Tuberc Respir Dis.  2013 Mar;74(3):97-103.

Multiple Cystic Lung Disease

• Koo HK
• Yoo CG

Abstract

A lung cyst is an air-filled lucent structure surrounded by a thin wall. The presence of multiple intrapulmonary cysts is defined as cystic lung disease. Although cystic lung disease is rare, incidental detection has increased significantly in recent years by screening using computed tomography. There are many conditions that can mimic lung cysts and cause cystic lung disease. Clinical, radiographic, and histologic findings are all necessary for a proper diagnosis, and multidisciplinary approaches are frequently required. The aim of this report is to review the causes and characteristics of cystic lung disease to better understand and improve treatment.

Keyword

Cysts; Lung Diseases; Lymphangioleiomyomatosis; Histiocytosis, Langerhans-Cell; Birt-Hogg-Dube Syndrome

MeSH Terms

Birt-Hogg-Dube Syndrome
Histiocytosis, Langerhans-Cell
Hydrazines
Lung
Lung Diseases
Lymphangioleiomyomatosis
Mass Screening
Hydrazines

Reference

1. Hansell DM, Bankier AA, MacMahon H, McLoud TC, Muller NL, Remy J. Fleischner Society: glossary of terms for thoracic imaging. Radiology. 2008; 246:697–722. PMID: 18195376.

2. Grant LA, Babar J, Griffin N. Cysts, cavities, and honeycombing in multisystem disorders: differential diagnosis and findings on thin-section CT. Clin Radiol. 2009; 64:439–448. PMID: 19264190.

3. Seaman DM, Meyer CA, Gilman MD, McCormack FX. Diffuse cystic lung disease at high-resolution CT. AJR Am J Roentgenol. 2011; 196:1305–1311. PMID: 21606293.

4. Lee KH, Lee JS, Lynch DA, Song KS, Lim TH. The radiologic differential diagnosis of diffuse lung diseases characterized by multiple cysts or cavities. J Comput Assist Tomogr. 2002; 26:5–12. PMID: 11801898.

5. Thurlbeck WM, Muller NL. Emphysema: definition, imaging, and quantification. AJR Am J Roentgenol. 1994; 163:1017–1025. PMID: 7976869.

6. Stanford W, Galvin JR. The diagnosis of bronchiectasis. Clin Chest Med. 1988; 9:691–699. PMID: 3069297.

7. Carrington CB, Cugell DW, Gaensler EA, Marks A, Redding RA, Schaaf JT, et al. Lymphangioleiomyomatosis: physiologic-pathologic-radiologic correlations. Am Rev Respir Dis. 1977; 116:977–995. PMID: 931190.

8. McCormack F, Brody A, Meyer C, Leonard J, Chuck G, Dabora S, et al. Pulmonary cysts consistent with lymphangioleiomyomatosis are common in women with tuberous sclerosis: genetic and radiographic analysis. Chest. 2002; 121(3 Suppl):61S. PMID: 11893688.

9. Green AJ, Smith M, Yates JR. Loss of heterozygosity on chromosome 16p13.3 in hamartomas from tuberous sclerosis patients. Nat Genet. 1994; 6:193–196. PMID: 8162074.

10. Ryu JH, Moss J, Beck GJ, Lee JC, Brown KK, Chapman JT, et al. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med. 2006; 173:105–111. PMID: 16210669.

11. 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; 25:803–828. PMID: 15888627.

12. McCormack FX. Lymphangioleiomyomatosis: a clinical update. Chest. 2008; 133:507–516. PMID: 18252917.

13. Johnson S. Costabel U, du Bois RM, Egan JJ, editors. Lymphangioleiomyomatosis. Diffuse parenchymal lung disease. Progress in respiratory research. 2007. Vol. 36. Basel: Karger;p. 275–284.

14. Johnson SR, Cordier JF, Lazor R, Cottin V, Costabel U, Harari S, et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010; 35:14–26. PMID: 20044458.

15. Taylor JR, Ryu J, Colby TV, Raffin TA. Lymphangioleiomyomatosis: clinical course in 32 patients. N Engl J Med. 1990; 323:1254–1260. PMID: 2215609.

16. Eliasson AH, Phillips YY, Tenholder MF. Treatment of lymphangioleiomyomatosis: a meta-analysis. Chest. 1989; 96:1352–1355. PMID: 2582843.

17. McCormack FX, Inoue Y, Moss J, Singer LG, Strange C, Nakata K, et al. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 2011; 364:1595–1606. PMID: 21410393.

18. Abbott GF, Rosado-de-Christenson ML, Franks TJ, Frazier AA, Galvin JR. From the archives of the AFIP: pulmonary Langerhans cell histiocytosis. Radiographics. 2004; 24:821–841. PMID: 15143231.

19. Harari S, Caminati A. Costabel U, du Bois RM, Egan JJ, editors. Pulmonary Langerhans' cell histiocytosis. Diffuse parenchymal lung disease. Progress in respiratory research. 2007. Vol. 36. Basel: Karger;p. 264–274.

20. Cosgrove GP, Frankel SK, Brown KK. Challenges in pulmonary fibrosis. 3. Cystic lung disease. Thorax. 2007; 62:820–829. PMID: 17726170.

21. Vassallo R, Ryu JH, Schroeder DR, Decker PA, Limper AH. Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults. N Engl J Med. 2002; 346:484–490. PMID: 11844849.

22. Tazi A. Adult pulmonary Langerhans' cell histiocytosis. Eur Respir J. 2006; 27:1272–1285. PMID: 16772390.

23. Tazi A, Soler P, Hance AJ. Adult pulmonary Langerhans' cell histiocytosis. Thorax. 2000; 55:405–416. PMID: 10770823.

24. Dauriat G, Mal H, Thabut G, Mornex JF, Bertocchi M, Tronc F, et al. Lung transplantation for pulmonary Langerhans' cell histiocytosis: a multicenter analysis. Transplantation. 2006; 81:746–750. PMID: 16534477.

25. Etienne B, Bertocchi M, Gamondes JP, Thevenet F, Boudard C, Wiesendanger T, et al. Relapsing pulmonary Langerhans cell histiocytosis after lung transplantation. Am J Respir Crit Care Med. 1998; 157:288–291. PMID: 9445312.

26. Cha SI, Fessler MB, Cool CD, Schwarz MI, Brown KK. Lymphoid interstitial pneumonia: clinical features, associations and prognosis. Eur Respir J. 2006; 28:364–369. PMID: 16571614.

27. Adley BP, Smith ND, Nayar R, Yang XJ. Birt-Hogg-Dube syndrome: clinicopathologic findings and genetic alterations. Arch Pathol Lab Med. 2006; 130:1865–1870. PMID: 17149965.

28. Ayo DS, Aughenbaugh GL, Yi ES, Hand JL, Ryu JH. Cystic lung disease in Birt-Hogg-Dube syndrome. Chest. 2007; 132:679–684. PMID: 17505035.

29. Agarwal PP, Gross BH, Holloway BJ, Seely J, Stark P, Kazerooni EA. Thoracic CT findings in Birt-Hogg-Dube syndrome. AJR Am J Roentgenol. 2011; 196:349–352. PMID: 21257886.

30. Traweek T, Rotter AJ, Swartz W, Azumi N. Cystic pulmonary metastatic sarcoma. Cancer. 1990; 65:1805–1811. PMID: 2156605.

31. Chan DP, Griffith JF, Lee TW, Chow LT, Yim AP. Cystic pulmonary metastases from epithelioid cell sarcoma. Ann Thorac Surg. 2003; 75:1652–1654. PMID: 12735604.

32. Aubry MC, Myers JL, Colby TV, Leslie KO, Tazelaar HD. Endometrial stromal sarcoma metastatic to the lung: a detailed analysis of 16 patients. Am J Surg Pathol. 2002; 26:440–449. PMID: 11914621.