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Lung tumor

Benign tumors

Langerhans cell histiocytosis

Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 7 January 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Neoplastic disorder of Langerhans cells; most common pulmonary histocytic lesion (Arch Pathol Lab Med 2008;132:1171)
● Also called eosinophilic granuloma, Langerhans cell granulomatosis, histiocytosis X (“H-X”), Hand-Schuller-Christian disease, Letterer-Siwe disease
● Usually ages 20-39 years
● Strongly associated with smokers
● 50% of cases only involve lung; 20% with multicentric disease (bone, skin, lymph nodes, spleen, pituitary) have lung involvement
● Often associated with pneumothorax, Pneumocystis carinii pneumonia
● Lung biopsy is necessary for a definitive diagnosis, although may not be required if imaging findings are highly characteristic (Orphanet J Rare Dis 2012 Mar 19;7:16)
● Usually lung disease resolves or stabilizes, but 10-20% may progress to respiratory failure

Case reports
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● 40 year old woman with Stage IV diffuse large B cell lymphoma (Arch Pathol Lab Med 2002;126:747)

Gross description
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● Lesion of upper lobes, local or diffuse, with nodules and cavitary lesions and late honeycombing

Micro description
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● Interstitial scarring with nodular aggregates of Langerhans cells with a bronchiolocentric distribution
● Langerhans cells have abundant eosinophilic cytoplasm and grooved nuclei with indented nuclear membranes
● Also prominent eosinophils and mesothelial cells
● Frequent hemosiderin, necrosis, alveolar lining cell hyperplasia, pigmented alveolar macrophages
● Variable vasculitis
● Older lesions have fewer eosinophils and more interstitial fibrosis
● Sarcomatous variant has significant atypia and mitotic figures

Micro images
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Various images


Fibrotic nodule with histiocyte-like cells

Positive stains
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● CD1a, S100, HLA-DR

Electron microscopy description
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● Birbeck’s granules (pentilaminar intracytoplasmic structures, tennis racket shaped)

Electron microscopy images
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Birbeck granules, not necessary from lung


Binucleated Langerhans cells, from orbit

Differential diagnosis
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● Eosinophilic pleuritis: no Langerhans cell, although mesothelial cells may appear similar
● Reactive Langerhans cells in inflammatory conditions: no sheets or groups of Langerhans cells
● Desquamative interstitial pneumonitis

End of Lung tumor > Benign tumors > Langerhans cell histiocytosis

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