A Rare Case of Sporadic Lymphangioleiomyomatosis in a Male Patient Diagnosed Following Pneumothorax

We report a rare case of sporadic lymphangioleiomyomatosis (LAM) in a 51-year-old male patient presenting with pneumothorax. High-resolution CT revealed multiple thin-walled cysts throughout both lungs. A history of renal angiomyolipoma and histopathologic findings confirmed the diagnosis. Immunohistochemical staining was positive for HMB-45 and α-smooth muscle actin. This case underscores the importance of including sporadic LAM in the differential diagnosis of cystic lung disease in men, despite its rarity. Early radiologic recognition and tissue confirmation are essential for accurate diagnosis and appropriate management.

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Publication Details

Journal
Journal of Thoracic Imaging
Published
2026-03-31
DOI
https://doi.org/10.1097/rti.0000000000000883
Primary Topic
Tuberous Sclerosis Complex Research
Type
article
Field-Weighted Citation Impact
0.00
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article

A Rare Case of Sporadic Lymphangioleiomyomatosis in a Male Patient Diagnosed Following Pneumothorax

Sakashi Fujimori, Takeshi Fujii, Hironori Uruga, T. Masumoto et al.
Journal of Thoracic Imaging
Tuberous Sclerosis Complex Research
article

A Rare Case of Sporadic Lymphangioleiomyomatosis in a Male Patient Diagnosed Following Pneumothorax

Sakashi Fujimori, Takeshi Fujii, Hironori Uruga, T. Masumoto, Katsunori Oikado, Atsushi Miyamoto, Hiroshi Hirakawa, Satoshi Nakamura, Meiyo Tamaoka, Daisuke Ito
article en

Abstract

We report a rare case of sporadic lymphangioleiomyomatosis (LAM) in a 51-year-old male patient presenting with pneumothorax. High-resolution CT revealed multiple thin-walled cysts throughout both lungs. A history of renal angiomyolipoma and histopathologic findings confirmed the diagnosis. Immunohistochemical staining was positive for HMB-45 and α-smooth muscle actin. This case underscores the importance of including sporadic LAM in the differential diagnosis of cystic lung disease in men, despite its rarity. Early radiologic recognition and tissue confirmation are essential for accurate diagnosis and appropriate management.

Journal of Thoracic Imaging
Kitasato University Hospital (JP), Toranomon Hospital (JP)
Good health and well-being
Openalex Percentile: Top 25%
Tuberous Sclerosis Complex Research
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