Non-classical pulmonary exacerbation in cystic fibrosis revealing ALK-Translocated lung cancer: A case report
Lung cancer is uncommon among people with cystic fibrosis (pwCF). We describe the case of a 35-year-old man with mild, stable CF disease who presented with severe respiratory distress, systemic symptoms, elevated liver enzymes and hypereosinophilia along with a lung mass and pleural effusion. The pa...
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| Language: | English |
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Elsevier
2025-01-01
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| Series: | Respiratory Medicine Case Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2213007125000073 |
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| author | Mohamad Hadhud Johnathan Arnon Anat Hershko-Moshe Adi Hollander Noa Hurvitz-Lehmann Assaf Potruch Henny Azmanov Rottem Kuint Nurith Hiller Elie Picard Naama Sebbag-Sznajder Shira Leebhoff Michael Wilschanski Myriam Grunewald Liron Birimberg-Schwartz Malena Cohen-Cymberknoh |
| author_facet | Mohamad Hadhud Johnathan Arnon Anat Hershko-Moshe Adi Hollander Noa Hurvitz-Lehmann Assaf Potruch Henny Azmanov Rottem Kuint Nurith Hiller Elie Picard Naama Sebbag-Sznajder Shira Leebhoff Michael Wilschanski Myriam Grunewald Liron Birimberg-Schwartz Malena Cohen-Cymberknoh |
| author_sort | Mohamad Hadhud |
| collection | DOAJ |
| description | Lung cancer is uncommon among people with cystic fibrosis (pwCF). We describe the case of a 35-year-old man with mild, stable CF disease who presented with severe respiratory distress, systemic symptoms, elevated liver enzymes and hypereosinophilia along with a lung mass and pleural effusion. The patient was subsequently diagnosed with non-small cell lung carcinoma (NSCLC), featuring anaplastic lymphoma kinase (ALK) translocation. Following treatment with a targeted tyrosine kinase inhibitor (TKI) there was a rapid tumor regression, however, his dyspnea and hypoxemia subsequently worsened. A trial of Elexacaftor/Tezacaftor/Ivacaftor (ETI) led to significant clinical improvement and enhanced pulmonary function. In vitro testing using patient-derived intestinal organoids was performed in parallel and also demonstrated a significant response to ETI. The deterioration observed following the initiation of ALK inhibitor treatment and subsequent improvement with CFTR modulators suggest that ALK inhibitor therapy may potentially impair CFTR activity. A better understanding of the relationship between these pathways could provide valuable insights and contribute to the development of more effective and tailored treatment strategies for patients with coexisting conditions. To our knowledge, this is the first reported case of ALK-translocated lung cancer in a CF patient, underscoring the necessity for a high degree of clinical suspicion in atypical presentations of pulmonary exacerbation and potentially linking ALK-EML4 activation pathways, TKI therapy and CFTR. Care for pwCF with lung cancer requires a unique multi-disciplinary approach to optimize their complex multifactorial treatment. |
| format | Article |
| id | doaj-art-9a95f9bda5c549f8bee867d10f8231cf |
| institution | Kabale University |
| issn | 2213-0071 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Respiratory Medicine Case Reports |
| spelling | doaj-art-9a95f9bda5c549f8bee867d10f8231cf2025-02-09T05:00:23ZengElsevierRespiratory Medicine Case Reports2213-00712025-01-0153102171Non-classical pulmonary exacerbation in cystic fibrosis revealing ALK-Translocated lung cancer: A case reportMohamad Hadhud0Johnathan Arnon1Anat Hershko-Moshe2Adi Hollander3Noa Hurvitz-Lehmann4Assaf Potruch5Henny Azmanov6Rottem Kuint7Nurith Hiller8Elie Picard9Naama Sebbag-Sznajder10Shira Leebhoff11Michael Wilschanski12Myriam Grunewald13Liron Birimberg-Schwartz14Malena Cohen-Cymberknoh15Pediatric Pulmonary Unit and Cystic Fibrosis Center, Hadassah Medical Center, Jerusalem, Israel; Faculty of Medicine, Hebrew University of Jerusalem, IsraelFaculty of Medicine, Hebrew University of Jerusalem, Israel; Sharett Institute of Oncology, Hadassah Medical Center, Jerusalem, IsraelFaculty of Medicine, Hebrew University of Jerusalem, Israel; Internal Medicine Department, Hadassah Medical Center, Ein Karem, Jerusalem, IsraelFaculty of Medicine, Hebrew University of Jerusalem, Israel; Internal Medicine Department, Hadassah Medical Center, Ein Karem, Jerusalem, IsraelFaculty of Medicine, Hebrew University of Jerusalem, Israel; Internal Medicine Department, Hadassah Medical Center, Ein Karem, Jerusalem, IsraelFaculty of Medicine, Hebrew University of Jerusalem, Israel; Internal Medicine Department, Hadassah Medical Center, Ein Karem, Jerusalem, IsraelFaculty of Medicine, Hebrew University of Jerusalem, Israel; Institute of Pulmonology, Hadassah Medical Center, Jerusalem, IsraelFaculty of Medicine, Hebrew University of Jerusalem, Israel; Institute of Pulmonology, Hadassah Medical Center, Jerusalem, IsraelFaculty of Medicine, Hebrew University of Jerusalem, Israel; Department of Radiology, Hadassah Medical Center, Mount Scopus, Jerusalem, IsraelPediatric Pulmonary Unit, Shaare Zedek Medical Center, Jerusalem, IsraelHadassah Organoid Center, Hadassah Medical Center, Jerusalem, IsraelHadassah Organoid Center, Hadassah Medical Center, Jerusalem, IsraelFaculty of Medicine, Hebrew University of Jerusalem, Israel; Pediatric Gastroenterology Unit and Cystic Fibrosis Center, Hadassah Medical Center, Jerusalem, IsraelFaculty of Medicine, Hebrew University of Jerusalem, Israel; Hadassah Organoid Center, Hadassah Medical Center, Jerusalem, Israel; Department of Developmental Biology and Cancer Research, Hadassah Medical Center, Jerusalem, IsraelFaculty of Medicine, Hebrew University of Jerusalem, Israel; Hadassah Organoid Center, Hadassah Medical Center, Jerusalem, Israel; Pediatric Gastroenterology Unit and Cystic Fibrosis Center, Hadassah Medical Center, Jerusalem, IsraelPediatric Pulmonary Unit and Cystic Fibrosis Center, Hadassah Medical Center, Jerusalem, Israel; Faculty of Medicine, Hebrew University of Jerusalem, Israel; Corresponding author. Pediatric Pulmonary Unit and Cystic fibrosis Center, Hadassah Medical Center, POB 12000, Jerusalem, 9112001, Israel.Lung cancer is uncommon among people with cystic fibrosis (pwCF). We describe the case of a 35-year-old man with mild, stable CF disease who presented with severe respiratory distress, systemic symptoms, elevated liver enzymes and hypereosinophilia along with a lung mass and pleural effusion. The patient was subsequently diagnosed with non-small cell lung carcinoma (NSCLC), featuring anaplastic lymphoma kinase (ALK) translocation. Following treatment with a targeted tyrosine kinase inhibitor (TKI) there was a rapid tumor regression, however, his dyspnea and hypoxemia subsequently worsened. A trial of Elexacaftor/Tezacaftor/Ivacaftor (ETI) led to significant clinical improvement and enhanced pulmonary function. In vitro testing using patient-derived intestinal organoids was performed in parallel and also demonstrated a significant response to ETI. The deterioration observed following the initiation of ALK inhibitor treatment and subsequent improvement with CFTR modulators suggest that ALK inhibitor therapy may potentially impair CFTR activity. A better understanding of the relationship between these pathways could provide valuable insights and contribute to the development of more effective and tailored treatment strategies for patients with coexisting conditions. To our knowledge, this is the first reported case of ALK-translocated lung cancer in a CF patient, underscoring the necessity for a high degree of clinical suspicion in atypical presentations of pulmonary exacerbation and potentially linking ALK-EML4 activation pathways, TKI therapy and CFTR. Care for pwCF with lung cancer requires a unique multi-disciplinary approach to optimize their complex multifactorial treatment.http://www.sciencedirect.com/science/article/pii/S2213007125000073Non-small cell lung cancerElexacaftor/tezacaftor/ivacaftorCystic fibrosisALK translocationIntestinal organoids |
| spellingShingle | Mohamad Hadhud Johnathan Arnon Anat Hershko-Moshe Adi Hollander Noa Hurvitz-Lehmann Assaf Potruch Henny Azmanov Rottem Kuint Nurith Hiller Elie Picard Naama Sebbag-Sznajder Shira Leebhoff Michael Wilschanski Myriam Grunewald Liron Birimberg-Schwartz Malena Cohen-Cymberknoh Non-classical pulmonary exacerbation in cystic fibrosis revealing ALK-Translocated lung cancer: A case report Respiratory Medicine Case Reports Non-small cell lung cancer Elexacaftor/tezacaftor/ivacaftor Cystic fibrosis ALK translocation Intestinal organoids |
| title | Non-classical pulmonary exacerbation in cystic fibrosis revealing ALK-Translocated lung cancer: A case report |
| title_full | Non-classical pulmonary exacerbation in cystic fibrosis revealing ALK-Translocated lung cancer: A case report |
| title_fullStr | Non-classical pulmonary exacerbation in cystic fibrosis revealing ALK-Translocated lung cancer: A case report |
| title_full_unstemmed | Non-classical pulmonary exacerbation in cystic fibrosis revealing ALK-Translocated lung cancer: A case report |
| title_short | Non-classical pulmonary exacerbation in cystic fibrosis revealing ALK-Translocated lung cancer: A case report |
| title_sort | non classical pulmonary exacerbation in cystic fibrosis revealing alk translocated lung cancer a case report |
| topic | Non-small cell lung cancer Elexacaftor/tezacaftor/ivacaftor Cystic fibrosis ALK translocation Intestinal organoids |
| url | http://www.sciencedirect.com/science/article/pii/S2213007125000073 |
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