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Case Reports
. 2025 Jul 19:57:102263.
doi: 10.1016/j.rmcr.2025.102263. eCollection 2025.

From diagnostic biopsy to bullectomy: A case of progressive complications in pulmonary langerhans cell histiocytosis

Yuan-Chia Hsin  1 , Yung-Chia Huang  1 , Yen-Lin Wu  2 , Shih-Wei Lee  1 , Po-Chun Lo  1   3
Affiliations
Case Reports

From diagnostic biopsy to bullectomy: A case of progressive complications in pulmonary langerhans cell histiocytosis

Yuan-Chia Hsin et al. Respir Med Case Rep. .

Abstract

A 38-year-old male smoker presented with a persistent dry cough lasting several weeks. Chest radiography showed bilateral reticulonodular opacities predominantly in the upper lung fields. Subsequent chest computed tomography revealed diffuse, thick-walled, irregular cystic lesions mainly involving the mid and upper lung zones with a centrilobular distribution. Histopathological examination obtained through video-assisted thoracoscopic surgery demonstrated bronchiolocentric nodules and cysts containing Langerhans cells with convoluted nuclei, confirmed by positive immunohistochemical staining for CD1a, S-100, and Langerin. These findings confirmed the diagnosis of pulmonary Langerhans Cell Histiocytosis (PLCH). The patient's symptoms improved initially following smoking cessation but were complicated by recurrent pneumothorax and the formation of giant bullae, ultimately requiring surgical intervention. This case emphasizes the association between PLCH and smoking, highlights potential complications following surgical biopsy, and underscores smoking cessation as a critical therapeutic measure, with targeted therapies reserved for refractory or mutation-specific cases.

Keywords: Asian; Case report; Positron emission tomography; SYT-SSX mutation; Synovial sarcoma; Video-assisted thoracic surgery.

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Conflict of interest statement

None.

Figures

Fig. 1
Fig. 1
Posterior-anterior chest radiography shows diffused reticulation and micro nodules. No pneumothorax is present. The lower lung field is relatively spared.
Fig. 2
Fig. 2
Chest HRCT showed diffuse nodules with central cavitation and cystic formation.
Fig. 3
Fig. 3
The pathology section showed lung tissue with bronchiogenic nodules and cysts of Langerhans cells with convoluted nuclei. (A) The Langerhans cells are positive for CD1a, S-100, and langerin. (B) The diagnosis of PLCH is confirmed.
Fig. 4
Fig. 4
Evolution of the patient's chest radiography. The HRCT was obtained at the initial clinic visit. (A) One month after the initial HRCT and the surgical biopsy. Pneumothorax can be appreciated from the right lung (B). The granulomatous lesion with cavitation evolved into cystic lesions within two months, accompanied by bullae formation at the apical lung. (C) The patient does not achieve spontaneous remission despite smoking cessation.
See this image and copyright information in PMC

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