NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report
Background: Neurotrophic tyrosine receptor kinase (NTRK) gene-fusion targeted molecules revolutionized the paradigm of treatment of a limited subgroup of cancers of various histologies. Entrectinib and larotrectinib obtained unprecedented response rates in patients with cancer harboring NTRK rearran...
| Main Authors: | , , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2023-10-01
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| Series: | Frontiers in Oncology |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fonc.2023.1252359/full |
| _version_ | 1797663271756496896 |
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| author | Emanuela Palmerini Giorgio Frega Marco Gambarotti Tommaso Frisoni Marilena Cesari Alberto Bazzocchi Marco Miceli Davide Maria Donati Davide Maria Donati Stefano Fanti Cristina Nanni Stefania Benini Alessandra Longhi Anna Paioli Andrea Marrari Rossella Hakim Alberto Righi Toni Ibrahim |
| author_facet | Emanuela Palmerini Giorgio Frega Marco Gambarotti Tommaso Frisoni Marilena Cesari Alberto Bazzocchi Marco Miceli Davide Maria Donati Davide Maria Donati Stefano Fanti Cristina Nanni Stefania Benini Alessandra Longhi Anna Paioli Andrea Marrari Rossella Hakim Alberto Righi Toni Ibrahim |
| author_sort | Emanuela Palmerini |
| collection | DOAJ |
| description | Background: Neurotrophic tyrosine receptor kinase (NTRK) gene-fusion targeted molecules revolutionized the paradigm of treatment of a limited subgroup of cancers of various histologies. Entrectinib and larotrectinib obtained unprecedented response rates in patients with cancer harboring NTRK rearrangements. This evidence recently led to the agnostic approval of these drugs, and evidence (confirmation) of their activity in a broader disease setting is emerging. Here, we report the case of a patient affected by EML4-NTRK3 rearranged undifferentiated spindle cell bone sarcoma treated with larotrectinib, and we argue (discuss about) the incidence and clinical presentation of NTRK gene-fusion positive bone sarcomas, the potential use of upfront treatment with NTRK inhibitors in neoadjuvant setting, and the role of a multidisciplinary tumor board. Despite the rarity of these rearrangements in patients with primitive bone sarcomas, the therapy with NTRK inhibitors represents a highly effective strategy to be pursued in selected cases even in neoadjuvant settings. The management of these very rare cancers should always be discussed in a multidisciplinary board of reference centers. |
| first_indexed | 2024-03-11T19:12:05Z |
| format | Article |
| id | doaj.art-65afba1c23ad453fa34d144e8c213b50 |
| institution | Directory Open Access Journal |
| issn | 2234-943X |
| language | English |
| last_indexed | 2024-03-11T19:12:05Z |
| publishDate | 2023-10-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Oncology |
| spelling | doaj.art-65afba1c23ad453fa34d144e8c213b502023-10-09T10:56:02ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2023-10-011310.3389/fonc.2023.12523591252359NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case reportEmanuela Palmerini0Giorgio Frega1Marco Gambarotti2Tommaso Frisoni3Marilena Cesari4Alberto Bazzocchi5Marco Miceli6Davide Maria Donati7Davide Maria Donati8Stefano Fanti9Cristina Nanni10Stefania Benini11Alessandra Longhi12Anna Paioli13Andrea Marrari14Rossella Hakim15Alberto Righi16Toni Ibrahim17Osteoncology, Soft Tissue and Bone Sarcomas, Innovative Therapy Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyOsteoncology, Soft Tissue and Bone Sarcomas, Innovative Therapy Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyDepartment of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyThird Orthopaedic Clinic and Traumatology, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyOsteoncology, Soft Tissue and Bone Sarcomas, Innovative Therapy Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyDiagnostic and Interventional Radiology, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyDiagnostic and Interventional Radiology, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyThird Orthopaedic Clinic and Traumatology, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyDepartment of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, ItalyIRCCS Azienda Ospedaliero-Universitaria di Bologna, Policlinico Di Sant’Orsola, Bologna, ItalyIRCCS Azienda Ospedaliero-Universitaria di Bologna, Policlinico Di Sant’Orsola, Bologna, ItalyDepartment of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyOsteoncology, Soft Tissue and Bone Sarcomas, Innovative Therapy Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyOsteoncology, Soft Tissue and Bone Sarcomas, Innovative Therapy Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyOsteoncology, Soft Tissue and Bone Sarcomas, Innovative Therapy Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyOsteoncology, Soft Tissue and Bone Sarcomas, Innovative Therapy Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyDepartment of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyOsteoncology, Soft Tissue and Bone Sarcomas, Innovative Therapy Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna, ItalyBackground: Neurotrophic tyrosine receptor kinase (NTRK) gene-fusion targeted molecules revolutionized the paradigm of treatment of a limited subgroup of cancers of various histologies. Entrectinib and larotrectinib obtained unprecedented response rates in patients with cancer harboring NTRK rearrangements. This evidence recently led to the agnostic approval of these drugs, and evidence (confirmation) of their activity in a broader disease setting is emerging. Here, we report the case of a patient affected by EML4-NTRK3 rearranged undifferentiated spindle cell bone sarcoma treated with larotrectinib, and we argue (discuss about) the incidence and clinical presentation of NTRK gene-fusion positive bone sarcomas, the potential use of upfront treatment with NTRK inhibitors in neoadjuvant setting, and the role of a multidisciplinary tumor board. Despite the rarity of these rearrangements in patients with primitive bone sarcomas, the therapy with NTRK inhibitors represents a highly effective strategy to be pursued in selected cases even in neoadjuvant settings. The management of these very rare cancers should always be discussed in a multidisciplinary board of reference centers.https://www.frontiersin.org/articles/10.3389/fonc.2023.1252359/fullneurotrophic tyrosine receptor kinaseundifferentiated spindle cell sarcomabone sarcomalarotrectinibentrectinibNTRK |
| spellingShingle | Emanuela Palmerini Giorgio Frega Marco Gambarotti Tommaso Frisoni Marilena Cesari Alberto Bazzocchi Marco Miceli Davide Maria Donati Davide Maria Donati Stefano Fanti Cristina Nanni Stefania Benini Alessandra Longhi Anna Paioli Andrea Marrari Rossella Hakim Alberto Righi Toni Ibrahim NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report Frontiers in Oncology neurotrophic tyrosine receptor kinase undifferentiated spindle cell sarcoma bone sarcoma larotrectinib entrectinib NTRK |
| title | NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report |
| title_full | NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report |
| title_fullStr | NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report |
| title_full_unstemmed | NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report |
| title_short | NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report |
| title_sort | ntrk rearranged sarcoma of the bone role for larotrectinib in the neoadjuvant setting of an ultra rare tumor a case report |
| topic | neurotrophic tyrosine receptor kinase undifferentiated spindle cell sarcoma bone sarcoma larotrectinib entrectinib NTRK |
| url | https://www.frontiersin.org/articles/10.3389/fonc.2023.1252359/full |
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