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. 2021 Mar 30;11:7170. doi: 10.1038/s41598-021-85999-5

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© The Author(s) 2021

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

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Representative HRCT images with ground-glass attenuation suggestive of lymphatic congestion and MFF. (A, B): A 53-year-old female with S-LAM. She was diagnosed as having LAM histopathologically at the age of 45 when she had a right pneumothorax. Subsequently, ground- glass attenuation was noted in the right upper lobe (A) which ameliorated spontaneously 3 months later (B). (C,D) A 48-year-old female with S-LAM. HRCT revealed a small number of cysts in lung parenchyma in which focal ground-glass attenuation and the thickening of interlobar septa were noted (C). These findings were suggestive of lymphatic congestion which exacerbated 3 months later (D). Lung biopsy confirmed the diagnosis of LAM. (E,F) Representative images of lymphatic congestion in the mediastinum. This patient was a 56-year-old female with S-LAM whose diagnosis was established histopathologically by biopsy of retroperitoneal tumors. HRCT images showed the pathognomonic cystic appearance with some large cysts due to advanced LAM disease. Note the mediastinal widening, especially of the area dorsal to the trachea (E). The image obtained with intravenous contrast material delineated low attenuated areas surrounded by soft tissue attenuation, suggesting the cystic dilatation of mediastinal lymphatics and/or cystic enlargement of the mediastinal lymph nodes (F). She had initiated a fat-restricted diet as sirolimus had not yet been approved. However, these mediastinal lesions had successfully shrunk while being on the fat-restricted diet. (G,H) A 63-year-old female with S-LAM. HRCT images showed MFF as focal low-attenuation areas in both the interatrial (arrowheads) and interventricular (arrows) septums, on both unenhanced (G) and enhanced (H) images.