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Weekly Chest CasesArchive of Old Cases

Case No : 979 Date 2016-08-01

  • Courtesy of Yeun Jeong Kim, Sang Hyun Paik / SoonChunHyang University Bucheon Hospital
  • Age/Sex 33 / F
  • Chief ComplaintSeizure
  • Figure 1
  • Figure 2
  • Figure 3

Diagnosis With Brief Discussion

Diagnosis
Multifocal micronodular pneumocyte hyperplasia (MMPH) and lymphangioleiomyomatosis in patient with tuberous sclerosis
Radiologic Findings
Chest radiograph demonstrates diffuse, ill-defined, nodular opacities in both lungs. Computed tomography of the chest shows sharply and poorly defined small nodules, varying in diameter from 1 to 5 mm, and smooth thin-walled cysts diffusely scattered in both lungs.
Brief Review
MMPH is a rare benign hamartomatous proliferation of type II pneumocytes, often associated with tuberous sclerosis complex (TSC) and/or lymphangioleiomyomatosis (LAM). Most patients with MMPH in TSC are associated with LAM. Tuberin, the TSC2 gene product, has been recently found to be expressed in LAM and MMPH. The diagnosis of MMPH is confirmed by pathology, which will show proliferation of type II pneumocytes without the typical nuclear atypia and aggregations of macrophages in the alveolar lumens.
Characteristic CT features of MMPH are multiple tiny nodules (1-8 mm in diameter), which are diffusely scattered in a random distribution. Differential diagnoses include multiple atypical adenomatous hyperplasia, early pulmonary Langerhans cell histiocytosis, minute hematogeneous lung metastases, and miliary tuberculosis.
References
1. Shigeaki Umeoka, Takashi Koyama, Yukio Miki, Mikio Akai, Kazushige Tsutsui, Kaori Togashi, Pictorial Review of Tuberous Sclerosis in Various Organs. Radiographics 2008; 28:e32
2. Maruyama H, Ohbayashi C, Hino O, Tsutsumi M, Konishi Y. Pathogenesis of multifocal micronodular pneumocyte hyperplasia and lymphangioleiomyomatosis in tuberous sclerosis and association with tuberous sclerosis genes TSC1 and TSC2. Pathol Int 2001; 51: 585–94.
3. Ristagno RL, Biddinger PW, Pina EM, Meyer CA. Multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis. AJR Am J Roentgenol. 2005;184:S37-S39.
Keywords
Lung, Interstitial lung disease, ILD,

No. of Applicants : 95

▶ Correct Answer : 59/95,  62.1%
  • - Maternity & child hospital , Saudi Arabia Sania ALBlushi
  • - university of montreal , Canada Andrei Gorgos I
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - The University of Tokyo Hospital , Japan Toshihiro Furuta
  • - nizam , India amit patel
  • - The University of Tokyo Hospital , Japan Yusuke Watanabe
  • - Fortis hospital , Mohali , India Shaleen Rana
  • - Saitama-Sekishinkai Hosptal , Japan Mihoko Yamazaki
  • - radiologist, aditya imaging centre , India vivek patel
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Narayana Multispeciality Hospital Jaipur Raj , India JAINENDRA JAIN
  • - The first affiliated Hospital of Xiamen University , China Qing Qiang Guo
  • - Maimonides Medical Center , United States naomi twersky
  • - MBAL BURGAS , Bulgaria VLADISLAV RUSINOV
  • - Government Medical College, Nagpur, India , India Krishna Prasad Bellam
  • - Samsung Medical Center , Korea (South) Boda Nam
  • - Otsu Municipal Hospital , Japan Noriatsu Ichiba
  • - University of Tsukuba, Dept of Radiology , Japan Manabu Minami
  • - Kizawa Memorial Hospital , Japan Tomohiro Ando
  • - McGill University Health Center , Canada Alexandre Semionov
  • - Northern Yokohama Showa university , Japan Kota Watanabe
  • - The University of Tokyo Hospital , Japan Kaoru Sumida
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Scans world , India Philson Mukkada
  • - Kyungpook national university hospital , Korea (South) Sung Jun Moon
  • - Apollo Victor Hospitals - Goa INDIA , India Paresh Desai
  • - NIMS, HYDERABAD , India bhaskar kakarla
  • - Asan Medical Center , Korea (South) Hyung Jung Koo
  • - PGIMER , Chandigarh , India Karthik Rayasam
  • - Asan Medical Center , Korea (South) Chan Ho Park
  • - Ulsan University Hospital , Korea (South) Soyeoun Lim
  • - Shiga University of Medical Science , Japan Akitoshi Inoue
  • - GHICL , France manuel toledano
  • - Yan Bian University hospital , China ZHANG YING
  • - Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
  • - Korea University Guro Hospital , Korea (South) Sun Hong
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Korea University Guro Hospital , Korea (South) Bit Na Park
  • - okayama university , Japan numa shingo
  • - Santosh hospital,Bengaluru , India Pravin Mahadevappa
  • - Policlinico Umberto I , Italy Paolo Baldassari
  • - DAYA General Hospital,Thrissur,Kerala , India Raveendran TK
  • - QCH , Saudi Arabia Hani AlSalam
  • - University of Tsukuba Hospital , Japan Shioto Oda
  • - the first affiliatited hospital of nanjing medical univercity , China Hai Xu
  • - Private sector , Greece Vasilios Tzilas
  • - Seirei Hamamatsu General Hospital , Japan Kenichi Mizuki
  • - Kizawa Memorial Hospital , Japan Shoji Okuda
  • - Mallinckrodt Institute of Radiology , United States Naganathan Mani
  • - Centre Imagerie de Fribourg , Switzerland Benoit RIZK
  • - DRRPGMC KANGRA AT TANDA , India Dinesh Sood
  • - Korea University Guro Hospital , Korea (South) Yoonmi Choi
  • - Department of Radiology, Seoul National University Bundang Hospital , Korea (South) Sung Hyun Yoon
  • - University of Tsukuba , Japan Toshitaka Ishiguro
  • - Kyung Hee University Hospital at Gangdong , Korea (South) Han Na Lee
  • - Nasaret Hospital , Korea (South) Hee Seok Choi
  • - Medicheck health care , Korea (South) Chae Lim
  • - Korea University Guro Hospital , Korea (South) Jeong Woo Kim
  • - Hyogo Prefectural Amagasaki General Medical Center , Japan Tomoaki Otani
▶ Correct Answer as Differential Diagnosis : 3/95,  3.2%
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - Kyoto University , Japan Akihiko Sakata
  • - Kanto Rosai Hospital , Japan Taku Tajima
▶ Semi-Correct Answer : 30/95,  31.6%
  • - SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OOKUBO
  • - ROKAF 10th Fighter Wing , Korea (South) Kun Woo Kang
  • - Aerospace medical group, Air Force Education and Training Command , Korea (South) Donghwan Kim
  • - Red Cross Society Himeji Hospital , Japan Soichiro Okamoto
  • - privato , Italy genchi enzo
  • - Seoul National University Hospital , Korea (South) Sunkyung Jeon
  • - SNUH , Korea (South) Juil Park
  • - Seoul National University Hospital , Korea (South) Hyoung-In Choi
  • - Dep. Radiology, Medical University Vienna , Austria Ursula Nemec
  • - PRINCE ALI KHAN HOSPITAL , India FAHAD LODHIA
  • - Tianjin medical university , India Mohammed Khalid
  • - , Korea (South)
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Shanghai Jiao Tong University Affiliated Sixth People萸 , China Jiayin Zhang
  • - Okayama University Hospital , Japan Toshiyuki Komaki
  • - Pusan national university hospital , Korea (South) Minkyu Kwak
  • - Osaka Rosai Hospital , Japan Hiromitsu Sumikawa
  • - Niigata University , Japan Atsushi Uehara
  • - Military service , Korea (South) Dong Jang
  • - �stanbul , Turkey Ayhan Yilmaz
  • - Kinki University Faculty of Medicine, , Japan Mitsuru Matsuki
  • - IRSA LA ROCHELLE , France, Metropolitan BIGOT
  • - CHU Poitiers , France CHAN paul
  • - istituti ospitalieri di cremona , Italy pietro sergio
  • - Les cedres St malo , France nicolas gautier
  • - Chungbuk national university , Korea (South) Yong Kim
  • - ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - Saitama Sekishinkai hospital , Japan Kazufumi Kimura
  • - CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
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