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Weekly Chest CasesArchive of Old Cases

Case No : 712 Date 2011-06-22

  • Courtesy of Jae-Young Kim, MD, Woon-Jung Kwon, MD. / Ulsan University Hospital
  • Age/Sex 41 / F
  • Chief ComplaintDyspnea for years
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Figure 1

Diagnosis With Brief Discussion

Diagnosis
Tuberous sclerosis associated Lymphangioleiomyomatosis
Radiologic Findings
Chest radiographies demonstrate a large bullae in the left upper lung zone and variable-sized air cysts or emphysema in the both lungs.
Axial contrast enhanced chest CT images (lung window setting) show numerous thin-walled cysts surrounded by normal lung parenchyma and distributed diffusely and bilaterally. Including abdominal images of axial contrast enhanced chest CT demonstrate the right kidney enlargement by numerous cysts and a mass of mixed attenuation containing vascular structures; variable-sized multiple cortical cysts and containing fat and enhancing vascular component.
Axial T2WI brain MR image shows multiple subependymal nodules (arrowheads) in the both lateral ventricles. Axial FLAIR brain MR image shows slightly increased signal intensity (white arrows) in the both frontal and right parietal cortex.

Figure 6

Figure 7

Brief Review
Lymphangioleiomyomatosis (LAM) is a rare interstitial lung disease that affects women exclusively, typically during their reproductive years. A small percentage of patients, also typically women, have LAM in association with tuberous sclerosis complex (TSC). LAM is characterized by the abnormal proliferation of smooth muscle cells (LAM cells) in the lungs and in the thoracic and retroperitoneal lymphatics. Affected patients are at risk of developing renal hamartomas or angiomyolipomas. Patients with LAM characteristically present with chronic dyspnea and cough and less commonly with spontaneous pneumothorax.
Tuberous Sclerosis Complex is an inheritable autosomal dominant disorder characterized by multifocal systemic hamartomas that may affect the central nervous system, skin, heart, kidneys, and other organs. The Vogt triad of epileptic seizures, mental retardation, and facial angiofibromas (formerly misnamed as adenoma sebaceum) is exhibited by only approximately 29% of affected patients.
Relationship between TSC and LAM; Although histologic and clinical features may be identical in women with LAM and patients with TSC-associated pulmonary involvement, there are reported differences. Patients with TSC- associated LAM, compared with patients with sporadic LAM, generally experience a longer delay between onset of symptoms and diagnosis of pulmonary disease, exhibit chylothoraces less frequently, and are more likely to present with gradual onset of dyspnea.
Radiographic abnormalities in patients with LAM have been reported as a pattern of generalized, symmetric, reticular, or reticulonodular opacities, seen in approximately 80%-90% of affected patients. Lung volumes have been reported as normal at chest radiography in 55%-78% of affected patients and increased in 22%-45%. Pneumothorax is a common clinical and radiographic presenting manifestation of LAM, reported in 39%-53% of patients. Pleural effusion (secondary to chylothorax) is a common radiographic feature, occurring in 10%-20% of patients, and may be unilateral or bilateral.
The CT manifestations of LAM are distinctive, characterized by numerous thin-walled cysts surrounded by normal lung parenchyma and distributed diffusely and bilaterally. Occasionally, a chylous effusion is suggested at chest CT based on low-attenuation (- 17HU) fluid that may relate to the presence of fat.
Renal angiomyolipomas have been observed on abdominal CT scans of 20%-54% of patients with LAM. These tumors are characterized by CT evidence of soft tissue, fat, and enhancing vessels in variable proportions within a renal mass.
A variety of intracranial manifestations of TS are known. Four common CNS abnormalities are cortical tubers, subependymal nodules, subependymal giant cell astrocytomas (SGCAs), and white matter abnormalities. Except for SGCAs, these abnormalities can be seen in almost all patients with TS.
References
1. Abbott GF, Rosado-de-Christenson ML, Frazier AA, Franks TJ, Pugatch RD, Galvin JR; From the archives of the AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation. Radiographics. 2005 May-Jun;25(3):803-28.
2. Johnson SR, Cordier JF, Lazor R, Cottin V, Costabel U, Harari S, Reynaud-Gaubert M, Boehler A, Brauner M, Popper H, Bonetti F, Kingswood C; Review Panel of the ERS LAM Task Force. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010 Jan;35(1):14-26.
3. Pallisa E, Sanz P, Roman A, Maj� J, Andreu J, C�ceres J. ; Lymphangioleiomyomatosis: Pulmonary and Abdominal Findings with Pathologic Correlation. Radiographics. 2002 Oct;22 Spec No:S185-98. Review.
Keywords
lung, congenital,

No. of Applicants : 128

▶ Correct Answer : 75/128,  58.6%
  • - clinique de SAVOIE , France, Metropolitan gay-depassier philippe
  • - Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
  • - Onomichi municipal hospital , Japan Ryotaro Kishi
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - Hopital Louis Pradel LYON , France matthieu ROUSSET
  • - McGill University Health Center , Canada Alexandre Semionov
  • - NDMVP Nashik , India Imran Jindani
  • - Istanbul , Turkey Armagan Sarac
  • - Radnet Teleradiology , Turkey Murat Ulusoy
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Hangang sacred heart hospital , Korea (South) You Mie Han
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - Dongnam Institute of Radiological and Medical Sciences , Korea (South) Dae Wook Yeh
  • - Samsung medical center , Korea (South) Hyun Su Kim
  • - Youngdong Hospital , Korea (South) Sang Min Lee
  • - Chung-Nam National University Hospital , Korea (South) KI-TAE HAN
  • - Vitalife Clinics , Pune , India. , India Rahul Deshmukh
  • - sunder lal jain hospital , India sanjeev vachher
  • - Chaeil Clinic , Korea (South) Sunkyung Lim
  • - DCA, , India Rajesh Gothi
  • - CHU NANCY , France, Metropolitan LEGOU Fran�ois
  • - James Paget Hospital , U.K , United Kingdom nabil mahmood
  • - Az Osp Santa Maria Terni , Italy angelo carloni
  • - Saga University , Japan Ryoko Egashira
  • - Catholic medical center , Korea (South) hyuna kim
  • - Goa Medical College , India Paresh Desai
  • - PingTung Christian Hospital ,China Medical University ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
  • - Chungnam National University hospital , Korea (South) sunkyoung you
  • - Government Medical college, Kozhikkode , India jineesh thottath
  • - Okayama university, Department of Pathology , Japan Junya Itakura
  • - St. Luke , Korea (South) usb
  • - Saint Malo , France jean-baptiste Noel
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - Fr Mullers Medical College , India Deepu Thomas
  • - Toyama University Hospital, Laboratory of Pathology , Japan TOMONORI TANAKA
  • - radiologist, aditya imaging centre , India vivek patel
  • - CHA Bundang hospital , Korea (South) Hyerin Kim
  • - EWHA WOMANS MOKDONG UNIVERSITY HOSPITAL , Korea (South) Sang Min Lee
  • - Teleradiology solutions , India harish bhambore
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - Armed Forces Yangju Hospital , Korea (South) Ho-Joon Lee
  • - Fukuyama daiichi Hospital , Japan Mototsugu Saeki
  • - University of British Columbia , Canada Amr Ajlan
  • - Mallinckrodt Institute of Radiology , United States Travis Henry
  • - Yonsei university Shinchon Severance hospital , Korea (South) Na-young Shin
  • - IMS, BHU,VARANASI , India Sriram Patwari
  • - Kohka Public Hospital , Japan Akitoshi Inoue
  • - IRSA , France jean-luc BIGOT
  • - royal melbourne hospital , Australia kimchi kings
  • - Hanyang university medical center , Korea (South) YOONAH SONG
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - KASH , Saudi Arabia Elbagir Nasser
  • - Bupyong Serim Hospital , Korea (South) Hee Seok Choi
  • - Teleradiology , India Savith Kumar
  • - National Jewish Health , United States Jonathan Chung
  • - Radiologie Brabois adultes , France ROPION helene
  • - Mallinckrodt Institute of Radiology , United States Naganathan Mani
  • - Soon Chun Hyang, Seoul , Korea (South) Yoo Na Kim
  • - Kizawa Memorial Hospital , Japan Yo Kaneko
  • - Calcutta Heart Research Centre, Kolkata , India Pankaj Nagori
  • - Hotel-Dieu Montreal , Canada nicolas gautier
  • - Seoul St Mary Hospital , Korea (South) Chae Lim
  • - Homs National Hospital , Syria Rami Abou Zalaf
  • - IRSA La Rochelle France , France Denis Chabassiere
  • - Yonsei University college of medicine Severance Hospital , Korea (South) Hua Sun Kim
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
  • - Pneumologia Universitaria, Policlinico di Bari , Italy Mario Damiani
  • - St-Luc Bruxelles , Belgium Tancredi Illario
  • - CHRU lille , France manuel toledano
  • - DrRP GMEDICAL COLLEGE TANDA , India DINESH SOOD
  • - NASA SCANS , India RAKESH BHATIA
  • - Severance hospital , Korea (South) Saerom Hong
  • - KUMC ansan , Korea (South) kihwan kim
  • - Okayama university , Japan Mayu Uka
▶ Correct Answer as Differential Diagnosis : 1/128,  0.8%
  • - HOSPITAL SOTIRIA , Greece AIKATERINI DELAVERI
▶ Semi-Correct Answer : 48/128,  37.5%
  • - HSC , Brazil Diogo Pinheiro
  • - Assam Medical college , India samudra borah
  • - NTUH , Taiwan Kuei-pin Chung
  • - All India Institute of medical sciences , India Justin Moses
  • - Okayama University , Japan Akihiro Tada
  • - Montreal , Canada D J
  • - ministry of health , United Arab Emirates jesia mansoor
  • - KMC,Manipal , India satish maddukuri
  • - yeong gwang general hospital , Korea (South) Mokhee Kim
  • - Hopital Louis Pradel Lyon , France Clement Proust
  • - Japanese Red Cross Society Himeji Hospital , Japan Yuichiro Kanie
  • - SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OOKUBO
  • - Inha University Hospital , Korea (South) Ju Won Lee
  • - Gangnam Severance Hospital , Korea (South) Min Jeong Kim
  • - China Medical University , China zhang wei
  • - Iwate Medical University , Japan Akio Akahane
  • - , Korea (South) Kim sooyeon
  • - Asan medical center , Korea (South) sang young oh
  • - Chonbuk National University Hospital , Korea (South) Jisoo Song
  • - radiology , Korea (South) Lee Sanghee
  • - A.P.Chest hospital ,hyderabad , India bhaskar kakarla
  • - Department of Radiology, Chonnam National University Hospital , Korea (South) Hyungjun Kim
  • - Chonnam National University Hospital , Korea (South) Daun Lee
  • - Seoul National University Hospital , Korea (South) Hyungjin Kim
  • - EKH-Berlin , Germany Michael Weber
  • - Virgin Mary Hospital Burgas , Bulgaria VLADISLAV RUSINOV
  • - Ishikawa Matto Central Hospital , Japan Manabu Akimoto
  • - Chonnam university hospital , Korea (South) LEE JI HYUN
  • - SMC , Korea (South) Yi Kyung Kim
  • - Univ. of Miami/Jackson Memorial , United States mantosh rattan
  • - Yonsei University College of Medicine, Shinchon Severance hospital , Korea (South) Young Joo Suh
  • - Saga University , Japan Tetsuya Kondo
  • - Himeji Red Cross Hospital , Japan ryota inai
  • - DongA University hospital , Korea (South) Jiyoung Kang
  • - Louisiana State University - Shreveport , United States Carlos Previgliano
  • - assam medical college , India shantiranjan sanyal
  • - Hangang Sacred Heart Hospital , Korea (South) Eil Seong Lee
  • - Vikram hospital , India Pravin Mahadevappa
  • - GOLD child and chest clinic tirunelvelli , India saravanan m
  • - Severance hospital , Korea (South) Beomseok Sohn
  • - Plus radiology , Korea (South) Park Sang Hyun
  • - All India Institute of Medical Sciences , India Ashish Gupta
  • - Vital Imaging Centre, Mumbai,India , India Ganesh Agrawal
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - Asan Medical Center , Korea (South) Sanghyun Choi
  • - medical , India anup gupta
  • - CNUH , Korea (South) Seoin Jeong
  • - Seoul National University Hospital , Korea (South) YongSub Song
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