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A 22-year-old woman presented with intermittent blood-tinged sputum for about 3 years. She reported experiencing exertional dyspnoea and had no history of chronic disease, family history or smoking. On physical examination, mild coarse breathing sounds and wheezing on the upper sternum were auscultated. Lab results were non-specific.
Chest CT scan (figure 1A,B) revealed a heterogeneous enhancing endotracheal mass of approximately 1.9 cm at mid-trachea extending to the boundary of the right tracheal wall or through the tracheal wall but not invading adjacent structures. The bronchoscopy showed a tracheal mass characterised by a lobulated surface with hypervascularity (figure 1C). 2-Deoxy-2-[18F]-fluorodeoxyglucose (FDG) positron emission tomography (PET)-CT demonstrated mild to moderate uptake increase of FDG (figure 1D) and no definitive invasion to the neighbouring structures was observed on images (figure 1E). The tracheal mass was resected with a sufficient resection margin via uniportal video-assisted thoracic surgery and was reconstructed by end-to-end anastomosis.
Histological examination revealed a tumour composed of cells with basophilic granular cytoplasm and others with finely vacuolated cytoplasm. Immunohistochemical staining for CK, DOG-1 and SOX-10 confirmed the diagnosis of tracheal acinic cell carcinoma (ACC) (figure 1H,I).1 The resection margin was negative, and our patient underwent frequent follow-up without any adjuvant therapy. After 10 months, bronchoscopy and PET-CT imaging substantiated the absence of any recurrence and until now, there has been no reemergence for 2 years (figure 1F,G).
Primary tracheal ACC is extremely uncommon and typically presents in the form of an endobronchial mass that originates from the submucosal glands of the trachea.1 Until now, there is no defined staging classification for tracheal tumours that predicts recurrence or survival. On the basis of a frequently quoted staging classification for primary tracheal tumours, our patient was treated as early-stage or localised disease.2 Furthermore, histopathology revealed no reported risk factors for recurrence of ACCs of various salivary glands,3 of which the histopathological features are extremely similar to those of tracheal ACC.1 Thus, the patient received regular follow-ups without any adjuvant therapy. Meanwhile, reported cases of tracheal ACC rarely documented recurrences following tumour excision.1 4 However, considering the fact that our patient is the youngest ever reported and taking into account the documented risk of recurrence in ACCs of various salivary origin,4 the patient is undergoing vigilant thoracic image surveillance with bronchoscopy with the interval of 3–6 months.
Here, we report a very rare manifestation of ACC in the central airway. To the best of our knowledge, there have been only seven reported cases of tracheal ACC since 1982. The average age was approximately 50 years old and our patient was 22 years old being the youngest patient ever reported.1 4
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Footnotes
JHP and JSJ are joint first authors.
JHP and JSJ contributed equally.
Contributors JHP and JSJ wrote the manuscript and generated the figures. MJC interpreted the data and generated the figures. JHK performed the acquisition of the specimen, interpreted the data, and generated the figures. JSJ and YCL interpreted the data, classified the imaging data, generated the figures, designed the study, and directed the project. YCL, JSJ and JHP have explained and administered the form to the patient.
Funding This work was supported by the National Research Foundation of Korea (NRF), funded by the Korean government (MSIT) (No. RS-2024-00356349) and Special Operating Subsidy of Jeonbuk National University Industrial Cooperation Foundation. Funding was also received from Korea Health Technology R&D Project through Korea Health Industry Development Institute (KHIDI), funded by the Ministry of Health & Welfare, Republic of Korea (No. RS-2024-00440408). Bio&Medical Technology Development Program of the National Research Foundation (NRF) also supported this research, funded by the Korean government (MSIT) (No. RS-2023-00236157). Lastly, this paper was also supported by the Biomedical Research Institute fund at Jeonbuk National University Hospital.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.