Rapidly Growing <scp>ALK</scp> ‐Negative <scp>NTRK3</scp> ‐Positive Inflammatory Myofibroblastic Tumour of the Lung

Inflammatory myofibroblastic tumours (IMT) are rare mesenchymal neoplasms that account for less than 1% of all lung tumours. Although ~50%-60% of IMT cases are anaplastic lymphoma kinase (ALK)-positive, ALK-negative IMT cases present diagnostic challenges owing to diverse pathological features. Here, we report a rare case of a rapidly growing ALK-negative, neurotrophic tyrosine receptor kinase 3 (NTRK3)-positive pulmonary IMT in a 46-year-old man with a history of pulmonary tuberculosis. A 13-mm nodule detected during routine screening grew to 18 mm within 1 month. Complete surgical resection was performed by video-assisted thoracic surgery. Histopathological examination revealed spindle cell proliferation with inflammatory cell infiltration. Immunohistochemistry was negative for ALK; however, molecular analysis identified an ETV6-NTRK3 fusion gene. The patient remains disease-free at 9 months postoperatively. This case highlights the importance of comprehensive molecular testing in ALK-negative IMT and demonstrates that complete surgical resection can achieve excellent outcomes even in rapidly growing lesions.

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Publication Details

Journal
Respirology Case Reports
Published
2025-12-01
DOI
https://doi.org/10.1002/rcr2.70427
Primary Topic
IgG4-Related and Inflammatory Diseases
Type
article
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article

Rapidly Growing ALK ‐Negative NTRK3 ‐Positive Inflammatory Myofibroblastic Tumour of the Lung

F. Kojima, Takeshi Ushigusa, Torahiko Jinta, Shinsaku Kabemura et al.
Respirology Case Reports
IgG4-Related and Inflammatory Diseases
article

Rapidly Growing ALK ‐Negative NTRK3 ‐Positive Inflammatory Myofibroblastic Tumour of the Lung

F. Kojima, Takeshi Ushigusa, Torahiko Jinta, Shinsaku Kabemura, Yoshihiro Satō, Toru Bando
article en

Abstract

Inflammatory myofibroblastic tumours (IMT) are rare mesenchymal neoplasms that account for less than 1% of all lung tumours. Although ~50%-60% of IMT cases are anaplastic lymphoma kinase (ALK)-positive, ALK-negative IMT cases present diagnostic challenges owing to diverse pathological features. Here, we report a rare case of a rapidly growing ALK-negative, neurotrophic tyrosine receptor kinase 3 (NTRK3)-positive pulmonary IMT in a 46-year-old man with a history of pulmonary tuberculosis. A 13-mm nodule detected during routine screening grew to 18 mm within 1 month. Complete surgical resection was performed by video-assisted thoracic surgery. Histopathological examination revealed spindle cell proliferation with inflammatory cell infiltration. Immunohistochemistry was negative for ALK; however, molecular analysis identified an ETV6-NTRK3 fusion gene. The patient remains disease-free at 9 months postoperatively. This case highlights the importance of comprehensive molecular testing in ALK-negative IMT and demonstrates that complete surgical resection can achieve excellent outcomes even in rapidly growing lesions.

Respirology Case ReportsVol. 13(12)
St. Luke's International Hospital (JP)
Openalex Percentile: Top 44%
IgG4-Related and Inflammatory Diseases
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