Spinal ependymoma in adults: from molecular advances to new treatment perspectives
Ependymomas are rare glial tumors with clinical and biological heterogeneity, categorized into supratentorial ependymoma, posterior fossa ependymoma, and spinal cord ependymoma, according to anatomical localization. Spinal ependymoma comprises four different types: spinal ependymoma, spinal ependymo...
| Main Authors: | , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2023-11-01
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| Series: | Frontiers in Oncology |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fonc.2023.1301179/full |
| _version_ | 1827644272268017664 |
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| author | Giulia Cerretti Federico Pessina Federico Pessina Enrico Franceschi Valeria Barresi Alessandro Salvalaggio Alessandro Salvalaggio Marta Padovan Renzo Manara Renzo Manara Vincenzo Di Nunno Beatrice Claudia Bono Beatrice Claudia Bono Giovanni Librizzi Mario Caccese Marta Scorsetti Marta Maccari Giuseppe Minniti Giuseppe Minniti Pierina Navarria Giuseppe Lombardi |
| author_facet | Giulia Cerretti Federico Pessina Federico Pessina Enrico Franceschi Valeria Barresi Alessandro Salvalaggio Alessandro Salvalaggio Marta Padovan Renzo Manara Renzo Manara Vincenzo Di Nunno Beatrice Claudia Bono Beatrice Claudia Bono Giovanni Librizzi Mario Caccese Marta Scorsetti Marta Maccari Giuseppe Minniti Giuseppe Minniti Pierina Navarria Giuseppe Lombardi |
| author_sort | Giulia Cerretti |
| collection | DOAJ |
| description | Ependymomas are rare glial tumors with clinical and biological heterogeneity, categorized into supratentorial ependymoma, posterior fossa ependymoma, and spinal cord ependymoma, according to anatomical localization. Spinal ependymoma comprises four different types: spinal ependymoma, spinal ependymoma MYCN-amplified, myxopapillary ependymoma, and subependymoma. The clinical onset largely depends on the spinal location of the tumor. Both non-specific and specific sensory and/or motor symptoms can be present. Owing to diverse features and the low incidence of spinal ependymomas, most of the current clinical management is derived from small retrospective studies, particularly in adults. Treatment involves primarily surgical resection, aiming at maximal safe resection. The use of radiotherapy remains controversial and the optimal dose has not been established; it is usually considered after subtotal resection for WHO grade 2 ependymoma and for WHO grade 3 ependymoma regardless of the extent of resection. There are limited systemic treatments available, with limited durable results and modest improvement in progression-free survival. Thus, chemotherapy is usually reserved for recurrent cases where resection and/or radiation is not feasible. Recently, a combination of temozolomide and lapatinib has shown modest results with a median progression-free survival (PFS) of 7.8 months in recurrent spinal ependymomas. Other studies have explored the use of temozolomide, platinum compounds, etoposide, and bevacizumab, but standard treatment options have not yet been defined. New treatment options with targeted treatments and immunotherapy are being investigated. Neurological and supportive care are crucial, even in the early stages. Post-surgical rehabilitation can improve the consequences of surgery and maintain a good quality of life, especially in young patients with long life expectancy. Here, we focus on the diagnosis and treatment recommendations for adults with spinal ependymoma, and discuss recent molecular advances and new treatment perspectives. |
| first_indexed | 2024-03-09T18:12:31Z |
| format | Article |
| id | doaj.art-2019bc976ff24d12812774f4c7f753ee |
| institution | Directory Open Access Journal |
| issn | 2234-943X |
| language | English |
| last_indexed | 2024-03-09T18:12:31Z |
| publishDate | 2023-11-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Oncology |
| spelling | doaj.art-2019bc976ff24d12812774f4c7f753ee2023-11-24T08:59:34ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2023-11-011310.3389/fonc.2023.13011791301179Spinal ependymoma in adults: from molecular advances to new treatment perspectivesGiulia Cerretti0Federico Pessina1Federico Pessina2Enrico Franceschi3Valeria Barresi4Alessandro Salvalaggio5Alessandro Salvalaggio6Marta Padovan7Renzo Manara8Renzo Manara9Vincenzo Di Nunno10Beatrice Claudia Bono11Beatrice Claudia Bono12Giovanni Librizzi13Mario Caccese14Marta Scorsetti15Marta Maccari16Giuseppe Minniti17Giuseppe Minniti18Pierina Navarria19Giuseppe Lombardi20Department of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Padua, ItalyDepartment of Neurosurgery, IRCCS Humanitas Research Hospital, Milan, ItalyDepartment of Biomedical Sciences, Humanitas University, Milan, ItalyNervous System Medical Oncology Department, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, ItalyDepartment of Diagnostics and Public Health, University of Verona, Verona, ItalyDepartment of Neuroscience, University of Padova, Padova, ItalyPadova Neuroscience Center (PNC), University of Padova, Padova, ItalyDepartment of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Padua, ItalyDepartment of Neuroscience, Azienda Ospedale-Università di Padova, Padua, ItalyDepartment of Medicine - DIMED, University of Padova, Padua, ItalyNervous System Medical Oncology Department, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, ItalyDepartment of Neurosurgery, IRCCS Humanitas Research Hospital, Milan, ItalyDepartment of Biomedical Sciences, Humanitas University, Milan, ItalyDepartment of Neuroscience, Azienda Ospedale-Università di Padova, Padua, ItalyDepartment of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Padua, Italy0Department of Radiotherapy and Radiosurgery, IRCCS Humanitas Research Hospital, Milan, ItalyDepartment of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Padua, Italy1Department of Radiological Sciences, Oncology and Anatomical Pathology, Sapienza University, Rome, Italy2IRCCS Neuromed, Pozzilli, Italy0Department of Radiotherapy and Radiosurgery, IRCCS Humanitas Research Hospital, Milan, ItalyDepartment of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Padua, ItalyEpendymomas are rare glial tumors with clinical and biological heterogeneity, categorized into supratentorial ependymoma, posterior fossa ependymoma, and spinal cord ependymoma, according to anatomical localization. Spinal ependymoma comprises four different types: spinal ependymoma, spinal ependymoma MYCN-amplified, myxopapillary ependymoma, and subependymoma. The clinical onset largely depends on the spinal location of the tumor. Both non-specific and specific sensory and/or motor symptoms can be present. Owing to diverse features and the low incidence of spinal ependymomas, most of the current clinical management is derived from small retrospective studies, particularly in adults. Treatment involves primarily surgical resection, aiming at maximal safe resection. The use of radiotherapy remains controversial and the optimal dose has not been established; it is usually considered after subtotal resection for WHO grade 2 ependymoma and for WHO grade 3 ependymoma regardless of the extent of resection. There are limited systemic treatments available, with limited durable results and modest improvement in progression-free survival. Thus, chemotherapy is usually reserved for recurrent cases where resection and/or radiation is not feasible. Recently, a combination of temozolomide and lapatinib has shown modest results with a median progression-free survival (PFS) of 7.8 months in recurrent spinal ependymomas. Other studies have explored the use of temozolomide, platinum compounds, etoposide, and bevacizumab, but standard treatment options have not yet been defined. New treatment options with targeted treatments and immunotherapy are being investigated. Neurological and supportive care are crucial, even in the early stages. Post-surgical rehabilitation can improve the consequences of surgery and maintain a good quality of life, especially in young patients with long life expectancy. Here, we focus on the diagnosis and treatment recommendations for adults with spinal ependymoma, and discuss recent molecular advances and new treatment perspectives.https://www.frontiersin.org/articles/10.3389/fonc.2023.1301179/fullependymomaspinal ependymomaradiotherapychemotherapytemozolomidelapatinib |
| spellingShingle | Giulia Cerretti Federico Pessina Federico Pessina Enrico Franceschi Valeria Barresi Alessandro Salvalaggio Alessandro Salvalaggio Marta Padovan Renzo Manara Renzo Manara Vincenzo Di Nunno Beatrice Claudia Bono Beatrice Claudia Bono Giovanni Librizzi Mario Caccese Marta Scorsetti Marta Maccari Giuseppe Minniti Giuseppe Minniti Pierina Navarria Giuseppe Lombardi Spinal ependymoma in adults: from molecular advances to new treatment perspectives Frontiers in Oncology ependymoma spinal ependymoma radiotherapy chemotherapy temozolomide lapatinib |
| title | Spinal ependymoma in adults: from molecular advances to new treatment perspectives |
| title_full | Spinal ependymoma in adults: from molecular advances to new treatment perspectives |
| title_fullStr | Spinal ependymoma in adults: from molecular advances to new treatment perspectives |
| title_full_unstemmed | Spinal ependymoma in adults: from molecular advances to new treatment perspectives |
| title_short | Spinal ependymoma in adults: from molecular advances to new treatment perspectives |
| title_sort | spinal ependymoma in adults from molecular advances to new treatment perspectives |
| topic | ependymoma spinal ependymoma radiotherapy chemotherapy temozolomide lapatinib |
| url | https://www.frontiersin.org/articles/10.3389/fonc.2023.1301179/full |
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