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Weekly Chest CasesArchive of Old Cases

Case No : 1368 Date 2024-01-09

  • Courtesy of Won Gi Jeong, Yun-Hyeon Kim / Chonnam National University Hospital and Chonnam National University Hwasun Hospital
  • Age/Sex 48 / F
  • Chief Complaintchest pain Past history: recurrent pneumothorax (two times) Family history: younger brother (nephrectomy due to renal cell carcinoma)
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Birt-Hogg-Dube syndrome
Radiologic Findings
Figure 1. Chest posteroanterior radiograph shows pneumothorax in the right hemithorax.
Figure 2-4. In axial HRCT scans, cysts of various sizes and irregular shapes are observed predominantly in the medial and lower lung zones of both lungs.
Brief Review
Birt-Hogg-Dubé syndrome is a rare genetic condition characterized by distinctive clinical and radiologic features.

Clinical Features
1. Skin lesions: The most noticeable clinical feature. These include fibrofolliculomas, trichodiscomas, and acrochordons, typically developing in the third decade of life. They are often found on the face, neck, and upper torso.
2. Renal tumors: An increased risk of renal tumors, including oncocytomas and hybrid oncocytic/chromophobe tumors. These are often multiple and bilateral.
3. Pulmonary cysts and spontaneous pneumothorax: Patients may have lung cysts, which increase the risk of spontaneous pneumothorax.
4. Other Neoplasms: There is an increased risk of other types of tumors, including colorectal and thyroid cancers.

Radiologic Findings
1. Chest CT: Radiologically, lung cysts are a key finding, predominantly located in the lower and medial zones of the lungs. They are of various sizes and irregularly shaped.
2. Abdominal Imaging: Renal tumors may be identified through ultrasound, CT, or MRI. These tumors often present as hybrid lesions with features of both oncocytomas and chromophobe renal cell carcinoma.
3. Dermatological Examination: Skin lesions can be observed and diagnosed using dermatoscopy, though biopsy is often required for definitive diagnosis.
References
1. Daccord, C., Good, J. M., Morren, M. A., Bonny, O., Hohl, D., & Lazor, R. (2020). Birt–hogg–dubé syndrome. European Respiratory Review, 29(157).
2. Lee, K. C., Kang, E. Y., Yong, H. S., Kim, C., Lee, K. Y., Hwang, S. H., & Oh, Y. W. (2019). A stepwise diagnostic approach to cystic lung diseases for radiologists. Korean journal of radiology, 20(9), 1368-1380.
Keywords

No. of Applicants : 111

▶ Correct Answer : 105/111,  94.6%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan SUZUNE TSUKAMOTO
  • - medical scanning , Japan HIROAKI ARAKAWA
  • - ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
  • - Showa General Hospital , Japan MASAFUMI KAIUME
  • - Shiga University of Medical Science , Japan AKITOSHI INOUE
  • - Kyoto University , Japan AKIHIKO SAKATA
  • - Affilitated Hospital of Jining Medical college , China JIANG SHENG HUA
  • - Chonnam National University Hwasun Hospital , Korea (South) SEONGWOO CHO
  • - McGill University Health Center , Canada ALEXANDRE SEMIONOV
  • - Oita university , Japan AYUMI KAMEI
  • - Kyoto City Hospital , Japan YUSUKE UTSUNOMIYA
  • - Chonnam National University Hospital , Korea (South) CHAEWON SONG
  • - NIMS, HYDERABAD , India BHASKAR K
  • - The University of Tokyo , Japan MOTO NAKAYA
  • - Chonnam National University Hospital , Korea (South) HYUNJIN KIM
  • - , Italy PAOLO BALDASSARI
  • - Healthy Longevity Medical Center , Japan SHIN-ICHI CHO
  • - Chonnam National University Hospital , Korea (South) JUNYEOL YANG
  • - Chonnam National University Hwasun Hospital , Korea (South) DONGHUN HAN
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONGSU KANG
  • - , China FANG YING GEN
  • - Cabinet Capricorne, clinique sainte-clotilde , Reunion FABIEN HO
  • - Secomedic Hospital , Japan FUMINORI MIYOSHI
  • - Oita University, Faculty of Medicine , Japan FUMITO OKADA
  • - , Korea (South) KANGHWI LEE
  • - The University of Tokyo Hospital , Japan WATARU GONOI
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - Samsung Medical Center , Korea (South) JAEMO KOO
  • - Korea University Ansan Hospital , Korea (South) HYO YOUNG KIM
  • - Osaka University , Japan AKINORI HATA
  • - Mie University , Japan HIKARI FUKUI
  • - , Korea (South) MINJI KIM
  • - Soonchunhyang University Hospital Bucheon , Korea (South) HYEJOO PARK
  • - , Japan YUKIKO USUI
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - , Japan YOSHIKI ISHII
  • - Yamanashi Prefectural Central Hospital , Japan HIROAKI WATANABE
  • - Inje University Pusan Paik Hospital , Korea (South) JIYEON HAN
  • - Ajou University Hospital , Korea (South) JAYOUNG MOON
  • - , Korea (South) JUN GU KANG
  • - Seoul National University Bundang Hospital , Korea (South) JOONHYUK LEE
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Nankai medical center , Japan NORITAKA KAMEI
  • - , Japan KENTARO KOTANI
  • - Kyoto University Hospital , Japan YASUHISA KURATA
  • - Other , Korea (South) WON HYEONG IM
  • - Other , Korea (South) MARI A
  • - Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
  • - , Japan SHUNJIRO NOGUCHI
  • - , Korea (South) HEONSEOK LEE
  • - Tokyo Medical and Dental University , Japan MIKIYA FUJII
  • - Chungbuk National University Hospital , Korea (South) MIHYEON PARK
  • - Jichi Medical University, School of Medicine , Japan MITSURU MATSUKI
  • - Mie university , Japan MIYUKO FUJITA
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Shiga General Hospital , Japan YUSAKU MORIBATA
  • - Centre Hospitalier Regional de Lanaudiere , Canada MATTHIEU STORME
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - Korea University Guro Hospital , Korea (South) LEE DAKYONG
  • - Osaka metropolitan university Hospital , Japan TATSUSHI OURA
  • - Mie university , Japan SHIKO OKABE
  • - GHICL , France MANUEL TOLEDANO
  • - Kyoto university , Japan HIROKAZU SAWAMURA
  • - Dokkyo Medical University , Japan HIROAKI ARAKAWA
  • - Manipal hospital Hebbal, Bengaluru , India PRAVIN KUMAR M
  • - Inje University Ilsan Paik Hospital , Korea (South) SOYEUN PARK
  • - Seoul National University Hospital , Korea (South) SUNGHO HONG
  • - Holy Family Hospital , India RAJESH GOTHI
  • - Others , India PURUSHOTHAMA RAO TUMMALA
  • - , China MING LU
  • - TB centre kasaragod. , India rikhy krishnan
  • - Chonnam National University Hospital , Korea (South) KIM SANG GYUN
  • - Korea University Anam Hospital , Korea (South) KYU-CHONG LEE
  • - Mie University Hospital , Japan KENSUKE DOMAE
  • - Kyoto University , Japan SHO KOYASU
  • - SUNY Downstate , United States SAMUEL REISMAN
  • - Hyogo Prefectural Kobe Children , Japan SHUHEI NORIMOTO
  • - Seoul National University Bundang Hospital , Korea (South) YOONAH DO
  • - Chonnam National University Hospital , Korea (South) SOMIN LEE
  • - Samsung Medical Center , Korea (South) YONGJAE KWON
  • - Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
  • - Fukuoka university , Japan KEISUKE SATO
  • - Kantou Rousai Hospital , Japan KAORU SUMIDA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Nerimahikarigaoka hospital , Japan TAKANA HAYASHI
  • - Matsunami General Hospital , Japan TARO TAKEDA
  • - Chonnam National University Hospital , Korea (South) SEUNG WAN KANG
  • - Gyeongsang National University Hospital , Korea (South) BYEONG JU KOO
  • - Sotiria Hospital, Athens , Greece VASILIOS TZILAS
  • - , Japan YUMI MAEHARA
  • - Shiga University of Medical Science , Japan RYO UEMURA
  • - College of Medicine, CHA University , Korea (South) PARK JONG WON
  • - Toyota Kosei Hospital , Japan YUKI HAYASHI
  • - The Catholic University of Korea Yoeuido St. Mary , Korea (South) CHAWOONG JEON
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Chonnam National University Hospital , Korea (South) JAEBONG YANG
  • - Other , Korea (South) YONG HO JANG
  • - , Korea (South) JIN WOO YOON
  • - Hanyang University Hospital , Korea (South) SEUNG JIN YOO
  • - , China LI GUO
  • - Juntendo University , Japan YUTAKA IKENOUCHI
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 1/111,  0.9%
  • - , Korea (South) JIN YOUNG LEE
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