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Weekly Chest CasesArchive of Old Cases

Case No : 1337 Date 2023-06-05

  • Courtesy of Geewon Lee / Pusan National University Hospital
  • Age/Sex 38 / F
  • Chief ComplaintCC. Routine checkup. Past Medical History. Seizure
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Lymphangioleiomyomatosis (LAM) and multifocal micronodular pneumocyte hyperplasia (MMPH) in a patient with tuberous sclerosis (TS)
Radiologic Findings
Fig 1-3. Axial CT scans in lung window setting demonstrate multiple lung cysts and multiple tiny nodules in the lungs.
Fig 4. Axial T2-weighted MR image demonstrates multiple bilateral subependymal tubers along the walls of the lateral ventricles.
Fig 5. Axial CT scans in bone window setting show multiple sclerotic lesions within the spine.
The patient had history of seizures several times a year, ever since infancy.
Brief Review
Tuberous sclerosis (TS) is an autosomal dominant inherited neurocutaneous syndrome characterized by a variety of hamartomatous lesions in various organs. The presence of common manifestations include cortical or subependymal tubers, white matter abnormalities, cardiac rhabdomyoma, and renal AML. The lungs, digestive system, retroperitoneum, and bone, which can be less frequently involved, should also be evaluated in patients with TS.
Subependymal tubers are considered to be closely related to the neurologic manifestations of TS, including epilepsy, cognitive disability, and neurobehavioral abnormalities.
Pulmonary involvement of TS includes lymphangioleiomyomatosis (LAM) and multifocal micronodular pneumocyte hyperplasia (MMPH). Thin-section CT can demonstrate characteristic features of pulmonary LAM and can obviate lung biopsy. The typical CT finding is round, thin-walled cysts of variable size and contour. Generally, the distribution of the cysts is symmetric and uniform throughout the lungs. MMPH is a rare pulmonary disorder that can be associated with TS. It is characterized by multicentric, well-demarcated nodular proliferation of type II pneumocytes along alveolar septa. At thin-section CT, multiple tiny nodules (1–8 mm in diameter) are diffusely scattered throughout the lung in a random distribution
Osseous manifestations of TS include cystlike lesions, hyperostosis of the inner table of the calvaria, osteoblastic changes, periosteal new bone formation, and scoliosis. These osseous lesions can occur anywhere in bone, commonly in the calvaria, short tubular bones of the hand or foot, spine, and pelvis. In particular, Avila et al. reported that the presence of four or more sclerotic bone lesions at body CT differentiates patients with sporadic LAM from those with TS/LAM with high sensitivities and specificity.
References
1. Umeoka S. et al. Radiographics. 2008;28:e32 Pictorial Review of Tuberous Sclerosis in Various Organs.
2. Avila N. et al. Radiology 2010;254:851-7 CT of Sclerotic Bone Lesions: Imaging Features Differentiating Tuberous Sclerosis Complex with Lymphangioleiomyomatosis from Sporadic Lymphangioleiomyomatosis
Keywords

No. of Applicants : 104

▶ Correct Answer : 46/104,  44.2%
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan HIROAKI ARAKAWA
  • - , Japan KYOKO NAGAI
  • - Shiga University of Medical Science , Japan AKITOSHI INOUE
  • - McGill University Health Center , Canada ALEXANDRE SEMIONOV
  • - Kyoto City Hospital , Japan YUSUKE UTSUNOMIYA
  • - NIMS, HYDERABAD , India BHASKAR K
  • - Healthy Longevity Medical Center , Japan SHIN-ICHI CHO
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONGSU KANG
  • - Korea University Guro Hospital , Korea (South) YEJU KANG
  • - , Korea (South) JIN YOUNG LEE
  • - Fukuoka University , Japan SHINJI TSUDA
  • - Oita University, Faculty of Medicine , Japan FUMITO OKADA
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - Korea University Ansan Hospital , Korea (South) HYO YOUNG KIM
  • - Osaka University , Japan AKINORI HATA
  • - Ajou University Hospital , Korea (South) HYUNSEUNG LEE
  • - , Japan YUKIKO USUI
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Ajou University Hospital , Korea (South) MINJI KIM
  • - Ajou University Hospital , Korea (South) JAYOUNG MOON
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Hukuda memorial hospital , Japan SHINTARO KANO
  • - , Korea (South) KIM JUNG YOON
  • - Presbyterian Medical Center , Korea (South) HANLIM SONG
  • - Chungbuk National University Hospital , Korea (South) MIHYEON PARK
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Korea University Guro Hospital , Korea (South) LEE DAKYONG
  • - Osaka metropolitan university Hospital , Japan TATSUSHI OURA
  • - Chonnam National University Hospital , Korea (South) KIM SANG GYUN
  • - Korea University Anam Hospital , Korea (South) KYU-CHONG LEE
  • - Kyung-hee University Medical Center , Korea (South) HYE SUN RYU
  • - Kyoto University , Japan SHO KOYASU
  • - Hyogo Prefectural Kobe Children , Japan SHUHEI NORIMOTO
  • - Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
  • - Kantou Rousai Hospital , Japan KAORU SUMIDA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - The University of Tokyo Hospital , Japan HIROSHI TAKUMIDA
  • - Chonnam National University Hospital , Korea (South) SEUNG WAN KANG
  • - Shiga University of Medical Science , Japan RYO UEMURA
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
  • - Shaanxi Provincial Forest Industry Staff Hospital , China GUO JIAN WEI
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - , Korea (South) JIN WOO YOON
  • - Juntendo University , Japan YUTAKA IKENOUCHI
▶ Correct Answer as Differential Diagnosis : 3/104,  2.9%
  • - , Korea (South) KANGHWI LEE
  • - OITA UNIVERSITY , Japan FUMIKA ISHITOBI
  • - Toyota Kosei Hospital , Japan YUKI HAYASHI
▶ Semi-Correct Answer : 55/104,  52.9%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - , Korea (South) JOHN BAEK
  • - , Japan SUZUNE TSUKAMOTO
  • - The University of Tokyo Hospital , Japan JUN KANZAWA
  • - ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
  • - Ajou University Hospital , Korea (South) SUNG HYUN AN
  • - Chonnam National University Hwasun Hospital , Korea (South) SEONGWOO CHO
  • - The University of Tokyo , Japan MOTO NAKAYA
  • - Seoul National University Hospital , Korea (South) HYUNGIN PARK
  • - Chonnam National University Hwasun Hospital , Korea (South) DONGHUN HAN
  • - , China FANG YING GEN
  • - Secomedic Hospital , Japan FUMINORI MIYOSHI
  • - Inje University Pusan Paik Hospital , Korea (South) GANGWON JEONG
  • - , United Arab Emirates GAYATHRI GANAPATHY
  • - Samsung Medical Center , Korea (South) JUHYUN KIM
  • - , Korea (South) HEESOO WON
  • - The University of Tokyo Hospital , Japan WATARU GONOI
  • - Chonbuk National University Hospital , Korea (South) JUNGHWAN KIM
  • - Qatif Central Hospital , Saudi Arabia HANI MAKKY ALSALAM
  • - Ajou University Hospital , Korea (South) HAEIN LEE
  • - Shimada General Medical Center , Japan HAYATO NOZAWA
  • - Soonchunhyang University Hospital Bucheon , Korea (South) HYEJOO PARK
  • - Yamanashi Prefectural Central Hospital , Japan HIROAKI WATANABE
  • - Hallym University Medical Center , Korea (South) JUNGMIN LIM
  • - Myongji Hospital , Korea (South) HYOUNG YEOB KIM
  • - , Japan KENTARO KOTANI
  • - Pusan National University Yangsan Hospital , Korea (South) KUN HEE KIM
  • - Chonbuk National University Hospital , Korea (South) HYUNSOO BAE
  • - Kyoto University Hospital , Japan YASUHISA KURATA
  • - Ajou University Hospital , Korea (South) YOU NA KIM
  • - Other , Korea (South) CHAEHUN LIM
  • - Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
  • - Jichi Medical University, School of Medicine , Japan MITSURU MATSUKI
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - , Italy PASQUALE FAVIA
  • - Other , Korea (South) SEONG WON JANG
  • - Dokkyo Medical University , Japan HIROAKI ARAKAWA
  • - Chungbuk National University Hospital , Korea (South) PARK JUN HYEON
  • - Manipal hospital Hebbal, Bengaluru , India PRAVIN KUMAR M
  • - Seoul National University Hospital , Korea (South) SUNGHO HONG
  • - Paras hospital, Panchkula , India SHALEEN RANA
  • - Seoul National University Bundang Hospital , Korea (South) YOONAH DO
  • - Chonnam National University Hospital , Korea (South) SOMIN LEE
  • - Chonbuk National University Hospital , Korea (South) SUBIN LEE
  • - Hanyang University Hospital , Korea (South) SUNJIN RYU
  • - Nerimahikarigaoka hospital , Japan TAKANA HAYASHI
  • - Matsunami General Hospital , Japan TARO TAKEDA
  • - Sotiria Hospital, Athens , Greece VASILIOS TZILAS
  • - , Japan YUMI MAEHARA
  • - The Catholic University of Korea Yoeuido St. Mary , Korea (South) CHAWOONG JEON
  • - , Korea (South) HM PARK
  • - Kyoto university , Japan AKIHIKO SAKATA
  • - , Korea (South) HYEONGYU YOO
  • - Other , Korea (South) YONG HO JANG
  • - Jiangsu province hospital , China WANGJIAN ZHA
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