Ensartinib in a primary pulmonary ALK-rearranged mesenchymal neoplasm harboring a novel HMBOX1::ALK fusion: a case report and literature review
Anaplastic lymphoma kinase (ALK) gene rearrangements have been increasingly detected in mesenchymal neoplasms. ALK-rearranged mesenchymal neoplasms occur mainly in superficial tissues but rarely in internal organs. Herein, we firstly report a primary lung lesion presenting as a rare ALK-rearranged m...
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Frontiers Media S.A.
2025-05-01
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| Series: | Frontiers in Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fmed.2025.1572632/full |
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| author | Qi Liang Xingyu Jiang Ziwei Zhou Yali Jiang Siqi Ni Tingyu Zhao Xiao Zhang Huanhuan Xu Qixing Gong Lingxiang Liu |
| author_facet | Qi Liang Xingyu Jiang Ziwei Zhou Yali Jiang Siqi Ni Tingyu Zhao Xiao Zhang Huanhuan Xu Qixing Gong Lingxiang Liu |
| author_sort | Qi Liang |
| collection | DOAJ |
| description | Anaplastic lymphoma kinase (ALK) gene rearrangements have been increasingly detected in mesenchymal neoplasms. ALK-rearranged mesenchymal neoplasms occur mainly in superficial tissues but rarely in internal organs. Herein, we firstly report a primary lung lesion presenting as a rare ALK-rearranged mesenchymal neoplasm. The patient diagnosed with primary pulmonary ALK-rearranged mesenchymal neoplasm (PPAMN) received ensartinib as postoperative adjuvant therapy, achieving a disease-free survival of 10 months. Continuation of ensartinib as first-line treatment enabled him to benefit from a partial response, with a progression-free survival of 11 months. Second next-generation sequencing (NGS) revealed elevated HMBOX1::ALK abundance along with secondary NF2 mutation. After local radiotherapy combined with ensartinib continuation, his disease was temporarily stable for 7 months. Unfortunately, this disease became uncontrolled with an overall survival (OS) of 34 months. This is the first case of ALK-rearranged mesenchymal neoplasm manifested as a primary lung lesion and a novel HMBOX1::ALK fusion was identified by NGS. The family of ALK-rearranged mesenchymal neoplasms is expanding and ensartinib could be a potential treatment option for patients with HMBOX1::ALK. Repeated biopsy and NGS detection are critical to guide treatment selection at disease progression. |
| format | Article |
| id | doaj-art-3dae10c2f0f74dc7bf226fb79ed6b357 |
| institution | DOAJ |
| issn | 2296-858X |
| language | English |
| publishDate | 2025-05-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Medicine |
| spelling | doaj-art-3dae10c2f0f74dc7bf226fb79ed6b3572025-08-20T03:07:28ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2025-05-011210.3389/fmed.2025.15726321572632Ensartinib in a primary pulmonary ALK-rearranged mesenchymal neoplasm harboring a novel HMBOX1::ALK fusion: a case report and literature reviewQi Liang0Xingyu Jiang1Ziwei Zhou2Yali Jiang3Siqi Ni4Tingyu Zhao5Xiao Zhang6Huanhuan Xu7Qixing Gong8Lingxiang Liu9Department of Oncology, The First Affiliated Hospital with Nanjing Medical University, Nanjing, ChinaDepartment of Oncology, The First Affiliated Hospital with Nanjing Medical University, Nanjing, ChinaDepartment of Nuclear Medicine, The First Affiliated Hospital with Nanjing Medical University, Nanjing, ChinaCentral Laboratory, The Friendship Hospital of Ili Kazakh Autonomous Prefecture, Ili & Jiangsu Joint Institute of Health, Yining, ChinaDepartment of Oncology, The First Affiliated Hospital with Nanjing Medical University, Nanjing, ChinaDepartment of Oncology, The First Affiliated Hospital with Nanjing Medical University, Nanjing, ChinaDepartment of Oncology, The First Affiliated Hospital with Nanjing Medical University, Nanjing, ChinaDepartment of Hematology and Oncology, Department of Geriatric Lung Cancer Research Laboratory, Jiangsu Province Geriatric Hospital, Nanjing, ChinaDepartment of Pathology, The First Affiliated Hospital with Nanjing Medical University, Nanjing, ChinaDepartment of Oncology, The First Affiliated Hospital with Nanjing Medical University, Nanjing, ChinaAnaplastic lymphoma kinase (ALK) gene rearrangements have been increasingly detected in mesenchymal neoplasms. ALK-rearranged mesenchymal neoplasms occur mainly in superficial tissues but rarely in internal organs. Herein, we firstly report a primary lung lesion presenting as a rare ALK-rearranged mesenchymal neoplasm. The patient diagnosed with primary pulmonary ALK-rearranged mesenchymal neoplasm (PPAMN) received ensartinib as postoperative adjuvant therapy, achieving a disease-free survival of 10 months. Continuation of ensartinib as first-line treatment enabled him to benefit from a partial response, with a progression-free survival of 11 months. Second next-generation sequencing (NGS) revealed elevated HMBOX1::ALK abundance along with secondary NF2 mutation. After local radiotherapy combined with ensartinib continuation, his disease was temporarily stable for 7 months. Unfortunately, this disease became uncontrolled with an overall survival (OS) of 34 months. This is the first case of ALK-rearranged mesenchymal neoplasm manifested as a primary lung lesion and a novel HMBOX1::ALK fusion was identified by NGS. The family of ALK-rearranged mesenchymal neoplasms is expanding and ensartinib could be a potential treatment option for patients with HMBOX1::ALK. Repeated biopsy and NGS detection are critical to guide treatment selection at disease progression.https://www.frontiersin.org/articles/10.3389/fmed.2025.1572632/fullanaplastic lymphoma kinase (ALK)homeobox containing 1 (HMBOX1)ALK-rearranged mesenchymal neoplasmensartinibcase report |
| spellingShingle | Qi Liang Xingyu Jiang Ziwei Zhou Yali Jiang Siqi Ni Tingyu Zhao Xiao Zhang Huanhuan Xu Qixing Gong Lingxiang Liu Ensartinib in a primary pulmonary ALK-rearranged mesenchymal neoplasm harboring a novel HMBOX1::ALK fusion: a case report and literature review Frontiers in Medicine anaplastic lymphoma kinase (ALK) homeobox containing 1 (HMBOX1) ALK-rearranged mesenchymal neoplasm ensartinib case report |
| title | Ensartinib in a primary pulmonary ALK-rearranged mesenchymal neoplasm harboring a novel HMBOX1::ALK fusion: a case report and literature review |
| title_full | Ensartinib in a primary pulmonary ALK-rearranged mesenchymal neoplasm harboring a novel HMBOX1::ALK fusion: a case report and literature review |
| title_fullStr | Ensartinib in a primary pulmonary ALK-rearranged mesenchymal neoplasm harboring a novel HMBOX1::ALK fusion: a case report and literature review |
| title_full_unstemmed | Ensartinib in a primary pulmonary ALK-rearranged mesenchymal neoplasm harboring a novel HMBOX1::ALK fusion: a case report and literature review |
| title_short | Ensartinib in a primary pulmonary ALK-rearranged mesenchymal neoplasm harboring a novel HMBOX1::ALK fusion: a case report and literature review |
| title_sort | ensartinib in a primary pulmonary alk rearranged mesenchymal neoplasm harboring a novel hmbox1 alk fusion a case report and literature review |
| topic | anaplastic lymphoma kinase (ALK) homeobox containing 1 (HMBOX1) ALK-rearranged mesenchymal neoplasm ensartinib case report |
| url | https://www.frontiersin.org/articles/10.3389/fmed.2025.1572632/full |
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