Anaplastic lymphoma kinase-positive pulmonary inflammatory myofibroblastic tumour: a case report
Abstract Background Pulmonary inflammatory myofibroblastic tumour (IMT) is a rare condition that usually presents in young individuals and is associated with anaplastic lymphoma kinase (ALK)-translocation. Case presentation We report a case of an 18-year-old Caucasian man with ALK-translocated pulmo...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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BMC
2024-04-01
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| Series: | Journal of Medical Case Reports |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13256-024-04472-9 |
| _version_ | 1827284304464445440 |
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| author | Daniel Tong Julia Chisholm Brendan Madden Merina Ahmed |
| author_facet | Daniel Tong Julia Chisholm Brendan Madden Merina Ahmed |
| author_sort | Daniel Tong |
| collection | DOAJ |
| description | Abstract Background Pulmonary inflammatory myofibroblastic tumour (IMT) is a rare condition that usually presents in young individuals and is associated with anaplastic lymphoma kinase (ALK)-translocation. Case presentation We report a case of an 18-year-old Caucasian man with ALK-translocated pulmonary IMT treated with multimodality therapy. The patient presented with breathlessness and was found to have a collapsed left lung. Further investigations revealed an ALK-translocated pulmonary IMT. This is usually treated with an ALK-inhibitor but patient declined after discussing potential side-effects and had repeated rigid bronchoscopic interventions for local disease control. Due to persistent local recurrence, patient received radical external beam radiotherapy (EBRT) with pulse steroids, and one year later started on Ibuprofen, a non-steroidal anti-inflammatory agent (NSAID). Following multimodality treatment, he developed a complete response. He remains treatment-free for the past seven years. Eleven years on from his diagnosis, he remains in remission with a ECOG performance status of zero. Conclusions Achieving long-term local control in pulmonary IMT can be challenging. Multimodality treatment is sometimes needed but the overall outlook remains good. |
| first_indexed | 2024-04-24T09:53:30Z |
| format | Article |
| id | doaj.art-a5da198b110b49ed8bc2953aafdc2226 |
| institution | Directory Open Access Journal |
| issn | 1752-1947 |
| language | English |
| last_indexed | 2024-04-24T09:53:30Z |
| publishDate | 2024-04-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Medical Case Reports |
| spelling | doaj.art-a5da198b110b49ed8bc2953aafdc22262024-04-14T11:17:29ZengBMCJournal of Medical Case Reports1752-19472024-04-011811810.1186/s13256-024-04472-9Anaplastic lymphoma kinase-positive pulmonary inflammatory myofibroblastic tumour: a case reportDaniel Tong0Julia Chisholm1Brendan Madden2Merina Ahmed3Lung Unit, The Royal Marsden NHS Foundation TrustChildren and Young People’s Unit, Institute of Cancer Research, Royal Marsden HospitalDepartment of Cardiothoracic Medicine, St Georges HospitalLung Unit, The Royal Marsden NHS Foundation TrustAbstract Background Pulmonary inflammatory myofibroblastic tumour (IMT) is a rare condition that usually presents in young individuals and is associated with anaplastic lymphoma kinase (ALK)-translocation. Case presentation We report a case of an 18-year-old Caucasian man with ALK-translocated pulmonary IMT treated with multimodality therapy. The patient presented with breathlessness and was found to have a collapsed left lung. Further investigations revealed an ALK-translocated pulmonary IMT. This is usually treated with an ALK-inhibitor but patient declined after discussing potential side-effects and had repeated rigid bronchoscopic interventions for local disease control. Due to persistent local recurrence, patient received radical external beam radiotherapy (EBRT) with pulse steroids, and one year later started on Ibuprofen, a non-steroidal anti-inflammatory agent (NSAID). Following multimodality treatment, he developed a complete response. He remains treatment-free for the past seven years. Eleven years on from his diagnosis, he remains in remission with a ECOG performance status of zero. Conclusions Achieving long-term local control in pulmonary IMT can be challenging. Multimodality treatment is sometimes needed but the overall outlook remains good.https://doi.org/10.1186/s13256-024-04472-9Pulmonary inflammatory myofibroblastic tumourALKMultimodalityRadiotherapy and pulse steroidsLong-term remission |
| spellingShingle | Daniel Tong Julia Chisholm Brendan Madden Merina Ahmed Anaplastic lymphoma kinase-positive pulmonary inflammatory myofibroblastic tumour: a case report Journal of Medical Case Reports Pulmonary inflammatory myofibroblastic tumour ALK Multimodality Radiotherapy and pulse steroids Long-term remission |
| title | Anaplastic lymphoma kinase-positive pulmonary inflammatory myofibroblastic tumour: a case report |
| title_full | Anaplastic lymphoma kinase-positive pulmonary inflammatory myofibroblastic tumour: a case report |
| title_fullStr | Anaplastic lymphoma kinase-positive pulmonary inflammatory myofibroblastic tumour: a case report |
| title_full_unstemmed | Anaplastic lymphoma kinase-positive pulmonary inflammatory myofibroblastic tumour: a case report |
| title_short | Anaplastic lymphoma kinase-positive pulmonary inflammatory myofibroblastic tumour: a case report |
| title_sort | anaplastic lymphoma kinase positive pulmonary inflammatory myofibroblastic tumour a case report |
| topic | Pulmonary inflammatory myofibroblastic tumour ALK Multimodality Radiotherapy and pulse steroids Long-term remission |
| url | https://doi.org/10.1186/s13256-024-04472-9 |
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