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Beyond Langerhans Cell Histiocytosis Related to Smoking

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Beyond Langerhans Cell Histiocytosis Related to Smoking Radiología. 2019;61(3):215---224 www.elsevier.es/rx RADIOLOGY THROUGH IMAGES Pulmonary histiocytosis: Beyond Langerhans cell ଝ histiocytosis related to smoking a b a c d b,e,∗ C. Trejo Gallego , J. Bueno , E. Cruces , E.B. Stelow , N. Mancheno˜ , L. Flors a Servicio de Radiología, Hospital Universitario Morales Meseguer, Universidad de Murcia, Murcia, Spain b Department of Radiology and Medical Imaging, University of Virginia Health System, Charlottesville, VA, United States c Department of Pathology, University of Virginia Health System, Charlottesville, VA, United States d Servicio de Anatomía Patológica, Hospital Universitario y Politécnico la Fe, Valencia, Spain e Department of Radiology, One Hospital Dr, University of Missouri Health System, Columbia, MO, United States Received 21 October 2017; accepted 16 November 2018 Available online 24 January 2019 KEYWORDS Abstract Langerhans cells Objective: To review the imaging findings for the different types of pulmonary histiocy- histiocytosis; tosis. In particular, in addition to the well-known pulmonary Langerhans cell histiocytosis Erdheim---Chester related to smoking and its possible appearance in nonsmokers, we focus on non-Langerhans disease; cell histiocytosis in Rosai---Dorfman disease and Erdheim---Chester disease. We also review the Sinus histiocytosis; etiopathogenesis, histology, clinical presentation, and treatment of pulmonary histiocytosis. Computed Conclusion: Langerhans cell histiocytosis, Rosai---Dorfman disease, and Erdheim---Chester dis- tomography ease are idiopathic diseases in which the proliferation and infiltration of histiocytes is the histologic finding that confirms the diagnosis. Langerhans cell histiocytosis manifests as nod- ules and cysts that spare the costophrenic angles; it typically appears in smokers. Although it is uncommon in nonsmokers, Langerhans cell histiocytosis should also be considered in nonsmokers treated with chemotherapy and radiotherapy in whom cavitated nodules appear and should be included in the differential diagnosis together with metastatic disease and opportunistic infec- tions. Rosai---Dorfman disease and Erdheim---Chester disease present with less specific thoracic findings such as adenopathies, interstitial thickening, and pleural effusion. In Erdheim---Chester disease, the characteristic extrathoracic manifestations are usually key for the diagnosis. © 2019 SERAM. Published by Elsevier Espana,˜ S.L.U. All rights reserved. ଝ Please cite this article as: Trejo Gallego C, Bueno J, Cruces E, Stelow EB, Mancheno˜ N, Flors L. Histiocitosis pulmonar: más allá de la histiocitosis de células de Langerhans relacionada con el tabaco. Radiología. 2019;61:215---224. ∗ Corresponding author. E-mail address: fl[email protected] (L. Flors). 2173-5107/© 2019 SERAM. Published by Elsevier Espana,˜ S.L.U. All rights reserved. 216 C. Trejo Gallego et al. PALABRAS CLAVE Histiocitosis pulmonar: más allá de la histiocitosis de células de Langerhans relacionada con el tabaco Histiocitosis de células de Langerhans; Resumen Objetivo: Revisar los hallazgos de imagen de las diferentes histiocitosis pulmonares. En con- Enfermedad de creto, además de la conocida histiocitosis de células de Langerhans relacionada con el tabaco y Erdheim Chester; su posible aparición sin antecedentes de este, la enfermedad de Rosai-Dorfman y la enfermedad Histiocitosis sinusal; Tomografía de Erdheim-Chester. También se revisa su etiopatogenia, histología, clínica y tratamiento. Conclusión: computarizada La histiocitosis de células de Langerhans, la enfermedad de Rosai-Dorfman y la enfermedad de Erdheim-Chester son un conjunto de patologías de causa idiopática en las que la proliferación e infiltración de histiocitos es el hallazgo anatomopatológico diagnóstico. La histiocitosis de células de Langerhans se manifiesta en forma de nódulos y quistes que respetan los ángulos costofrénicos, característicamente en pacientes fumadores. Aunque es poco fre- cuente, debe pensarse en esta entidad en pacientes no fumadores, en tratamiento quimio y radioterapéutico, con nódulos cavitados de nueva aparición e incluirse en el diagnóstico diferencial junto con la enfermedad metastásica y la infección oportunista. La enfermedad de Rosai-Dorfman y la enfermedad de Erdheim-Chester se presentan con hallazgos torácicos más inespecíficos, como adenopatías, engrosamiento intersticial y derrame pleural. En la enfer- medad de Erdheim-Chester, las características manifestaciones extratorácicas suelen ser claves en el diagnóstico. © 2019 SERAM. Publicado por Elsevier Espana,˜ S.L.U. Todos los derechos reservados. Introduction Table 1 Summary of primary histiocytic disorders of non- neoplastic (or unknown) aetiology and those with malignant behaviour. Histiocytosis is a group of diseases characterised by abnor- mal proliferation of histiocytes with infiltration of a specific Primary histiocytic disorders organ. This group of disorders derives from the phago- Non-neoplastic or unknown aetiology cytic mononuclear system, the main roles of which are Langerhans cell histiocytosis a phagocytosis of foreign material, antigen processing and Adult form (lung involvement) antigen presentation to lymphocytes. The mononuclear Paediatric form (multisystem involvement) phagocyte system includes macrophages and dendritic cells. Letterer---Siwe disease Macrophages are distributed all around the body, but they Hand---Schüller---Christian disease are found mainly in mucous membranes and other poten- Hashimoto---Pritzker syndrome a tial gateways for microorganisms, where they function as Rosai---Dorfman disease a innate and specific immunity. In the lungs, they are usually Erdheim---Chester disease present in the alveoli. Dendritic cells are located primar- Histiocytic neoplasms ily in the skin, mucous membranes, bone marrow, spleen, Follicular dendritic cell tumour thymus gland and lymph nodes, and, although they have a Histiocytic sarcoma less important role in phagocytosis, they are more impor- Langerhans cell sarcoma 1 tant at the beginning of the T cell-dependent response. Reticulum cell sarcoma Langerhans cells (LC) are specifically derived from dendritic a Primary non-neoplastic disorders which affect the adult and cells and are found in the epidermal layer of the skin. 8,9 are discussed in this manuscript. Langerhans cell histiocytosis (LCH), which is typically asso- ciated with smoking and manifests itself with distinctive we have focused on primary non-neoplastic pulmonary his- 8,9 radiological findings, is the most common and well-known tiocytosis in adults (Table 1). condition in which these cells are involved. An associa- The aim of our study was to review the imaging find- tion has been reported between LCH and certain types of ings for the different thoracic manifestations of the most 2---5 cancer, such as Hodgkin’s lymphoma, and after treat- common types of histiocytosis, and provide the keys to an 6,7 ment with chemotherapy and radiotherapy. LCH is not accurate diagnosis. We also describe the aetiopathogenesis, the only histiocytosis which affects the respiratory system. histology, clinical presentation and treatment. Other lesser-known types of pulmonary histiocytosis are Rosai---Dorfman disease (RDD) (derived from macrophages) Langerhans cell histiocytosis and Erdheim---Chester disease (ECD) (derived from non- Langerhans dendritic cells). The secondary and malignant LCH is the most common disorder associated with dendritic forms of histiocytosis are not discussed in this manuscript; 10 cells. Cell behaviour in LCH varies according to the age Pulmonary histiocytosis: Beyond Langerhans cells related to smoking 217 × Figure 1 Histology image stained with haematoxylin---eosin (H---E 20 ). (a) Star-shaped cellular nodule (asterisk). (b) Histolog- × ical image at higher magnification (H---E 200 ) showing the lesion to be predominantly composed of Langerhans cells (arrow) in combination with eosinophils and other inflammatory cells. × Figure 2 Panoramic histological image stained with haematoxylin---eosin (H---E 2.5 ). (a) Spiculated nodule (asterisk) with irregular borders adjacent to a bronchus. This dense cellular aggregate is composed mainly of Langerhans cells and some eosinophils. (b) × Higher magnification histological image (H---E 20 ) showing infiltrates of Langerhans cells (arrows), characterised by their large size, oval shape, kidney-shaped nucleus and granular inclusion bodies (Birbeck granules) which are accompanied by eosinophilic × × infiltration (arrowheads). (c) Immunohistochemical staining for CD-1a (2.5 ) and S-100 (2.5 ). (d) Image showing extensive and intense cytoplasmic positivity in the Langerhans cells which confirms the diagnosis of Langerhans cell histiocytosis (same patient as in Fig. 5). of the patient. In adults, the pathological LC behave reac- In this article, we focus on LCH with lung involvement in tively, with no neoplastic growth, and the most commonly, adults. and usually the only, affected organ is the lung. In contrast, in childhood, proliferation of pathological LC is charac- 8 terised by their clonal neoplasm behaviour and multisystem Aetiopathogenesis involvement. Letterer---Siwe and Hand---Schüller---Christian diseases and Hashimoto---Pritzker syndrome are LCH with Although the aetiology is unknown, a possible antigenic multisystem involvement which typically begin in childhood. cause, somatic mutations or infectious aetiology have Lung involvement is rare in these cases and, if it does occur, 11,12 all been suggested. LCH occurs almost exclusively in is part of the
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