Immunophenotypic features of dedifferentiated skull base chordoma: An insight into the intratumoural heterogeneity
Chordomas are rare and low-grade malignant solid tumours, despite their histologically benign appearance, that arise in the bone from embryonic notochordal vestiges of the axial skeleton, a mesoderm-derived structure that is involved in the process of neurulation and embryonic development. Chordomas...
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| Format: | Article |
| Language: | English |
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Termedia Publishing House
2017-12-01
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| Series: | Contemporary Oncology |
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| Online Access: | https://www.termedia.pl/Immunophenotypic-features-of-dedifferentiated-skull-base-chordoma-An-insight-into-the-intratumoural-heterogeneity,3,31345,1,1.html |
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| author | Kelvin Manuel Pińa Batista Kenia Yoelvi Alvarez Reyes Fátima Pérez Lopez Andrés Coca Pelaz Ivan Fernandez Vega José Luis Llorente Pendás Antonio Saiz Ayala Aurora Astudillo Jorge Andrés Nuńez Rojas Patricia Barrio Fernandez |
| author_facet | Kelvin Manuel Pińa Batista Kenia Yoelvi Alvarez Reyes Fátima Pérez Lopez Andrés Coca Pelaz Ivan Fernandez Vega José Luis Llorente Pendás Antonio Saiz Ayala Aurora Astudillo Jorge Andrés Nuńez Rojas Patricia Barrio Fernandez |
| author_sort | Kelvin Manuel Pińa Batista |
| collection | DOAJ |
| description | Chordomas are rare and low-grade malignant solid tumours, despite their histologically benign appearance, that arise in the bone from embryonic notochordal vestiges of the axial skeleton, a mesoderm-derived structure that is involved in the process of neurulation and embryonic development. Chordomas occurring in the skull base tend to arise in the basiocciput along the clivus. Three major morphological variants have been described (classical, chondroid, and atypical/dedifferentiated). The pathogenesis and molecular mechanisms involved in chordoma development remain uncertain. From a pathological standpoint, the microenvironment of a chordoma is heterogeneous, showing a dual epithelial-mesenchymal differentiation. These tumours are characterised by slow modality of biologic growth, local recurrence, low incidence of metastasis rates, and cancer stem cell (CSC) phenotype. The main molecular findings are connected with brachyury immunoexpression and activation of the downstream Akt and mTOR signalling pathways. The differentiation between typical and atypical chordomas is relevant because the tumoural microenvironment and prognosis are partially different. This review provides an insight into the recent and relevant concepts and histochemical markers expressed in chordomas, with special emphasis on dedifferentiated chordomas and their prognostic implications. |
| format | Article |
| id | doaj-art-18f46f9f67304d5aab797e7c7d0d151a |
| institution | OA Journals |
| issn | 1428-2526 1897-4309 |
| language | English |
| publishDate | 2017-12-01 |
| publisher | Termedia Publishing House |
| record_format | Article |
| series | Contemporary Oncology |
| spelling | doaj-art-18f46f9f67304d5aab797e7c7d0d151a2025-08-20T02:18:40ZengTermedia Publishing HouseContemporary Oncology1428-25261897-43092017-12-0121426727310.5114/wo.2017.7238531345Immunophenotypic features of dedifferentiated skull base chordoma: An insight into the intratumoural heterogeneityKelvin Manuel Pińa BatistaKenia Yoelvi Alvarez ReyesFátima Pérez LopezAndrés Coca PelazIvan Fernandez VegaJosé Luis Llorente PendásAntonio Saiz AyalaAurora AstudilloJorge Andrés Nuńez RojasPatricia Barrio FernandezChordomas are rare and low-grade malignant solid tumours, despite their histologically benign appearance, that arise in the bone from embryonic notochordal vestiges of the axial skeleton, a mesoderm-derived structure that is involved in the process of neurulation and embryonic development. Chordomas occurring in the skull base tend to arise in the basiocciput along the clivus. Three major morphological variants have been described (classical, chondroid, and atypical/dedifferentiated). The pathogenesis and molecular mechanisms involved in chordoma development remain uncertain. From a pathological standpoint, the microenvironment of a chordoma is heterogeneous, showing a dual epithelial-mesenchymal differentiation. These tumours are characterised by slow modality of biologic growth, local recurrence, low incidence of metastasis rates, and cancer stem cell (CSC) phenotype. The main molecular findings are connected with brachyury immunoexpression and activation of the downstream Akt and mTOR signalling pathways. The differentiation between typical and atypical chordomas is relevant because the tumoural microenvironment and prognosis are partially different. This review provides an insight into the recent and relevant concepts and histochemical markers expressed in chordomas, with special emphasis on dedifferentiated chordomas and their prognostic implications.https://www.termedia.pl/Immunophenotypic-features-of-dedifferentiated-skull-base-chordoma-An-insight-into-the-intratumoural-heterogeneity,3,31345,1,1.htmldedifferentiated chordoma skull base immunophenotypic tumor |
| spellingShingle | Kelvin Manuel Pińa Batista Kenia Yoelvi Alvarez Reyes Fátima Pérez Lopez Andrés Coca Pelaz Ivan Fernandez Vega José Luis Llorente Pendás Antonio Saiz Ayala Aurora Astudillo Jorge Andrés Nuńez Rojas Patricia Barrio Fernandez Immunophenotypic features of dedifferentiated skull base chordoma: An insight into the intratumoural heterogeneity Contemporary Oncology dedifferentiated chordoma skull base immunophenotypic tumor |
| title | Immunophenotypic features of dedifferentiated skull base chordoma: An insight into the intratumoural heterogeneity |
| title_full | Immunophenotypic features of dedifferentiated skull base chordoma: An insight into the intratumoural heterogeneity |
| title_fullStr | Immunophenotypic features of dedifferentiated skull base chordoma: An insight into the intratumoural heterogeneity |
| title_full_unstemmed | Immunophenotypic features of dedifferentiated skull base chordoma: An insight into the intratumoural heterogeneity |
| title_short | Immunophenotypic features of dedifferentiated skull base chordoma: An insight into the intratumoural heterogeneity |
| title_sort | immunophenotypic features of dedifferentiated skull base chordoma an insight into the intratumoural heterogeneity |
| topic | dedifferentiated chordoma skull base immunophenotypic tumor |
| url | https://www.termedia.pl/Immunophenotypic-features-of-dedifferentiated-skull-base-chordoma-An-insight-into-the-intratumoural-heterogeneity,3,31345,1,1.html |
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