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Weekly Chest CasesArchive of Old Cases

Case No : 867 Date 2014-06-09

  • Courtesy of Hyun Jung Kim, Myung Hee Chung / Bucheon St. Mary's Hospital, The Catholic University of Korea
  • Age/Sex 50 / F
  • Chief ComplaintExertional dyspnea for several years
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Pulmonary lymphangioleiomyomatosis (LAM) with extrapulmonary lymphangioleiomyoma
Radiologic Findings
Chest CT shows diffuse thin-walled cysts in both lungs, sparing the upper posterior lung fields. Low density lesions with weak enhancement are revealed in the retrocrural and retroperitoneal regions. On FDG-PET, there is no appreciable FDG uptake by masses. Retrocrural lymphangioleiomyoma is identified after surgical resection, and pulmonary lesions by VATS biopsy.
Brief Review
Lymphangioleiomyomatosis (LAM) is a rare interstitial lung disease that affects women exclusively, typically during their reproductive years. LAM is characterized by the abnormal proliferation of smooth muscle cells (LAM cells) in the lungs, and thoracic and retroperitoneal lymphatics. The primary target of LAM is the chest. However, extrapulmonary LAM rarely occurs with or without subsequent involvement of the lung.
21% of pulmonary LAM patients had extrapulmonary lymphangioleiomyomas. Avila et al. observed a positive trend between the presence of lymphangioleiomyomas and the severity of lung disease, with the probability of lymphangioleiomyomas increasing severity of the lung disease (p=0.12).
Diurnal variation of lymphangioleiomyomas with normal morning pressure and increasing size of mass in the afternoon resulting from increased chyle during the day after meal and exercise provides useful radiographic features in distinguishing lymphangioleiomyomas (when it has thick and irregular wall, or solid), from lymphoma, solid lymphatic mass, and sarcoma.
References
1.Avil NA, Bechtle J, Dwyer AJ, Ferrans VJ, Moss J. Lymphangioleiomyomatosis: CT of Diurnal variation of lymphangioleiomyomas. Radiology 2001;221:415-421
2.Matsui K, Tatsuguchi A, Valencia J, Yu Zx, Bechtle J, Beasley MB, Avila N, Travis WD, Moss J, Ferrans VJ. Extrapulmonary lymphangioleiomyomatosis (LAM): clinicopathologic features in 22 cases. Hum Pathol. 2000;31(10):1242-1248
Keywords
Lung, Mediastinum, Interstitial lung disease, LAM, ILD,

No. of Applicants : 93

▶ Correct Answer : 60/93,  64.5%
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - Niigata City General Hospital , Japan Takao Kiguchi
  • - Avrasya Hospital , Turkey Murat Ulusoy
  • - DELTA CARE HOSPITAL, THANJAVUR, INDIA , India SIVARAJA SUBRAMANIAM
  • - , Korea (South) Choi Seul-Gi
  • - Pamukkale University, School of Medicine, Dept. of Radiology , Turkey Nevzat Karabulut
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Kyoto University , Japan Akihiko Sakata
  • - Khoo Teck Phuat Hospital Singapore , Singapore ashish chawla
  • - MBAL Chernomorska , Bulgaria VLADISLAV RUSINOV
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - university of montreal , Canada Andrei Gorgos I
  • - Kobe City Medical Center General Hospital , Japan Yasuhisa Kurata
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - Samsung medical center , Korea (South) Hyun Su Kim
  • - stanley medical college , India mahes waran
  • - National Hospital Organization Okinawa Hospital , Japan Yasuji Oshiro
  • - radiologist, aditya imaging centre , India vivek patel
  • - Pneumology GHSR , Reunion fabrice paganin
  • - Radiology Department, the First Affliated Hospital of Nanjing Medical University , China Qiguang Cheng
  • - Radiology University of Messina , Italy Michele Gaeta
  • - University of British Columbia , Canada Amr Ajlan
  • - IRSA La Rochelle , France Denis Chabassiere
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Seoul National University Hospital , Korea (South) Sang Min Lee
  • - Jichi Medical University Hospital , Japan Yuichiro Kawahara
  • - McGill University Health Center , Canada Alexandre Semionov
  • - Japanese national center hospital of neurology and psychiatry , Japan Kaoru Sumida
  • - National Taiwan University Hospital , Taiwan Li-Ta Keng
  • - Goa Medical College , India Paresh Desai
  • - NIMS, HYDERABAD , India bhaskar kakarla
  • - Government Medical College, Nagpur, India , India Krishna Prasad Bellam
  • - The University of Tokyo Hosipital , Japan Toshihiro Furuta
  • - HUEC , Brazil Diogo Pinheiro
  • - Kyung Hee University Hospital at Gangdong , Korea (South) Seong Jong Yun
  • - Ondokuz Mayis University , Turkey Cetin Celenk
  • - Seirei Hamamatsu General Hospital , Japan Kenichi Mizuki
  • - Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
  • - Ishikawa Matto Central Hospital , Japan Manabu Akimoto
  • - Soonchunhyang University Seoul Hospital , Korea (South) EUNSUN OH
  • - Nagasaki University Hospital, Department of Pathology , Japan TOMONORI TANAKA
  • - Kizawa Memorial Hospital , Japan Yo Kaneko
  • - Seoul National University Gangnam Center , Korea (South) Hua Sun Kim
  • - gthtm. chennai.tamilnadu. , India mani saravanan
  • - IRSA LA ROCHELLE , France, Metropolitan BIGOT
  • - Saint Malo , France jean-baptiste Noel
  • - Niigata University , Japan Atsushi Uehara
  • - ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
  • - CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
  • - Centre Imagerie de Fribourg , Switzerland Benoit Rizk
  • - CLCC Rennes , France nicolas gautier
  • - Royal Perth Hospital , Australia Yuranga Weerakkody
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - Chungbuk national university , Korea (South) Yong Hun Kim
  • - Oita University , Japan Haruka Sato
  • - Medicheck health care , Korea (South) Chae Lim
  • - Private sector , Greece Vasilios Tzilas
  • - Pneumologia Universitaria, Policlinico di Bari , Italy Mario Damiani
  • - The University of Tokyo Hospital , Japan Takana Yamakawa
▶ Correct Answer as Differential Diagnosis : 9/93,  9.7%
  • - ssims&rc , India akhila d
  • - The University of Tokyo Hospital , Japan Takeyuki Watadani
  • - GHICL , France manuel toledano
  • - chp st martin , France Mariotte benoit
  • - district tuberculosis centre,poonamallee , India gayathri ganesh
  • - Shiga University of Medical Science , Japan Akitoshi Inoue
  • - CHU Poitiers , France CHAN paul
  • - NASA SCANS , India RAKESH BHATIA
  • - Onomichi municipal hospital , Japan Toshiyuki Komaki
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