From Cancer Mimicking Orphan Lung Disease to Orphan Thoracic Oncology

  • Nicolas Girard
  • Vincent Cottin
  • Jean-François Cordier


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.


Lung cancer Rare disease Pseudotumor Inflammation Sarcoma Lymphoma 


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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

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