From Cancer Mimicking Orphan Lung Disease to Orphan Thoracic Oncology

  • Nicolas Girard
  • Vincent Cottin
  • Jean-François Cordier
Chapter

Abstract

A variety of rare neoplastic and non-neoplastic disorders may develop in the lung, the pleura, and the mediastinum. Some may have a propensity to mimic lung carcinoma as well as benign orphan lung diseases at some level of examination, as they may share with these clinical, imaging, pathological, and even molecular features. Challenges in the differential diagnoses among reciprocal mimics are well illustrated through examples as bronchioloalveolar carcinoma, primary pulmonary lymphomas, and vascular sarcomas. Pseudotumors have further been described, actually corresponding to a heterogeneous group of diseases characterised by circumscribed fibrous tissue and inflammatory cells. Among the inflammatory pseudotumors, neoplastic/non-neoplastic borderline disorders have been identified, such as inflammatory myofibroblastic tumor, which presents with clonal proliferation and has eventually emerged as a true neoplasm. Finally, some rare pulmonary diseases are emerging as borderline neoplastic non-neoplastic disorders, that require multidisciplinary expertise both in the field of orphan pulmonary diseases and in thoracic oncology, including amyloidosis or even Langerhans cell histiocytosis. Ultimately, implementing multi-disciplinary expert consensus is mandatory to determine the optimal management of these disorders.

Keywords

Lung cancer Rare disease Pseudotumor Inflammation Sarcoma Lymphoma 

References

  1. 1.
    Gabrielson E. Worldwide trends in lung cancer pathology. Respirology. 2006;11:533–8.PubMedCrossRefGoogle Scholar
  2. 2.
    Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC. Pathology and genetics of tumors of the lung, pleura, thymus, and heart. World Health Organization Classification of Tumors. Lyon: IARC Press; 2004.Google Scholar
  3. 3.
    Girard N, Barbareschi M, Cordier JF, Murer B. What is a rare tumor, and how to deal with it clinically? Eur Respir Soc Monogr. 2007;39:85–133.CrossRefGoogle Scholar
  4. 4.
    Wick MR, Tazelaar HD. Pseudoneoplastic lesions: general considerations. Arch Pathol Lab Med. 2010;134:351–61.PubMedGoogle Scholar
  5. 5.
    Borczuk AC. Benign tumors and tumorlike conditions of the lung. Arch Pathol Lab Med. 2008;132:1133–48.PubMedGoogle Scholar
  6. 6.
    Girard N, Cordier JF. Pseudotumours and reciprocal mimics of neoplastic and non-neoplastic pulmonary disorders. Eur Respir Monogr. 2011;54:341–65.Google Scholar
  7. 7.
    Cerfolio RJ, Allen MS, Nascimento AG, Deschamps C, Trastek VF, Miller DL, Pairolero PC. Inflammatory pseudotumors of the lung. Ann Thorac Surg. 1999;67:933–6.PubMedCrossRefGoogle Scholar
  8. 8.
    Eyden B, Banerjee SS, Shenjere P, Fisher C. The myofibroblast and its tumors. J Clin Pathol. 2009;62:236–49.PubMedCrossRefGoogle Scholar
  9. 9.
    Cordier JF. Cryptogenic organising pneumonia. Eur Respir J. 2006;28:422–46.PubMedCrossRefGoogle Scholar
  10. 10.
    Cohen MB, Austin JH, Smith-Vaniz A, Lutzky J, Grimes MM. Nodular bleomycin toxicity. Am J Clin Pathol. 1989;92:101–4.PubMedGoogle Scholar
  11. 11.
    Garfield DH, Cadranel JL, Wislez M, Franklin WA, Hirsch FR. The bronchioloalveolar carcinoma and peripheral adenocarcinoma spectrum of diseases. J Thorac Oncol. 2006;1:344–59.PubMedCrossRefGoogle Scholar
  12. 12.
    Travis WD, Brambilla E, Noguchi M, Nicholson AG, Geisinger KR, Yatabe Y, Beer DG, Powell CA, Riely GJ, Van Schil PE, Garg K, Austin JH, Asamura H, Rusch VW, Hirsch FR, Scagliotti G, Mitsudomi T, Huber RM, Ishikawa Y, Jett J, Sanchez-Cespedes M, Sculier JP, Takahashi T, Tsuboi M, Vansteenkiste J, Wistuba I, Yang PC, Aberle D, Brambilla C, Flieder D, Franklin W, Gazdar A, Gould M, Hasleton P, Henderson D, Johnson B, Johnson D, Kerr K, Kuriyama K, Lee JS, Miller VA, Petersen I, Roggli V, Rosell R, Saijo N, Thunnissen E, Tsao M, Yankelewitz D. International Association for the Study of Lung Cancer/American Thoracic Society/European Respiratory Society International Multidisciplinary Classification of Lung Adenocarcinoma. J Thorac Oncol. 2011;6:244–85.PubMedCrossRefGoogle Scholar
  13. 13.
    Kim TJ, Park CM, Goo JM, Lee KW. Is there a role for FDG PET in the management of lung cancer manifesting predominantly as ground-glass opacity? AJR Am J Roentgenol. 2012;198:83–8.PubMedCrossRefGoogle Scholar
  14. 14.
    Wislez M, Massiani MA, Milleron B, Souidi A, Carette MF, Antoine M, Cadranel J. Clinical characteristics of pneumonic-type adenocarcinoma of the lung. Chest. 2003;123:1868–77.PubMedCrossRefGoogle Scholar
  15. 15.
    Duruisseaux M, Baudrin L, Quoix E, Wislez M, Moro-Sibilot D, Coëtmeur D, Monnet I, Mourlanette P, Morère JF, Soria JC, Westeel V, Morin F, Cadranel J. Chemotherapy effectiveness after first-line gefitinib treatment for advanced lepidic predominant adenocarcinoma (formerly advanced bronchioloalveolar carcinoma): exploratory analysis of the IFCT-0401 trial. J Thorac Oncol. 2012;7:1423–31.PubMedCrossRefGoogle Scholar
  16. 16.
    Cadranel J, Wislez M, Antoine M. Primary pulmonary lymphoma. Eur Respir J. 2002;20:750–62.PubMedCrossRefGoogle Scholar
  17. 17.
    Cordier JF, Chailleux E, Lauque D, Reynaud-Gaubert M, Dietemann-Molard A, Dalphin JC, Blanc-Jouvan F, Loire R. Primary pulmonary lymphomas. A clinical study of 70 cases in nonimmunocompromised patients. Chest. 1993;103:201–8.PubMedCrossRefGoogle Scholar
  18. 18.
    Borie R, Wislez M, Thabut G, Antoine M, Rabbat A, Couderc LJ, Monnet I, Nunes H, Blanc FX, Mal H, Bergeron A, Dusser D, Israël-Biet D, Crestani B, Cadranel J. Clinical characteristics and prognostic factors of pulmonary MALT lymphoma. Eur Respir J. 2009;34:1408–16.PubMedCrossRefGoogle Scholar
  19. 19.
    Remstein ED, Dogan A, Einerson RR, Paternoster SF, Fink SR, Law M, Dewald GW, Kurtin PJ. The incidence and anatomic site specificity of chromosomal translocations in primary extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) in North America. Am J Surg Pathol. 2006;30:1546–53.PubMedCrossRefGoogle Scholar
  20. 20.
    Gitelson E, Al-Saleem T, Smith MR. Review: lymphomatoid granulomatosis: challenges in diagnosis and treatment. Clin Adv Hematol Oncol. 2009;7:68–70.PubMedGoogle Scholar
  21. 21.
    Charest M, Armanious S. Prognostic implication of the lymphangitic carcinomatosis pattern on perfusion lung scan. Can Assoc Radiol J. 2012;63:294–303.PubMedCrossRefGoogle Scholar
  22. 22.
    Wu JW, Chiles C. Lymphangitic carcinomatosis from prostate carcinoma. J Comput Assist Tomogr. 1999;23:761–3.PubMedCrossRefGoogle Scholar
  23. 23.
    Bruce DM, Heys SD, Eremin O. Lymphangitis carcinomatosa: a literature review. J R Coll Surg Edinb. 1996;41:7–13.PubMedGoogle Scholar
  24. 24.
    Vento S, Cainelli F, Temesgen Z. Lung infections after cancer chemotherapy. Lancet Oncol. 2008;9:982–92.PubMedCrossRefGoogle Scholar
  25. 25.
    Dail DH, Liebow AA, Gmelich JT, Friedman PJ, Miyai K, Myer W, Patterson SD, Hammar SP. Intravascular, bronchiolar, and alveolar tumor of the lung (IVBAT). An analysis of twenty cases of a peculiar sclerosing endothelial tumor. Cancer. 1983;51:452–64.PubMedCrossRefGoogle Scholar
  26. 26.
    Mehrabi A, Kashfi A, Fonouni H, Schemmer P, Schmied BM, Hallscheidt P, Schirmacher P, Weitz J, Friess H, Buchler MW, Schmidt J. Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy. Cancer. 2006;107:2108–21.PubMedCrossRefGoogle Scholar
  27. 27.
    Kitaichi M, Nagai S, Nishimura K, Itoh H, Asamoto H, Izumi T, Dail DH. Pulmonary epithelioid haemangioendothelioma in 21 patients, including three with partial spontaneous regression. Eur Respir J. 1998;12:89–96.PubMedCrossRefGoogle Scholar
  28. 28.
    Boudousquie AC, Lawce HJ, Sherman R, Olson S, Magenis RE, Corless CL. Complex translocation (7;22) identified in an epithelioid hemangioendothelioma. Cancer Genet Cytogenet. 1996;92:116–21.PubMedCrossRefGoogle Scholar
  29. 29.
    Katzenstein AL, Doxtader E, Narendra S. Lymphomatoid granulomatosis: insights gained over 4 decades. Am J Surg Pathol. 2010;34:e35–48.PubMedCrossRefGoogle Scholar
  30. 30.
    Taniere P, Thivolet-Bejui F, Vitrey D, Isaac S, Loire R, Cordier JF, Berger F. Lymphomatoid granulomatosis-A report on four cases: evidence for B phenotype of the tumoral cells. Eur Respir J. 1998;12:102–6.PubMedCrossRefGoogle Scholar
  31. 31.
    Pisani RJ, DeRemee RA. Clinical implications of the histopathologic diagnosis of pulmonary lymphomatoid granulomatosis. Mayo Clin Proc. 1990;65:151–63.PubMedCrossRefGoogle Scholar
  32. 32.
    Robak T, Kordek R, Urbanska-Rys H, Robak P, Bartkowiak J, Bednarek A, Chudobinski C, Chojnowski K. High activity of rituximab combined with cladribine and cyclophosphamide in a patient with pulmonary lymphomatoid granulomatosis and bone marrow involvement. Leuk Lymphoma. 2006;47:1667–9.PubMedCrossRefGoogle Scholar
  33. 33.
    Guinee Jr DG, Perkins SL, Travis WD, Holden JA, Tripp SR, Koss MN. Proliferation and cellular phenotype in lymphomatoid granulomatosis: implications of a higher proliferation index in B cells. Am J Surg Pathol. 1998;22:1093–100.PubMedCrossRefGoogle Scholar
  34. 34.
    Hansell DM, Bankier AA, MacMahon H, McLoud TC, Müller NL, Remy J. Fleischner Society: glossary of terms for thoracic imaging. Radiology. 2008;246:697–722.PubMedCrossRefGoogle Scholar
  35. 35.
    Hoag JB, Sherman M, Fasihuddin Q, Lund ME. A comprehensive review of spontaneous pneumothorax complicating sarcoma. Chest. 2010;138:510–8.PubMedCrossRefGoogle Scholar
  36. 36.
    Tateishi U, Hasegawa T, Kusumoto M, Yamazaki N, Iinuma G, Muramatsu Y, Moriyama N. Metastatic angiosarcoma of the lung: spectrum of CT findings. AJR Am J Roentgenol. 2003;180:1671–4.PubMedCrossRefGoogle Scholar
  37. 37.
    Traweek T, Rotter AJ, Swartz W, Azumi N. Cystic pulmonary metastatic sarcoma. Cancer. 1990;65:1805–11.PubMedCrossRefGoogle Scholar
  38. 38.
    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;26:440–9.PubMedCrossRefGoogle Scholar
  39. 39.
    Mahadeva R, Stewart S, Wallwork J. Metastatic endometrial stromal sarcoma masquerading as pulmonary lymphangioleiomyomatosis. J Clin Pathol. 1999;52:147–8.PubMedCentralPubMedCrossRefGoogle Scholar
  40. 40.
    Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, Rottem M, Fauci AS. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116:488–98.PubMedCrossRefGoogle Scholar
  41. 41.
    Farber HW, Loscalzo J. Pulmonary arterial hypertension. N Engl J Med. 2004;351:1655–65.PubMedCrossRefGoogle Scholar
  42. 42.
    Masri FA, Xu W, Comhair SA, Asosingh K, Koo M, Vasanji A, Drazba J, Anand-Apte B, Erzurum SC. Hyperproliferative apoptosis-resistant endothelial cells in idiopathic pulmonary arterial hypertension. Am J Physiol Lung Cell Mol Physiol. 2007;293:L548–54.PubMedCrossRefGoogle Scholar
  43. 43.
    Parish JM, Rosenow 3rd EC, Swensen SJ, Swensen SJ, Crotty TB. Pulmonary artery sarcoma. Clinical features. Chest. 1996;110:1480–8.PubMedCrossRefGoogle Scholar
  44. 44.
    Yi CA, Lee KS, Choe YH, Han D, Kwon OJ, Kim S. Computed tomography in pulmonary artery sarcoma: distinguishing features from pulmonary embolic disease. J Comput Assist Tomogr. 2004;28:34–9.PubMedCrossRefGoogle Scholar
  45. 45.
    Girard N, Triby-Moreau C, Benabidallah S, Tronc F, Revel D, Giammarile F, Cordier JF. Pulmonary artery sarcoma, a paradigm of orphan thoracic oncology. Presse Med. 2009;38:1167–70.PubMedCrossRefGoogle Scholar
  46. 46.
    Mussot S, Ghigna MR, Mercier O, Fabre D, Fadel E, Le Cesne A, Simonneau G, Dartevelle P. Retrospective institutional study of 31 patients treated for pulmonary artery sarcoma. Eur J Cardiothorac Surg. 2012. doi: 10.1093/ejcts/ezs387.Google Scholar
  47. 47.
    Pettinato G, Manivel JC, De Rosa N, Dehner LP. Inflammatory myofibroblastic tumor (plasma cell granuloma). Clinicopathologic study of 20 cases with immunohistochemical and ultrastructural observations. Am J Clin Pathol. 1990;94:538–46.PubMedGoogle Scholar
  48. 48.
    Matsubara O, Tan-Liu NS, Kenney RM, Mark EJ. Inflammatory pseudotumors of the lung: progression from organizing pneumonia to fibrous histiocytoma or to plasma cell granuloma in 32 cases. Hum Pathol. 1988;19:807–14.PubMedCrossRefGoogle Scholar
  49. 49.
    Gal AA, Koss MN, McCarthy WF, Hochholzer L. Prognostic factors in pulmonary fibrohistiocytic lesions. Cancer. 1994;73:1817–24.PubMedCrossRefGoogle Scholar
  50. 50.
    Gómez-Román JJ, Sánchez-Velasco P, Ocejo-Vinyals G, Hernández-Nieto E, Leyva-Cobián F, Val-Bernal JF. Human herpesvirus-8 genes are expressed in pulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). Am J Surg Pathol. 2001;25:624–9.PubMedCrossRefGoogle Scholar
  51. 51.
    Yamamoto H, Yamaguchi H, Aishima S, Oda Y, Kohashi K, Oshiro Y, Tsuneyoshi M. Inflammatory myofibroblastic tumor versus IgG4-related sclerosing disease and inflammatory pseudotumor: a comparative clinicopathologic study. Am J Surg Pathol. 2009;33:1330–40.PubMedCrossRefGoogle Scholar
  52. 52.
    Snyder CS, Dell’Aquila M, Haghighi P, Baergen RN, Suh YK, Yi ES. Clonal changes in inflammatory pseudotumor of the lung: a case report. Cancer. 1995;76:1545–9.PubMedCrossRefGoogle Scholar
  53. 53.
    Coffin CM, Hornick JL, Fletcher CD. Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. Am J Surg Pathol. 2007;31:509–20.PubMedCrossRefGoogle Scholar
  54. 54.
    Lawrence B, Perez-Atayde A, Hibbard MK, Rubin BP, Dal Cin P, Pinkus JL, Pinkus GS, Xiao S, Yi ES, Fletcher CD, Fletcher JA. TPM3-ALK and TPM4-ALK oncogenes in inflammatory myofibroblastic tumors. Am J Pathol. 2000;157:377–84.PubMedCentralPubMedCrossRefGoogle Scholar
  55. 55.
    Jung SH, Yim SH, Hu HJ, Jung CK, Lee SH, Kim DH, Chung YJ. Copy number alterations and expression profiles of candidate genes in a pulmonary inflammatory myofibroblastic tumor. Lung Cancer. 2010;70:152–7.PubMedCrossRefGoogle Scholar
  56. 56.
    Kim TS, Han J, Kim GY, Lee KS, Kim H, Kim J. Pulmonary inflammatory pseudotumor (inflammatory myofibroblastic tumor): CT features with pathologic correlation. J Comput Assist Tomogr. 2005;29:633–9.PubMedCrossRefGoogle Scholar
  57. 57.
    Hosler GA, Steinberg DM, Sheth S, Hamper UM, Erozan YS, Ali SZ. Inflammatory pseudotumor: a diagnostic dilemma in cytopathology. Diagn Cytopathol. 2004;31:267–70.PubMedCrossRefGoogle Scholar
  58. 58.
    Fabre D, Fadel E, Singhal S, de Montpreville V, Mussot S, Mercier O, Chataigner O, Dartevelle PG. Complete resection of pulmonary inflammatory pseudotumors has excellent long-term prognosis. J Thorac Cardiovasc Surg. 2009;137:435–40.PubMedCrossRefGoogle Scholar
  59. 59.
    Kim JH, Cho JH, Park MS, Chung JH, Lee JG, Kim YS, Kim SK, Kim SK, Shin DH, Choi BW, Choe KO, Chang J. Pulmonary inflammatory pseudotumor–a report of 28 cases. Korean J Intern Med. 2002;17:252–8.PubMedGoogle Scholar
  60. 60.
    Butrynski JE, D’Adamo DR, Hornick JL, Dal Cin P, Antonescu CR, Jhanwar SC, Ladanyi M, Capelletti M, Rodig SJ, Ramaiya N, Kwak EL, Clark JW, Wilner KD, Christensen JG, Jänne PA, Maki RG, Demetri GD, Shapiro GI. Crizotinib in ALK-rearranged inflammatory myofibroblastic tumor. N Engl J Med. 2010;363:1727–33.PubMedCentralPubMedCrossRefGoogle Scholar
  61. 61.
    Flieder DB, Suster S, Moran CA. Idiopathic fibroinflammatory (fibrosing/ sclerosing) lesions of the mediastinum: a study of 30 cases with emphasis on morphologic heterogeneity. Mod Pathol. 1999;12:257–64.PubMedGoogle Scholar
  62. 62.
    Rossi SE, McAdams HP, Rosado-de-Christenson ML, Franks TJ, Galvin JR. Fibrosing mediastinitis. Radiographics. 2001;21:737–57.PubMedCrossRefGoogle Scholar
  63. 63.
    Mole TM, Glover J, Sheppard MN. Sclerosing mediastinitis: a report on 18 cases. Thorax. 1995;50:280–3.PubMedCentralPubMedCrossRefGoogle Scholar
  64. 64.
    Mathisen DJ, Grillo HC. Clinical manifestation of mediastinal fibrosis and histoplasmosis. Ann Thorac Surg. 1992;54:1053–7.PubMedCrossRefGoogle Scholar
  65. 65.
    Inoue M, Nose N, Nishikawa H, Takahashi M, Zen Y, Kawaguchi M. Successful treatment of sclerosing mediastinitis with a high serum IgG4 level. Gen Thorac Cardiovasc Surg. 2007;55:431–3.PubMedCrossRefGoogle Scholar
  66. 66.
    Yousem SA, Hochholzer L. Pulmonary hyalinizing granuloma. Am J Clin Pathol. 1987;87:1–6.PubMedGoogle Scholar
  67. 67.
    Chapman EM, Gown A, Mazziotta R, Churg A. Pulmonary hyalinizing granuloma with associated elevation in serum and tissue IgG4 occurring in a patient with a history of sarcoidosis. Am J Surg Pathol. 2012;36:774–8.PubMedCrossRefGoogle Scholar
  68. 68.
    Kaleem Z, Fitzpatrick MM, Ritter JH. Primary pulmonary meningioma. Report of a case and review of the literature. Arch Pathol Lab Med. 1997;121:631–6.PubMedGoogle Scholar
  69. 69.
    Fletcher JA, Pinkus GS, Donovan K, Naeem R, Sugarbaker DJ, Mentzer S, Pinkus JL, Longtine J. Clonal rearrangement of chromosome band 6p21 in the mesenchymal component of pulmonary chondroid hamartoma. Cancer Res. 1992;52:6224–8.PubMedGoogle Scholar
  70. 70.
    Mizutani E, Tsuta K, Maeshima AM, Asamura H, Matsuno Y. Minute pulmonary meningothelial-like nodules: clinicopathologic analysis of 121 patients. Hum Pathol. 2009;40:678–82.PubMedCrossRefGoogle Scholar
  71. 71.
    Flieder DB, Koss MN, Nicholson A, Sesterhenn IA, Petras RE, Travis WD. Solitary pulmonary papillomas in adults: a clinicopathologic and in situ hybridization study of 14 cases combined with 27 cases in the literature. Am J Surg Pathol. 1998;22:1328–42.PubMedCrossRefGoogle Scholar
  72. 72.
    Al-Saleem T, Peale AR, Norris CM. Multiple papillomatosis of the lower respiratory tract: clinical and pathologic study of 11 cases. Cancer. 1968;22:1173–84.PubMedCrossRefGoogle Scholar
  73. 73.
    Rezazadeh A, Laber DA, Ghim SJ, Jenson AB, Kloecker G. The role of human papilloma virus in lung cancer: a review of the evidence. Am J Med Sci. 2009;338:64–7.PubMedCrossRefGoogle Scholar
  74. 74.
    Popper HH, Wirnsberger G, Juttner-Smolle FM, Pongratz MG, Sommersgutter M. The predictive value of human papilloma virus (HPV) typing in the prognosis of bronchial squamous cell papillomas. Histopathology. 1992;21:323–30.PubMedCrossRefGoogle Scholar
  75. 75.
    Yuan H, Myers S, Wang J, Zhou D, Woo JA, Kallakury B, Ju A, Bazylewicz M, Carter YM, Albanese C, Grant N, Shad A, Dritschilo A, Liu X, Schlegel R. Use of reprogrammed cells to identify therapy for respiratory papillomatosis. N Engl J Med. 2012;367:1220–7.PubMedCentralPubMedCrossRefGoogle Scholar
  76. 76.
    Abbondanzo SL, Rush W, Bijwaard KE, Koss MN. Nodular lymphoid hyperplasia of the lung: a clinicopathologic study of 14 cases. Am J Surg Pathol. 2000;24:587–97.PubMedCrossRefGoogle Scholar
  77. 77.
    Cordier JF, Loire R, Brune J. Amyloidosis of the lower respiratory tract. Clinical and pathologic features in a series of 21 patients. Chest. 1986;90:827–31.PubMedCrossRefGoogle Scholar
  78. 78.
    Zhang LN, Xue XY, Wang N, Wang JX. Mimicking pulmonary multiple metastatic tumors: a case of primary nodular parenchymal pulmonary amyloidosis with review of the literature. Oncol Lett. 2012;4:1366–70.PubMedCentralPubMedGoogle Scholar
  79. 79.
    Podbielski FJ, Nelson DG, Pearsall Jr GF, Marquez GD, Connolly MM. Nodular pulmonary amyloidosis. J Thorac Cardiovasc Surg. 1997;114:289–91.PubMedCrossRefGoogle Scholar
  80. 80.
    Girard N, Vasiljevic A, Cottin V, Falchero L, Meyronet D, Thivolet-Bejui F, Cordier JF. Respiratory failure with diffuse bronchiectases and cryoglobulinaemia. Eur Respir J. 2008;31:1374–8.PubMedCrossRefGoogle Scholar
  81. 81.
    Colombat M, Stern M, Groussard O, Droz D, Brauner M, Valeyre D, Mal H, Taillé C, Monnet I, Fournier M, Herson S, Danel C. Pulmonary cystic disorder related to light chain deposition disease. Am J Respir Crit Care Med. 2006;173:777–80.PubMedCrossRefGoogle Scholar
  82. 82.
    Bhargava P, Rushin JM, Rusnock EJ, Hefter LG, Franks TJ, Sabnis SG, Travis WD. Pulmonary light chain deposition disease: report of five cases and review of the literature. Am J Surg Pathol. 2007;31:267–76.PubMedCrossRefGoogle Scholar
  83. 83.
    Vassallo R, Ryu JH, Colby TV, et al. Pulmonary Langerhans’-cell histiocytosis. N Engl J Med. 2000;342:1969–78.PubMedCrossRefGoogle Scholar
  84. 84.
    Harari S, Caminati A. Pulmonary Langerhans’ cell histiocytosis. Eur Respir Soc Monogr. 2009;46:155–75.Google Scholar
  85. 85.
    Tazi A. Adult pulmonary Langerhans’ cell histiocytosis. Eur Respir J. 2006;27:1272–85.PubMedCrossRefGoogle Scholar
  86. 86.
    Tazi A, Moreau J, Bergeron A, Dominique S, Hance AJ, Soler P. Evidence that Langerhans cells in adult pulmonary Langerhans cell histiocytosis are mature dendritic cells: importance of the cytokine microenvironment. J Immunol. 1999;163:3511–5.PubMedGoogle Scholar
  87. 87.
    Yu RC, Chu C, Buluwela L, Chu AC. Clonal proliferation of Langerhans cells in Langerhans cell histiocytosis. Lancet. 1994;343:767–8.PubMedCrossRefGoogle Scholar
  88. 88.
    Haroche J, Cohen-Aubart F, Emile JF, Arnaud L, Maksud P, Charlotte F, Cluzel P, Drier A, Hervier B, Benameur N, Besnard S, Donadieu J, Amoura Z. Dramatic efficacy of vemurafenib in both multisystemic and refractory Erdheim-Chester disease and Langerhans cell histiocytosis harboring the BRAF V600E mutation. Blood. 2012. doi: 10.1182/blood-2012-07-446286.Google Scholar
  89. 89.
    Krajicek BJ, Ryu JH, Hartman TE, Lowe VJ, Vassallo R. Abnormal fluorodeoxyglucose PET in pulmonary Langerhans cell histiocytosis. Chest. 2009;135:1542–9.PubMedCrossRefGoogle Scholar
  90. 90.
    Saven A, Burian C. Cladribine activity in adult Langerhans-cell histiocytosis. Blood. 1999;93:4125–30.PubMedGoogle Scholar
  91. 91.
    Lazor R, Etienne-Mastroianni B, Khouatra C, Tazi A, Cottin V, Cordier JF. Progressive diffuse pulmonary Langerhans cell histiocytosis improved by cladribine chemotherapy. Thorax. 2009;64:274–5.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag London 2015

Authors and Affiliations

  • Nicolas Girard
    • 1
    • 2
  • Vincent Cottin
    • 3
  • Jean-François Cordier
    • 3
  1. 1.Pilot Unit for the Management of Rare Intrathoracic Tumors, Department of Respiratory Medicine, Reference Centre for Orphan Pulmonary DiseasesLouis Pradel Hospital, Claude Bernard Lyon 1 University, UMR 754LyonFrance
  2. 2.Department of Respiratory MedicineLouis Pradel Hospital, Hospices Civils de Lyon, UMR 754LyonFrance
  3. 3.Department of Pulmonary Medicine, National Reference Center for Orphan Pulmonary DiseasesHospital Louis Pradel, Hospices Civils de LyonLyonFrance

Personalised recommendations