Weekly Chest CasesArchive of Old Cases

Case No : 696 Date 2011-02-27

  • Courtesy of Jin Ho Shin, Hyun Joo Lee, Eun Jin Chae / Asan Medical Center
  • Age/Sex 23 / M
  • Chief ComplaintRecurrent chest pain and fever
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Congenital pulmonary airway malformations (CPAM), type II.
Radiologic Findings
Chest radiograph shows a mass-like increased opacity in the right upper lobe. Chest CT image shows a patchy area of consolidation accompanying low attenuation within it in the right upper lobe. This lesion showed as the multiple cystic lesions on the chest CT scanned on 8 years ago.
Brief Review
A congenital pulmonary airway malformation (CPAM), historically referred to congenital cystic adenomatoid malformations (CCAM), is a rare developmental, non-hereditary, hamartomatous abnormality of the lung. CPAM constitutes up to 25% of congenital cystic lung lesions. 80~85% of cases are diagnosed in the first 2 years of life, rarely, the presentation is delayed until adulthood. Clinically, adults most frequently present with recurrent or persistent pulmonary infections. Other modes of presentation include hemoptysis, hemothorax, pneumothorax, pyopneumothorax, shortness of breath or as an incidental finding on a chest radiograph.
Radiological abnormalities range from a soft-tissue mass containing single or multiple air-filled cysts of varying sizes to a solid homogeneous mass that may be mistaken for a lung abscess or pneumonia.
After the morphologic description in 1975, Stocker et al. classified CPAM into three subtypes based on the clinical, gross and histological features. Type I is the most commonly encountered (50~70% of cases) with single or multiple large cysts (2 cm in diameter). Type II lesions (approximately 40% of cases) are characterized by multiple small cysts less than 2 cm in diameter (rarely larger). Type III lesions are rare (approximately 3% of cases). It usually shows as a bulky, solid mass.
Because the majority of cases are associated with recurrent infection and risk for development of carcinoma, Treatment of CPAM is usually surgical, involving complete resection of the relevant part of the lung.
References
1. Huang HJ, Talbot AR, Liu KC,et al. Infected cystic adenomatoid malformation in an adult. Ann Thorac Surg. 2004;78(1):337-9.
2. Herrero Y, Pinilla I, Torres I, et al. Cystic adenomatoid malformation of the lung presenting in adulthood. Ann Thorac Surg. 2005;79(1):326-9.
3. Oh BJ, Lee JS, Kim JS, et al. Congenital cystic adenomatoid malformation of the lung in adults: clinical and CT evaluation of seven patients. Respirology. 2006;11(4):496-501.
4. Khan NU, Jones MT, Greaves M. Case report: Congenital cystic adenomatoid malformation of an entire lung in a 33-year-old man: a case report and review of the literature. Br J Radiol. 2008;81(971):e276-8.
Keywords
Airway, Congenital, Bronchial abnormlity,

No. of Applicants : 111

▶ Correct Answer : 32/111,  28.8%
  • - radiologist, aditya imaging centre , India vivek patel
  • - Okayama University , Japan Akihiro Tada
  • - Chonnam National University Hwasoon Hospiral , Korea (South) You Ri Choi
  • - Univ. of Miami/Jackson Memorial , United States mantosh rattan
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - Hangang sacred heart hospital , Korea (South) You Mie Han
  • - Youngdong Hospital , Korea (South) Sang Min Lee
  • - Montreal , Canada D J
  • - BMC , United States Sri Sompalli
  • - Ewha Womans University Hospital , Korea (South) YOOKYUNG KIM
  • - PingTung Christian Hospital ,China Medical University ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
  • - Assam Medical College , India Pankaj Nagori
  • - Vital Imaging Centre, Mumbai,India , India Ganesh Agrawal
  • - All India Institute of Medical Sciences , India Ashish Gupta
  • - Iwate Medical University , Japan Akio Akahane
  • - Kangnam Sacred Heart Hospital , Korea (South) Jaewon Kim
  • - Chungnam National University hospital, Korea , Korea (South) sunkyoung you
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - Hopital Louis Pradel Lyon , France Clement Proust
  • - Songtan public health center, Goduk substation , Korea (South) Jaewang Shin
  • - IRSA La Rochelle France , France Denis Chabassiere
  • - Seoul National University Hospital , Korea (South) Yeo Kim
  • - Radiologie Brabois adultes , France ROPION helene
  • - Catholic medical center , Korea (South) hyuna kim
  • - Saga University , Japan Ryoko Egashira
  • - Toyama University Hospital, Laboratory of Pathology , Japan TOMONORI TANAKA
  • - chp st martin , France Mariotte benoit
  • - Saint Malo , France jean-baptiste Noel
  • - IRSA , France jean-luc BIGOT
  • - Istanbul , Turkey Armagan Sarac
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - Seoul St Mary Hospital , Korea (South) Chaehun Lim
▶ Correct Answer as Differential Diagnosis : 16/111,  14.4%
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - Fukuyama daiichi Hospital , Japan Mototsugu Saeki
  • - clinique de SAVOIE , France, Metropolitan gay-depassier philippe
  • - Doctors Hospital , Bahamas muneesh sharma
  • - CHRU lille , France manuel toledano
  • - Osaka University , Japan Hiromitsu Sumikawa
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - McGill University Health Center , Canada Alexandre Semionov
  • - Inha University Hospital , Korea (South) Ju Won Lee
  • - McGill University , Canada Ben Smith
  • - royal melbourne hospital , Australia KIMCHI KINGS
  • - EKH-Berlin , Germany Michael Weber
  • - IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
  • - Mallinckrodt Institute of Radiology , United States Naganathan Mani
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - All India Institute of medical sciences , India Justin Moses
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