as members of the subgroup B of herpesvirus, in that the virus remained primarily cell associated.

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