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PTEN R130Q Papillary Tumor of the Pineal Region (PTPR) with Chromosome 10 Loss Successfully Treated with Everolimus: A Case Report
Papillary tumors of the pineal region (PTPR) can be observed among adults with poor prognosis and high recurrence rates. Standards of therapy involve total surgical excision along with radiation therapy, with no promising prospects for primary adjuvant chemotherapy, as long-term treatment options ha...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8025816/ https://www.ncbi.nlm.nih.gov/pubmed/33804593 http://dx.doi.org/10.3390/curroncol28020121 |
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author | Assi, Hazem I. Kakati, Rasha T. Berro, Juliett Saikali, Ibrahim Youssef, Bassem Hourany, Roula Alameh, Ibrahim Tabbarah, Abeer Khoury, Jessica Darwish, Houssein Alame, Saada |
author_facet | Assi, Hazem I. Kakati, Rasha T. Berro, Juliett Saikali, Ibrahim Youssef, Bassem Hourany, Roula Alameh, Ibrahim Tabbarah, Abeer Khoury, Jessica Darwish, Houssein Alame, Saada |
author_sort | Assi, Hazem I. |
collection | PubMed |
description | Papillary tumors of the pineal region (PTPR) can be observed among adults with poor prognosis and high recurrence rates. Standards of therapy involve total surgical excision along with radiation therapy, with no promising prospects for primary adjuvant chemotherapy, as long-term treatment options have not been explored. Chromosome 10 loss is characteristic of PTPR, and PTEN gene alterations are frequently encountered in a wide range of human cancers and may be treated with mTORC1 inhibitors such as everolimus. In parallel, there are no reports of treating PTPR with everolimus alone as a monopharmacotherapy. We report the case of a patient diagnosed with PTPR (grade III) characterized by a PTEN R130Q alteration with chromosome 10 loss that was treated with everolimus pharmacotherapy alone, resulting in an asymptomatic course and tumor regression, a rare yet notable phenomenon not described in the literature so far with potential to alter the management approach to patients with PTPR. |
format | Online Article Text |
id | pubmed-8025816 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-80258162021-04-08 PTEN R130Q Papillary Tumor of the Pineal Region (PTPR) with Chromosome 10 Loss Successfully Treated with Everolimus: A Case Report Assi, Hazem I. Kakati, Rasha T. Berro, Juliett Saikali, Ibrahim Youssef, Bassem Hourany, Roula Alameh, Ibrahim Tabbarah, Abeer Khoury, Jessica Darwish, Houssein Alame, Saada Curr Oncol Case Report Papillary tumors of the pineal region (PTPR) can be observed among adults with poor prognosis and high recurrence rates. Standards of therapy involve total surgical excision along with radiation therapy, with no promising prospects for primary adjuvant chemotherapy, as long-term treatment options have not been explored. Chromosome 10 loss is characteristic of PTPR, and PTEN gene alterations are frequently encountered in a wide range of human cancers and may be treated with mTORC1 inhibitors such as everolimus. In parallel, there are no reports of treating PTPR with everolimus alone as a monopharmacotherapy. We report the case of a patient diagnosed with PTPR (grade III) characterized by a PTEN R130Q alteration with chromosome 10 loss that was treated with everolimus pharmacotherapy alone, resulting in an asymptomatic course and tumor regression, a rare yet notable phenomenon not described in the literature so far with potential to alter the management approach to patients with PTPR. MDPI 2021-03-20 /pmc/articles/PMC8025816/ /pubmed/33804593 http://dx.doi.org/10.3390/curroncol28020121 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) ). |
spellingShingle | Case Report Assi, Hazem I. Kakati, Rasha T. Berro, Juliett Saikali, Ibrahim Youssef, Bassem Hourany, Roula Alameh, Ibrahim Tabbarah, Abeer Khoury, Jessica Darwish, Houssein Alame, Saada PTEN R130Q Papillary Tumor of the Pineal Region (PTPR) with Chromosome 10 Loss Successfully Treated with Everolimus: A Case Report |
title | PTEN R130Q Papillary Tumor of the Pineal Region (PTPR) with Chromosome 10 Loss Successfully Treated with Everolimus: A Case Report |
title_full | PTEN R130Q Papillary Tumor of the Pineal Region (PTPR) with Chromosome 10 Loss Successfully Treated with Everolimus: A Case Report |
title_fullStr | PTEN R130Q Papillary Tumor of the Pineal Region (PTPR) with Chromosome 10 Loss Successfully Treated with Everolimus: A Case Report |
title_full_unstemmed | PTEN R130Q Papillary Tumor of the Pineal Region (PTPR) with Chromosome 10 Loss Successfully Treated with Everolimus: A Case Report |
title_short | PTEN R130Q Papillary Tumor of the Pineal Region (PTPR) with Chromosome 10 Loss Successfully Treated with Everolimus: A Case Report |
title_sort | pten r130q papillary tumor of the pineal region (ptpr) with chromosome 10 loss successfully treated with everolimus: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8025816/ https://www.ncbi.nlm.nih.gov/pubmed/33804593 http://dx.doi.org/10.3390/curroncol28020121 |
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