대한흉부영상의학회 Weekly Chest Cases

Case No : 819 Date 2013-07-08

Courtesy of Kyung Eun Shin / Kyung Hee Medical Center
Age/Sex 53 / M
Chief Complaintchylothorax

Figure 1
Figure 2
Figure 3
Figure 4
Figure 5

Diagnosis With Brief Discussion

Diagnosis: Diffuse pulmonary lymphangiomatosis
Radiologic Findings: Chest PA shows increased interstitial markings with haziness in both lungs. Also, large amount of pleural effusion is noted on left hemithorax.
CT scans show diffuse interlobular septal thickening and combined ground glass opacities in both lungs. Large amount of pleural effusion is also noted on left hemithorax.
Pathologically, lymphocytic infiltrates are present through the stroma. Immunohistochemical staining with D2-40 confirms the proliferation of lymphatic channels. The smooth muscle fibers adjacent to the lymphatic channels are positive with Desmin and negative for HMB-45. These findings suggest the lymphangiomatosis rather than lymphangiomyomatosis.
Brief Review: Diffuse pulmonary lymphangiomatosis occurs predominantly in infants and children and is characterized by the proliferation of anastomosing lymphatics along the subpleural space and interlobular septa, and to a lesser degree along the bronchovascular bundles. D2-40 immunostain should aid in differentiating them from veins when muscularization of the lymphatics is present.
CT findings include diffuse thickening of the interlobular septa and bronchovascular bundle. Pleural effusion and pleural thickening are also prevalent. In some patients the condition may progress to end-stage lung disease and death, with no effective treatment except for lung transplantation.
From a pure imaging standpoint, the primary differential considerations in cases of pulmonary lymphangiomatosis would include congestive heart failure, pulmonary veno-occlusive disease, and Erdheim-Chester disease. In cases of congestive heart failure, there is often associated cardiomegaly and pulmonary venous hypertension seen on imaging. In cases of pulmonary veno-occlusive disease, there is typically also prominence of the central pulmonary arteries. In Erdheim-Chester disease, there are sclerotic changes in the diaphyses and metaphyses of the long bones and frequent extrathoracic soft tissue masses.
References: 1.Boland JM, Tazelaar HD, et al. Diffuse pulmonary lymphatic disease presenting as interstitial lung disease in adulthood: report of 3 cases. Am J Surg Pathol. 2012 Oct;36(10):1548-54
2.Swensen SJ, Hartman TE, Mayo JR, et al. Diffuse pulmonary lymphangiomatosis: CT findings. J Comput Assist Tomogr. 1995;19:348
Keywords: Lung, Interstitial lung disease,

No. of Applicants : 62

▶ Correct Answer : 10/62, 16.1%

- Kyung Hee University Hospital at Gangdong , Korea (South) Seong Jong Yun
- Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
- Onomichi Municipal Hospital , Japan Yoshihisa Masaoka
- Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
- Severance Hospital , Korea (South) Hyun Gi Kim
- Kyoto University , Japan Akihiko Sakata
- chungbuk national university hospital , Korea (South) Soo Jung Lee
- CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
- All India Institute of medical sciences , India Justin Moses
- IRCCS Istituto Oncologico - Bari , Italy Carlo Florio

▶ Correct Answer as Differential Diagnosis : 10/62, 16.1%

- Oita University, Faculty of Medicine , Japan Fumito Okada
- University of British Columbia , Canada Amr Ajlan
- Nagasaki University Hospital, Department of Pathology , Japan TOMONORI TANAKA
- James Paget Hospital , U.K , United Kingdom nabil mahmood
- Onomichi municipal hospital , Japan Hirofumi Mifune
- Chungbuk national university hospital , Korea (South) Jisun Lee
- chungbuk national university hospital , Korea (South) jeehye kim
- Kizawa Memorial Hospital , Japan Yo Kaneko
- All India Institute of Medical Sciences , India Ashish Gupta
- NASA SCANS , India RAKESH BHATIA

대한흉부영상의학회 Korean Society of Thoracic Radiology

Cases by Disease Category

Case No : 819 Date 2013-07-08

Discussion

No. of Applicants : 62