대한흉부영상의학회 Weekly Chest Cases

Case No : 422 Date 2005-11-28

Courtesy of Tae Jung Kim, MD / Seoul National University Bundang Hospital, Korea
Age/Sex 41 / M
Chief ComplaintIncidental abnormality on screening chest CT

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Diagnosis With Brief Discussion

Diagnosis: Pulmonary Langerhans cell histiocytosis (LCH)
Radiologic Findings: Chest PA shows no remarkable finding. HRCT scans show multiple thin-walled air cysts with bizarre shapes and small irregular nodules in both upper and middle lung zones. The patient was a 41-year-old male with 20 pack years of smoking history. CT-guided hook wire localization of cyst in left upper lobe was performed. Diagnosis of pulmonary LCH was made by video-assisted-thoracoscopic surgery (VATS).
Brief Review: On HRCT, the distribution of the disease is similar to that on CXR with upper lobe predominance. In the early stages, findings include centrilobular opacities and small nodules (1 to 5 mm, but up to 1.5 cm). The nodules may be few or innumerable and typically have irregular margins. Later in the disorder there is cystic cavitation of small nodules, and cysts initially thick walled and later thin walled (typically lesions progress in this fashion). Some authors feel that the nodules do not cavitate and that the cysts represent paracicatrical emphysematous change adjacent to the nodules. The cysts are usually less than 10 mm in size, although cysts larger than 10 mm are found in over half the cases. The walls of the cysts are usually thin (1mm or less), but can be variable, and the cysts are not necessarily round (they may be bilobed or branching). The intervening lung parenchyma appears normal. In the late stage, there may be diffuse cysts, with no nodules evident, while in the early stages, only nodules may be seen. In the late stages, the disorder may be indistinguishable from lymphangiomyomatosis but sparing of the costophrenic angles suggests the diagnosis of LCH. Mediastinal adenopathy has been described in some series, but is usually uncommon. Other authors report medistinal adenopathy in up to 30% of LCH patients.
References: 1. Brauner MW, Grenier P, Mouelhi MM, Mompoint D, Lenoir S. Pulmonary histiocytosis X: evaluation with HRCT. Radiology 1989;172:255-258
2. Moor ADA, Godwin DJ, Muller NL, et al. Pulmonary histiocytosis X: comparison of radiographic and CT findings. Radiology 1989;172:249-254
3. Webb WR, Muller NL, Naidich DP. High-resolution CT of the lung. 3rd. Lippincott Williams & Wilkins, 2001:421-429
Keywords: Lung, Interstitial lung disease, LCH, smoking related ILD,

No. of Applicants : 35

▶ Correct Answer : 16/35, 45.7%

- Regional Imaging, Australia Rashid Hashmi
- Kyungpook National University Hospital Ju- Wan Choi
- Auxerre Hospital, France Larive Sebastien
- Clinic 4, Moscow, Russia Lepikhina Dasha
- Pgimer, India Saurabh Khandelwal
- Riverside Radiology Associates, Columbus, Ohio, USA Sumit K Seth
- Busan National University Hospital Su-mi Shin
- Radiologie Guiton, La Rochelle, France Jean-Luc Bigot
- Kyung-Hee University Hospital Su Youn Sim
- Chikuhou Rousai Hospital, Japan Uchida Kouei
- Homs National Hospital, Homs, Syria Rami Abou Zalaf
- ASL Bologna, Maggiore Hospital, Bologna, Italy Marcellino Burzi
- Radiologie Guiton, La Rochelle, France Denis Chabassiere
- China Medical University Hospital,Taiwan Jun-Jun Yeh
- Monaldi Hospital, Naples, Italy Gaetano Rea
- New Hyde Park, New York, USA Pinar Karakas

▶ Correct Answer as Differential Diagnosis : 7/35, 20.0%

- Hanyang University Hospital, Seoul, Korea Yo Won Choi
- Social Security Hospital, Ankara, Turkey Meric Tuzun
- Annecy Hospital, France Gilles Genin
- Seoul National University Hospital Heesun Park
- Marien Hospital, Germany Davis Chiramel
- Hangang Sacred Heart Hospital, Seoul, Korea Eil Seong Lee
- Max Hospital, New Delhi, India Vickrant Malhotra

▶ Semi-Correct Answer : 1/35, 2.9%

- Jikei University Hospital, Tokyo, Japan Masuo Ujita

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 422 Date 2005-11-28

Discussion

No. of Applicants : 35