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Weekly Chest CasesArchive of Old Cases

Case No : 543 Date 2008-03-24

  • Courtesy of Ho Yun Lee, MD, Chin A Yi, MD. Kyung Soo Lee, MD. / Samsung Medical Center, Korea
  • Age/Sex 22 / F
  • Chief ComplaintRecurrent spontaneous pneumothorax
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Tuberous Sclerosis Complex with Lymphangioleiomyomatosis
Radiologic Findings
Fig 1. Chest PA shows diffuse reticular markings in both lungs and small amount of pneumothorax is seen in right hemithorax.
Fig 2. Chest CT image shows innumerable thin-walled cysts and small nodules in both lungs without sparing any specific lung regions.
Fig 3,4. Abdomen CT images show fat containing renal masses and fatty hepatic nodule.
Brief Review
Lymphangioleiomyomatosis (LAM) is characterized by proliferation of abnormal appearing smooth muscle cells (LAM cells) in the lungs, the kidneys, and the lymphatic system. Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by hamartomas, seizures, and mental retardation. TSC is caused by mutation of two genes, TSC1 or TSC2; its occurrence can be sporadic (approximately 60% of cases) or inherited. Up to a third of women with TSC may exhibit lung cysts, the hallmark feature of LAM; these patients have been categorized as having TSC/LAM.

The same set of findings (lung cysts, renal and hepatic angiomyolipomas (AMLs) comprises the CT features of both sporadic LAM and TSC/LAM; however, the frequency and severity with which they occur differ between the two groups. Hepatic and renal AMLs and lung nodules are more common in TSC/LAM, while lymphatic involvement뾲horacic duct dilatation, chylous pleural effusion, and ascites뾦s more common in LAM. These results appear to be relatively independent of age in adult patients.

Noncalcified pulmonary nodules likely represent a multifocal micronodular pneumocyte hyperplasia, which was initially described in patients with TSC/LAM. These benign noncalcified pulmonary nodules range in size from 2 mm to 1 cm and are distributed randomly in the lungs; they are composed of thickened fibrotic alveolar septa lined by pleomorphic type II pneumocytes. The absence of immunohistochemical staining for HMB45 (stains smooth muscle cells, or "LAM cells," diagnostic for LAM) suggests a histogenesis separate from the lesions in LAM.

Assessment of the relation between these phenotypic differences and patients' genetic data may provide insight into the genetic basis of LAM and TSC/LAM.
References
Nilo A. Avila, Andrew J. Dwyer, et al. Sporadic Lymphangioleiomyomatosis and Tuberous Sclerosis Complex with Lymphangioleiomyomatosis: Comparison of CT Features. Radiology 2007; 242: 277-285.
Keywords
Lung, Multiple organ, Interstitial lung disease, LAM, Tuberous Sclerosis, ILD,

No. of Applicants : 106

▶ Correct Answer : 96/106,  90.6%
  • - Daegu Fatima Hospital , Korea (South) Tae-Hun Kim
  • - Osaka University , Japan Osamu Honda
  • - Shiga University of Medical Science , Japan Norihisa Nitta
  • - Yonsei university Shinchon Severance hospital , Korea (South) Na-young Shin
  • - , Korea (South) ui byun
  • - Almana.G.Hospital , Saudi Arabia Jamal Abazid
  • - National Taiwan University Hospital , Taiwan chi-hsien chen
  • - Shinsegye Rad Clinic, Daegu, Korea , Korea (South) Gi Beom Kim
  • - Marien Hospital , Germany Davis Chiramel
  • - Kashan University of Medical Sciences, kashan, Iran , Iran Ebrahim Razi
  • - Apollo Hospital, Ahmedabad. , India Sandip Shah
  • - hospital Sao Paulo , Brazil israel missrie
  • - Assam medical college, INDIA , India karunakaran M
  • - Yonsei University college of medicine Severance hospital, Seoul, Korea , Korea (South) Hua Sun Kim
  • - Konkuk university hospital , Korea (South) Jeong Geun Yi
  • - Saboo Hospital , India sandeep saboo
  • - Department of Radiology, Kangbuk Samsung Hospital , Korea (South) Pyeong Guk Kang
  • - seoul national university hospital , Korea (South) Ijin Joo
  • - All India Institute of medical sciences , India Justin Moses
  • - Hopital universitaire Lausanne , Switzerland abed kourhani
  • - Saga University, Japan , Japan Ryoko Egashira
  • - Trakya Univercity School of Medicine , Turkey armagan sarac
  • - Trakya University School of Medicine , Turkey Derya Demir
  • - EKH-Berlin , Germany Michael Weber
  • - christie hopsital , United Kingdom rohit kochhar
  • - Louisiana State University - Shreveport , United States Carlos Previgliano
  • - Seoul National University Hospital , Korea (South) Hyun Ju Lee
  • - Az Osp Santa Maria , Italy angelo carloni
  • - suwon baiksung hospital , Korea (South) cheolyoung kim
  • - Auckland City Hospital , New Zealand (Aotearoa) Remy Lim
  • - Trakya University School of Medicine Deparment of Radiology , Turkey Devrim Urut
  • - National Taiwan University Hospital , Taiwan Chin-Chung Shu
  • - universidad de chile , Chile aguilera enrique
  • - SNUH , Korea (South) Mi-Jin Kang
  • - kyungpook national university hospital , Korea (South) Jae-kwang Lim
  • - hallym university sacred hospital , Korea (South) Kim Eun Soo
  • - , Korea (South) Kim hee jung
  • - Samsung Medical Center , Korea (South) Dae Kun Oh
  • - Kasturba Medical College Manipal , India Paresh Desai
  • - University of Tennessee, Memphis , United States XAVIER PACKIANATHAN
  • - Clinic 1 , Russian Federation Lepikhina Darya
  • - University Hospital of Antwerp , Belgium Rodrigo Salgado
  • - Chung-Ang University Hospital , Korea (South) Mi Kyung Kim
  • - China Medical University,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
  • - Lilavati Hospital & Research Centre , India Mahavir Swami
  • - Gimpo Woori Hospital , Korea (South) Son Youl Lee
  • - E-DA Hospital / I-Shou University , Taiwan Yu-Feng Wei
  • - Diagnostic Radiology , Korea (South) Dae Jung Kim
  • - Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
  • - Vital Imaging Centre , India Ganesh Agrawal
  • - Dongguk University International Hospital , Korea (South) Hee Seok Choi
  • - Asan medical center , Korea (South) Dae Yoon Kim
  • - homs national hospital , Syria basem hasen
  • - IRSA LA ROCHELLE FRANCE , France jean BIGOT
  • - Riverside Radiology Associates , United States Sumit Seth
  • - Maimonides Medical Center , United States naomi twersky
  • - CH de la Ce Basque , France Paul ARDILOUZE
  • - Dept. radiology, Chung-Ang univ. , Korea (South) JIN KYOUNG KIM
  • - Seoul National University Hospital , Korea (South) Sang Min Lee
  • - , Korea (South) kim hyoung
  • - trakya university , Turkey nermin tuncbilek
  • - Private Clinic , Bahamas Trupti Dabholkar
  • - CREM , Tunisia triki rached
  • - MEDINOVA DIAGNOSTICS , India Harsha NS
  • - CHU GRENOBLE , France anne-line MORONI
  • - Regional Imaging Ltd. - Riverina , Australia Rashid Hashmi
  • - Retd. Prof Thoracic Surgery B.J.MEDIDCAL COLLEGE Punr India , India Bankim Amin
  • - Vitalife Clinics , Pune , India. , India rahul deshmukh
  • - National Taiwan University Hospital , Taiwan Nin Chieh Hsu
  • - Shinchon Severance Hospital , Korea (South) Ho-Joon Lee
  • - Hangang Sacred Heart Hospital , Korea (South) Eil Seong Lee
  • - , Korea (South)
  • - Alnoor specialist hospital , Tanzania Magdy Akl
  • - Lou-Tung Poh-Ai Hospital , Taiwan Chen Chia
  • - Ondokuz Mayis University , Turkey Cetin Celenk
  • - radiology department ANNECY , France gilles GENIN
  • - Shin Kong Wu Ho-Su Memorial Hospital , Taiwan CHEN-CHUN LIN
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - ULTRA CARE , India Debabrata Das
  • - National Taiwan University Hospital , Taiwan Bangbin Chen
  • - Mokpo HanKook Hospital , Korea (South) Sang Uk Park
  • - Chungnam National University hospital , Korea (South) Kyoung Jin Oh
  • - Polyclinique de SAVOIE , France, Metropolitan GAY-DEPASSIER Philippe
  • - Jiangsu Prov Official Hosp , China dong jin
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - Jackson Memorial Hospital , United States Naganathan Mani
  • - IRSA La Rochelle France , France denis chabassie
  • - Homs National Hospital , Syria Rami Abou zalaf
  • - OSWAL CANCER HOSPITAL , India LOVKESH MITTAL
  • - Depatment of Radiology, Yongsan Hospital, College of Medicine, Chung-Ang University , Korea (South) Jae Seung Seo
  • - Armed Forces Chun-Cheon Hospital , Korea (South) ChaeHun Lim
  • - PITTWATER RADIOLOGY , Australia Julie Arora
  • - PITTWATER RADIOLOGY , Australia Saurabh Khandelwal
  • - All India Institute of Medical Sciences , India Ashish Gupta
  • - Command Hospital (Eastern Command) , India Vivek Sharma
  • - Mackay Memorial Hospital, Taipei, Taiwan , Taiwan Chia-Fu Tsai
▶ Correct Answer as Differential Diagnosis : 6/106,  5.7%
  • - MD PG Resident, PIMS,LONI, MAHARASHTRA, INDIA , India Sushant Bhadane
  • - Trakya University School of Medicine , Turkey Umut Ugur
  • - Soonchunhyang university Bucheon hospital , Korea (South) Minhee Lee
  • - Universidade Federal de S Paulo , Brazil Julia Capobianco
  • - st martin , France Mariotte
  • - Pe d , France Fabrice Bing
▶ Semi-Correct Answer : 3/106,  2.8%
  • - UVA , United States Rachita Khot
  • - Chonnam university hospital , Korea (South) Minyoung Jung
  • - Soonchunhyang University Cheonan Hospital , Korea (South) Park Sang Hyun
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