Thymus and mediastinal node involvement in childhood langerhans cell histiocytosis: Long‐term follow‐up from the French national cohort
Identifieur interne : 001467 ( Main/Exploration ); précédent : 001466; suivant : 001468Thymus and mediastinal node involvement in childhood langerhans cell histiocytosis: Long‐term follow‐up from the French national cohort
Auteurs : Stephane Ducassou [France] ; Fanny Seyrig [France] ; Caroline Thomas [France] ; Anne Lambilliotte [France] ; Perrine Marec-Berard [France] ; Claire Berger [France] ; Genevieve Plat [France] ; Laurence Brugiere [France] ; Marie Ouache [France] ; Mohamed Barkaoui [France] ; Corinne Armari-Alla [France] ; Patrick Lutz [France] ; Guy Leverger [France] ; Xavier Rialland [France] ; Ludovic Mansuy [France] ; Helene Pacquement [France] ; Eric Jeziorski [France] ; Virginie Gandemer [France] ; François Chalard [France] ; Jean François Chateil [France] ; Abdellatif Tazi [France] ; Jean François Emile [France] ; Jean Donadieu [France]Source :
- Pediatric Blood & Cancer [ 1545-5009 ] ; 2013-11.
English descriptors
- Teeft :
- Arch pathol, Cava, Cell histiocytosis, Chest pain, Clinical symptoms, Cyst, High risk, Histiocytosis, Hopital, Incidental thymectomy, Initial diagnosis, Langerhans, Langerhans cell histiocytosis, Lymph, Median, Mediastinal, Mediastinal involvement, Mediastinal nodes, Myasthenia gravis, Natural history, Node, Oncologie, Oncologie pediatrique, Organ involvement, Overall survival, Pathol, Pediatr, Pediatr blood cancer, Pediatr oncol, Pediatr radiol, Pediatrique, Present study, Radiol, Radiological, Relapse, Respiratory distress, Respiratory symptoms, Risk organs, Service oncologie pediatrique, Severe infection, Steroid, Systemic therapy, Thymic, Thymic involvement, Thymus, Thymus enlargement, Thymus involvement, Tracheal, Tracheal compression, Vinblastine.
Abstract
Mediastinal involvement (MI) in Langerhans cell histiocytosis (LCH) has been rarely reported. Here, we describe the clinical, radiological, and biological presentation, and the outcome of childhood LCH with MI.
From the French LCH register, which includes 1,423 patients aged less than 18 years, we retrieved the medical charts of patients with mediastinal enlargement detected on chest X‐rays.
Thirty‐seven patients were retrieved, including 18 males; median age of diagnosis was 0.7 years, and median follow‐up time was 6.2 years. The prevalence of MI varied with the age at diagnosis, ranging from 7% below 1 year old to less than 1% at >5 years. Thirteen cases (35%) were diagnosed because of MI‐related symptoms, including respiratory distress (N = 4), superior venous cava syndrome (N = 2), and/or cough and polypnea (N = 10). CT scans performed in 32 cases at diagnosis showed tracheal compression (N = 5), cava thrombosis (N = 2), and/or calcification (N = 16). All patients presented multi‐system disease at LCH diagnosis, and 35/37 were initially treated with vinblastine and corticosteroids. Death occurred in five cases, due to MI (N = 1) or hematological refractory involvement (N = 4). The overall 5‐year survival was 87.1%, and immunodeficiency was not detected as a sequel.
MI in LCH mainly occurs in young children, and diagnosis was based on CT showing thymus enlargement and calcifications. Pediatr Blood Cancer 2013;60:1759–1765. © 2013 Wiley Periodicals, Inc.
Url:
DOI: 10.1002/pbc.24603
Affiliations:
- France
- Alsace (région administrative), Aquitaine, Auvergne-Rhône-Alpes, Grand Est, Hauts-de-France, Languedoc-Roussillon, Lorraine (région), Nord-Pas-de-Calais, Nouvelle-Aquitaine, Occitanie (région administrative), Pays de la Loire, Rhône-Alpes, Région Bretagne, Île-de-France
- Angers, Bordeaux, Grenoble, Lille, Lyon, Montpellier, Nancy, Nantes, Paris, Rennes, Strasbourg
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D'oncologie Pédiatrique, Institut Gustave Roussy, Villejuif</wicri:regionArea><wicri:noRegion>Villejuif</wicri:noRegion><wicri:noRegion>Villejuif</wicri:noRegion></affiliation></author><author><name sortKey="Ouache, Marie" sort="Ouache, Marie" uniqKey="Ouache M" first="Marie" last="Ouache">Marie Ouache</name><affiliation wicri:level="3"><country xml:lang="fr">France</country><wicri:regionArea>Service D'hématologie Pédiatrique, Hopital Robert Débré, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Barkaoui, Mohamed" sort="Barkaoui, Mohamed" uniqKey="Barkaoui M" first="Mohamed" last="Barkaoui">Mohamed Barkaoui</name><affiliation wicri:level="3"><country xml:lang="fr">France</country><wicri:regionArea>Service D'hémato Oncologie Pédiatrique, Centre de référence des histiocytoses, registre des histiocytoses, APHP 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Trousseau, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Armari Lla, Corinne" sort="Armari Lla, Corinne" uniqKey="Armari Lla C" first="Corinne" last="Armari-Alla">Corinne Armari-Alla</name><affiliation wicri:level="3"><country xml:lang="fr">France</country><wicri:regionArea>Service de Pédiatrie, Unité D'hémato Oncologie Pédiatrique, CHU Michallon, Grenoble</wicri:regionArea><placeName><region type="region">Auvergne-Rhône-Alpes</region><region type="old region">Rhône-Alpes</region><settlement type="city">Grenoble</settlement></placeName></affiliation></author><author><name sortKey="Lutz, Patrick" sort="Lutz, Patrick" uniqKey="Lutz P" first="Patrick" last="Lutz">Patrick Lutz</name><affiliation wicri:level="3"><country xml:lang="fr">France</country><wicri:regionArea>Service D'hémato Oncologie Pédiatrique, CHU de Strasbourg, Strasbourg</wicri:regionArea><placeName><region type="region">Grand Est</region><region type="old region">Alsace (région administrative)</region><settlement type="city">Strasbourg</settlement></placeName></affiliation></author><author><name sortKey="Leverger, Guy" sort="Leverger, Guy" uniqKey="Leverger G" first="Guy" last="Leverger">Guy Leverger</name><affiliation wicri:level="3"><country xml:lang="fr">France</country><wicri:regionArea>Service D'hémato Oncologie Pédiatrique, Centre de référence des histiocytoses, registre des histiocytoses, APHP Hôpital A. Trousseau, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Rialland, Xavier" sort="Rialland, Xavier" uniqKey="Rialland X" first="Xavier" last="Rialland">Xavier Rialland</name><affiliation wicri:level="3"><country xml:lang="fr">France</country><wicri:regionArea>Service de Pédiatrie, Unité d'hémato Oncologie Pédiatrique, CHU d'Angers, Angers</wicri:regionArea><placeName><region type="region">Pays de la Loire</region><region type="old region">Pays de la Loire</region><settlement type="city">Angers</settlement></placeName></affiliation></author><author><name sortKey="Mansuy, Ludovic" sort="Mansuy, Ludovic" uniqKey="Mansuy L" first="Ludovic" last="Mansuy">Ludovic Mansuy</name><affiliation wicri:level="3"><country xml:lang="fr">France</country><wicri:regionArea>Service de Médecine Infantile II, CHU de Brabois, Nancy</wicri:regionArea><placeName><region type="region">Grand Est</region><region type="old region">Lorraine (région)</region><settlement type="city">Nancy</settlement></placeName></affiliation></author><author><name sortKey="Pacquement, Helene" sort="Pacquement, Helene" uniqKey="Pacquement H" first="Helene" last="Pacquement">Helene Pacquement</name><affiliation wicri:level="3"><country xml:lang="fr">France</country><wicri:regionArea>Service de Pédiatrie, Institut Curie, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Jeziorski, Eric" sort="Jeziorski, Eric" uniqKey="Jeziorski E" first="Eric" last="Jeziorski">Eric Jeziorski</name><affiliation wicri:level="3"><country xml:lang="fr">France</country><wicri:regionArea>Service de Médecine Infantile, Hopital Arnaud de Villeneuve, CHU de Montpellier, Montpellier</wicri:regionArea><placeName><region type="region">Occitanie (région administrative)</region><region type="old region">Languedoc-Roussillon</region><settlement type="city">Montpellier</settlement></placeName></affiliation></author><author><name sortKey="Gandemer, Virginie" sort="Gandemer, Virginie" uniqKey="Gandemer V" first="Virginie" last="Gandemer">Virginie Gandemer</name><affiliation wicri:level="3"><country xml:lang="fr">France</country><wicri:regionArea>Service D'hémato Oncologie Pédiatrique, Hopital Sud CHU, Rennes</wicri:regionArea><placeName><region type="region">Région Bretagne</region><region type="old region">Région Bretagne</region><settlement type="city">Rennes</settlement></placeName></affiliation></author><author><name sortKey="Chalard, Francois" sort="Chalard, Francois" uniqKey="Chalard F" first="François" last="Chalard">François Chalard</name><affiliation wicri:level="3"><country xml:lang="fr">France</country><wicri:regionArea>Service de Radiologie APHP, Hopital Trousseau, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Chateil, Jean Francois" sort="Chateil, Jean Francois" uniqKey="Chateil J" first="Jean François" last="Chateil">Jean François Chateil</name><affiliation wicri:level="1"><country xml:lang="fr">France</country><wicri:regionArea>Service Radiologie Pédiatrique, CHU Bordeaux</wicri:regionArea><wicri:noRegion>CHU Bordeaux</wicri:noRegion><wicri:noRegion>CHU Bordeaux</wicri:noRegion></affiliation></author><author><name sortKey="Tazi, Abdellatif" sort="Tazi, Abdellatif" uniqKey="Tazi A" first="Abdellatif" last="Tazi">Abdellatif Tazi</name><affiliation wicri:level="3"><country xml:lang="fr">France</country><wicri:regionArea>Service de Pneumologie, Centre de référence des histiocytoses, APHP Hôpital Saint Louis, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Emile, Jean Francois" sort="Emile, Jean Francois" uniqKey="Emile J" first="Jean François" last="Emile">Jean François Emile</name><affiliation wicri:level="1"><country xml:lang="fr">France</country><wicri:regionArea>Laboratoire D'anatomie et Cytologie Pathologique, APHP Hopital A Paré, Boulognes</wicri:regionArea><wicri:noRegion>Boulognes</wicri:noRegion><wicri:noRegion>Boulognes</wicri:noRegion></affiliation></author><author><name sortKey="Donadieu, Jean" sort="Donadieu, Jean" uniqKey="Donadieu J" first="Jean" last="Donadieu">Jean Donadieu</name><affiliation wicri:level="3"><country xml:lang="fr">France</country><wicri:regionArea>Service D'hémato Oncologie Pédiatrique, Centre de référence des histiocytoses, registre des histiocytoses, APHP Hôpital A. Trousseau, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author></analytic><monogr></monogr><series><title level="j" type="main">Pediatric Blood & Cancer</title><title level="j" type="alt">PEDIATRIC BLOOD AND CANCER</title><idno type="ISSN">1545-5009</idno><idno type="eISSN">1545-5017</idno><imprint><biblScope unit="vol">60</biblScope><biblScope unit="issue">11</biblScope><biblScope unit="page" from="1759">1759</biblScope><biblScope unit="page" to="1765">1765</biblScope><biblScope unit="page-count">7</biblScope><date type="published" when="2013-11">2013-11</date></imprint><idno type="ISSN">1545-5009</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">1545-5009</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="Teeft" xml:lang="en"><term>Arch pathol</term><term>Cava</term><term>Cell histiocytosis</term><term>Chest pain</term><term>Clinical symptoms</term><term>Cyst</term><term>High risk</term><term>Histiocytosis</term><term>Hopital</term><term>Incidental thymectomy</term><term>Initial diagnosis</term><term>Langerhans</term><term>Langerhans cell histiocytosis</term><term>Lymph</term><term>Median</term><term>Mediastinal</term><term>Mediastinal involvement</term><term>Mediastinal nodes</term><term>Myasthenia gravis</term><term>Natural history</term><term>Node</term><term>Oncologie</term><term>Oncologie pediatrique</term><term>Organ involvement</term><term>Overall survival</term><term>Pathol</term><term>Pediatr</term><term>Pediatr blood cancer</term><term>Pediatr oncol</term><term>Pediatr radiol</term><term>Pediatrique</term><term>Present study</term><term>Radiol</term><term>Radiological</term><term>Relapse</term><term>Respiratory distress</term><term>Respiratory symptoms</term><term>Risk organs</term><term>Service oncologie pediatrique</term><term>Severe infection</term><term>Steroid</term><term>Systemic therapy</term><term>Thymic</term><term>Thymic involvement</term><term>Thymus</term><term>Thymus enlargement</term><term>Thymus involvement</term><term>Tracheal</term><term>Tracheal compression</term><term>Vinblastine</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract">Mediastinal involvement (MI) in Langerhans cell histiocytosis (LCH) has been rarely reported. Here, we describe the clinical, radiological, and biological presentation, and the outcome of childhood LCH with MI.</div><div type="abstract">From the French LCH register, which includes 1,423 patients aged less than 18 years, we retrieved the medical charts of patients with mediastinal enlargement detected on chest X‐rays.</div><div type="abstract">Thirty‐seven patients were retrieved, including 18 males; median age of diagnosis was 0.7 years, and median follow‐up time was 6.2 years. The prevalence of MI varied with the age at diagnosis, ranging from 7% below 1 year old to less than 1% at >5 years. Thirteen cases (35%) were diagnosed because of MI‐related symptoms, including respiratory distress (N = 4), superior venous cava syndrome (N = 2), and/or cough and polypnea (N = 10). CT scans performed in 32 cases at diagnosis showed tracheal compression (N = 5), cava thrombosis (N = 2), and/or calcification (N = 16). All patients presented multi‐system disease at LCH diagnosis, and 35/37 were initially treated with vinblastine and corticosteroids. Death occurred in five cases, due to MI (N = 1) or hematological refractory involvement (N = 4). The overall 5‐year survival was 87.1%, and immunodeficiency was not detected as a sequel.</div><div type="abstract">MI in LCH mainly occurs in young children, and diagnosis was based on CT showing thymus enlargement and calcifications. Pediatr Blood Cancer 2013;60:1759–1765. © 2013 Wiley Periodicals, Inc.</div></front></TEI><affiliations><list><country><li>France</li></country><region><li>Alsace (région administrative)</li><li>Aquitaine</li><li>Auvergne-Rhône-Alpes</li><li>Grand Est</li><li>Hauts-de-France</li><li>Languedoc-Roussillon</li><li>Lorraine (région)</li><li>Nord-Pas-de-Calais</li><li>Nouvelle-Aquitaine</li><li>Occitanie (région administrative)</li><li>Pays de la Loire</li><li>Rhône-Alpes</li><li>Région Bretagne</li><li>Île-de-France</li></region><settlement><li>Angers</li><li>Bordeaux</li><li>Grenoble</li><li>Lille</li><li>Lyon</li><li>Montpellier</li><li>Nancy</li><li>Nantes</li><li>Paris</li><li>Rennes</li><li>Strasbourg</li></settlement></list><tree><country name="France"><region name="Grand Est"><name sortKey="Ducassou, Stephane" sort="Ducassou, Stephane" uniqKey="Ducassou S" first="Stephane" last="Ducassou">Stephane Ducassou</name></region><name sortKey="Armari Lla, Corinne" sort="Armari Lla, Corinne" uniqKey="Armari Lla C" first="Corinne" last="Armari-Alla">Corinne Armari-Alla</name><name sortKey="Barkaoui, Mohamed" sort="Barkaoui, Mohamed" uniqKey="Barkaoui M" first="Mohamed" last="Barkaoui">Mohamed Barkaoui</name><name sortKey="Berger, Claire" sort="Berger, Claire" uniqKey="Berger C" first="Claire" last="Berger">Claire Berger</name><name sortKey="Brugiere, Laurence" sort="Brugiere, Laurence" uniqKey="Brugiere L" first="Laurence" last="Brugiere">Laurence Brugiere</name><name sortKey="Chalard, Francois" sort="Chalard, Francois" uniqKey="Chalard F" first="François" last="Chalard">François Chalard</name><name sortKey="Chateil, Jean Francois" sort="Chateil, Jean Francois" uniqKey="Chateil J" first="Jean François" last="Chateil">Jean François Chateil</name><name sortKey="Donadieu, Jean" sort="Donadieu, Jean" uniqKey="Donadieu J" first="Jean" last="Donadieu">Jean Donadieu</name><name sortKey="Ducassou, Stephane" sort="Ducassou, Stephane" uniqKey="Ducassou S" first="Stephane" last="Ducassou">Stephane Ducassou</name><name sortKey="Ducassou, Stephane" sort="Ducassou, Stephane" uniqKey="Ducassou S" first="Stephane" last="Ducassou">Stephane Ducassou</name><name sortKey="Emile, Jean Francois" sort="Emile, Jean Francois" uniqKey="Emile J" first="Jean François" last="Emile">Jean François Emile</name><name sortKey="Gandemer, Virginie" sort="Gandemer, Virginie" uniqKey="Gandemer V" first="Virginie" last="Gandemer">Virginie Gandemer</name><name sortKey="Jeziorski, Eric" sort="Jeziorski, Eric" uniqKey="Jeziorski E" first="Eric" last="Jeziorski">Eric Jeziorski</name><name sortKey="Lambilliotte, Anne" sort="Lambilliotte, Anne" uniqKey="Lambilliotte A" first="Anne" last="Lambilliotte">Anne Lambilliotte</name><name sortKey="Leverger, Guy" sort="Leverger, Guy" uniqKey="Leverger G" first="Guy" last="Leverger">Guy Leverger</name><name sortKey="Lutz, Patrick" sort="Lutz, Patrick" uniqKey="Lutz P" first="Patrick" last="Lutz">Patrick Lutz</name><name sortKey="Mansuy, Ludovic" sort="Mansuy, Ludovic" uniqKey="Mansuy L" first="Ludovic" last="Mansuy">Ludovic Mansuy</name><name sortKey="Marec Erard, Perrine" sort="Marec Erard, Perrine" uniqKey="Marec Erard P" first="Perrine" last="Marec-Berard">Perrine Marec-Berard</name><name sortKey="Ouache, Marie" sort="Ouache, Marie" uniqKey="Ouache M" first="Marie" last="Ouache">Marie Ouache</name><name sortKey="Pacquement, Helene" sort="Pacquement, Helene" uniqKey="Pacquement H" first="Helene" last="Pacquement">Helene Pacquement</name><name sortKey="Plat, Genevieve" sort="Plat, Genevieve" uniqKey="Plat G" first="Genevieve" last="Plat">Genevieve Plat</name><name sortKey="Rialland, Xavier" sort="Rialland, Xavier" uniqKey="Rialland X" first="Xavier" last="Rialland">Xavier Rialland</name><name sortKey="Seyrig, Fanny" sort="Seyrig, Fanny" uniqKey="Seyrig F" first="Fanny" last="Seyrig">Fanny Seyrig</name><name sortKey="Tazi, Abdellatif" sort="Tazi, Abdellatif" uniqKey="Tazi A" first="Abdellatif" last="Tazi">Abdellatif Tazi</name><name sortKey="Thomas, Caroline" sort="Thomas, Caroline" uniqKey="Thomas C" first="Caroline" last="Thomas">Caroline Thomas</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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