
==== Front
Respirol Case Rep
Respirol Case Rep
10.1002/(ISSN)2051-3380
RCR2
Respirology Case Reports
2051-3380
John Wiley & Sons, Ltd Chichester, UK

10.1002/rcr2.70019
RCR270019
Clinical Image
Clinical Image
Subglottic adenoid cystic carcinoma associated with pulmonary Langerhans cell histiocytosis
ADENOID CYSTIC CARCINOMA AND PLCH
Siddiqui
Siddiqui Atif Saleem https://orcid.org/0009-0004-3399-6163
1 atifsaleem19@yahoo.com

1 Department of Medicine, Division of Pulmonary and Critical Care Medicine Houston Methodist Hospital Houston Texas USA
* Correspondence
Atif Saleem Siddiqui, Pulmonary and Critical Care Medicine, Houston Methodist Hospital, 6550 Fannin St., Suite 2321, Houston, TX 77030, USA.
Email: atifsaleem19@yahoo.com

12 9 2024
9 2024
12 9 10.1002/rcr2.v12.9 e7001922 8 2024
02 9 2024
© 2024 The Author(s). Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology.
https://creativecommons.org/licenses/by-nc/4.0/ This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.

Key message

Pulmonary Langerhans cell histiocytosis can be associated with subglottic adenoid cystic carcinoma.

We present a rare case of pulmonary Langerhans cell histiocytosis associated with subglottic adenoid cystic carcinoma.

adenoid cystic carcinoma
dyspnea
pulmonary Langerhans cell histiocytosis
source-schema-version-number2.0
cover-dateSeptember 2024
details-of-publishers-convertorConverter:WILEY_ML3GV2_TO_JATSPMC version:6.4.8 mode:remove_FC converted:12.09.2024
Siddiqui AS . Subglottic adenoid cystic carcinoma associated with pulmonary Langerhans cell histiocytosis. Respirology Case Reports. 2024;12 (9 ):e70019. 10.1002/rcr2.70019

Associate Editor: Simon A Joosten
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pmcCLINICAL IMAGE

A 76‐year‐old with medical history significant for tobacco use, Pulmonary Langerhans cell histiocytosis (PLCH) diagnosed by transbronchial lung biopsies 8 years ago with a positive CD1a marker on immunohistochemistry tests and chest computed tomography (CT). He was treated with smoking cessation. He presented to a pulmonology clinic with dyspnea for 8 months. Bronchoscopy was performed that showed large subglottic mass (Figure 1A). He was admitted to the intensive care unit after tracheostomy, subglottic mass laryngoscopy, micro suspension and laser therapy of subglottic mass. CT chest showed cystic lesions (Figure 1B). Histopathology showed features of adenoid cystic carcinoma characterized by infiltrative growth with solid nests and cribriform glands with prominent extracellular spaces with myxoid material. The tumour cells were relatively monotonous basaloid with hyperchromatic nuclei (Figure 1C) Immunohistochemistry showed positive staining of neoplastic basaloid cells with p63, vimentin, BCL2 and beta‐catenin, while neoplastic ductal cells were highlighted by EMA and CD117. The fluorescence in situ hybridization study was positive for MYB‐NFIB. PLCH have been associated with the diagnosis of lymphoma, 1 adenocarcinoma of the lung, 2 multiple myeloma and other solid tumours. However, subglottic adenoid cystic carcinoma association with PLCH has not been reported in literature. Smoking is a known risk factor for PLCH and has been rarely associated with adenoid cystic carcinoma, which may be the primary link between these two rare diseases. The second proposed mechanism could be a mutation in the MYB. MYB is required for the development of haematopoietic stem cells. PLCH is also being driven by translocation involving MYB. A mutation in MYB may be the link between these two rare diseases. The exact pathophysiological mechanism behind this association remains unknown.

FIGURE 1 (A) Bronchoscopy view showing a large subglottic mass with a near total obstruction of the upper airway. (B) CT chest showing numerous cystic lesions in the upper lobes of both lungs. (C) H and E stain ×200 illustrating the salient features of adenoid cystic carcinoma with cribriform pattern.

AUTHOR CONTRIBUTIONS

The author has contributed to the study conception, design, acquisition, analysis, interpretation of the data, and drafting of the manuscript and final version of the manuscript for publication.

CONFLICT OF INTEREST STATEMENT

None declared.

ETHICS STATEMENT

The author declares that appropriate written informed consent was obtained for the publication of this manuscript and accompanying images.

DATA AVAILABILITY STATEMENT

The data that support the findings of this study are available from the corresponding author upon reasonable request.
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REFERENCES

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