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Article in Japanese
A case of lymphangioleiomyomatosis with atypical radiological features and slow progression
Midori Sugisakia,* Tatsuji Enomotoa,# Tetsuya Yokosukab Takuo Hayashic Ryota Ushioa,† Toshiko Kobayashib Shinji Abea
aDepartment of Respiratory Medicine, Tokyo Metropolitan Hiroo Hospital
bDepartment of Surgery, Tokyo Metropolitan Hiroo Hospital
cDepartment of Human Pathology, Juntendo University School of Medicine
*Present address: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center
#Present address: Department of Respiratory Medicine, Ofuna Chuo Hospital
†Present address: Department of Respiratory Medicine, Yokohama City University Hospital
A 37-year-old female was admitted because of recurrent pneumothorax. Chest CT showed right pneumothorax and 5 thin-wall cysts 2.0-3.5 cm in diameter. These findings had been almost stable for one year. A histopathological examination revealed typical findings of lymphangioleiomyomatosis (LAM) with an atypical area that showed a few lymphatic vessels and prominent capillaries and was also negative for α-SMA and VEGF-D. This case is thought to be a rare LAM lacking in lymphangiogenesis.
Lymphangioleiomyomatosis Pneumothorax Lung cyst Lymphangiogenesis
Received 6 Aug 2013 / Accepted 18 Nov 2013
AJRS, 3(2): 251-254, 2014