Langerhans cell histiocytosis: Differences and similarities in long-term outcome of paediatric and adult patients at a single institutional centre

Maia, Raquel Ciuvalschi; Rezende, Lídia Maria Magalhães de; Robaina, Marcela Cristina; Apa, Alexandre Gustavo; Klumb, Claudete Esteves

doi:10.1179/1607845414y.0000000173

Cited by 17 publications

(14 citation statements)

References 53 publications

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“…Although craniofacial involvement is the most common manifestation both in adults and in children, orbital lesions are more frequently found in children while mandibular lesions are more frequently seen in adults. Reactivation episodes and death are more common in adults than in children while the 10-year overall survival is similar in both groups [30]. Treatment modalities include surgery, radiation therapy (RT), chemotherapy (ChT), and intralesional corticosteroid injection [31].…”

Section: Primary Intraosseous Tumorsmentioning

confidence: 99%

Non-odontogenic tumors of the facial bones in children and adolescents: role of multiparametric imaging

et al. 2017

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Tumors of the pediatric facial skeleton represent a major challenge in clinical practice because they can lead to functional impairment, facial deformation, and long-term disfigurement. Their treatment often requires a multidisciplinary approach, and radiologists play a pivotal role in the diagnosis and management of these lesions. Although rare, pediatric tumors arising in the facial bones comprise a wide spectrum of benign and malignant lesions of osteogenic, fibrogenic, hematopoietic, neurogenic, or epithelial origin. The more common lesions include Langerhans cell histiocytosis and osteoma, while rare lesions include inflammatory myofibroblastic and desmoid tumors; juvenile ossifying fibroma; primary intraosseous lymphoma; Ewing sarcoma; and metastases to the facial bones from neuroblastoma, Ewing sarcoma, or retinoblastoma. This article provides a comprehensive approach for the evaluation of children with non-odontogenic tumors of the facial skeleton. Typical findings are discussed with emphasis on the added value of multimodality multiparametric imaging with computed tomography (CT), magnetic resonance imaging (MRI) with diffusion-weighted imaging (DWI), positron emission tomography CT (PET CT), and PET MRI. Key imaging findings and characteristic histologic features of benign and malignant lesions are reviewed and the respective role of each modality for pretherapeutic assessment and post-treatment follow-up. Pitfalls of image interpretation are addressed and how to avoid them.

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Section: Primary Intraosseous Tumorsmentioning

confidence: 99%

Non-odontogenic tumors of the facial bones in children and adolescents: role of multiparametric imaging

et al. 2017

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“…Although with the 3 LCH trials substantial progresses have been made in the treatment of pediatric LCH, this therapeutic strategy is unlikely to be successful in adults, as the only prospective randomized trial evaluating the efficacy of vinblastine/prednisone regimen in adults (LCHA1 trial) was prematurely closed for unacceptable toxicities (vinblastine related neurotoxicity and detrimental effects of prolonged steroid therapy) [ 3 ]. Moreover recent studies seem to indicate that this approach might have lower efficacy in adults compared to children, suggesting that adult and pediatric LCH could harbor different biological characteristics [ 6 , 7 ]. In conclusion, available data do not support the use of pediatric regimens in adult LCH, indicating that the concept of mild induction followed by maintenance therapy probably cannot be translated to adults.…”

Section: Introductionmentioning

confidence: 99%

“…In conclusion, available data do not support the use of pediatric regimens in adult LCH, indicating that the concept of mild induction followed by maintenance therapy probably cannot be translated to adults. Alternative approaches tested in adult LCH include nucleoside analogs such as cytarabine and cladribine (2-CDA) [ 6 , 8 ] but no consensus on the best frontline treatment strategy has been reached yet.…”

Section: Introductionmentioning

confidence: 99%

High efficacy of the MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis, a 20 year experience

et al. 2015

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BackgroundAdult Langerhans cell histiocytosis (LCH) is an orphan disease. Chemotherapy is usually reserved to patients presenting with single system multifocal (SS-m) or multisystem (MS) disease but due to the lack of randomized studies no standard first line therapy has been defined yet. Pediatric regimens based on the vinblastine/prednisone backbone are not well tolerated in adults and probably less effective. We previously demonstrated high efficacy of the dose dense polichemotherapy regimen MACOP-B in 7 adult patients with SS-m or MS-LCH, in terms of high response rate and durable responses. Here we report an update of these data with the purpose of evaluating the long term efficacy of MACOP-B in adult LCH.MethodsClinical data of all adult LCH patients (n = 17) diagnosed and treated at our Institution during the past 20-year period were retrospectively reviewed.ResultsA total of 11 patients (6 with SS-m and 5 with MS-LCH) were treated with MACOP-B from 1995 to 2014. The overall response rate was confirmed to be 100 %, with a complete response of 73 % and a partial response rate of 27 %. Overall progression free survival was 64 %, and disease free survival after achievement of initial CR was 87 %. Overall survival rate was 82 % after 6.7 years of median follow-up.ConclusionsThese data confirm high activity of MACOP-B in adult LCH, indicating that a substantial fraction of patients achieve long lasting responses and can be cured with this therapeutic approach.

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“…Unlike the slight female predominance reported by J. S. Malpas and A. J. Norton in 1996 [27], who also reported a large cohort in the literature, male patients counted more in our cohort. On the other hand, craniofacial osseous lesion is the most affected site in adult LCH patients which is similar to that in pediatric patients [28,29], manifesting with lytic bone lesions. Central nervous system (CNS) is also one of the most affected site in LCH patients, and unfortunately, this involvement always leads to late sequelae that is hard to cure, and may bother patients through their lives.…”

Section: Discussionmentioning

confidence: 53%

The combination of methotrexate and cytosine arabinoside in newly diagnosed adult Langerhans cell histiocytosis: a prospective phase II interventional clinical trial

et al. 2020

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Background: Langerhans Cell Histiocytosis (LCH) is a rare disease puzzling both children and adults, however outcome of adult patients is unfavorable. This prospective interventional trial aims to test the efficacy and safety of the combination of methotrexate and cytosine arabinoside in adult LCH patients. Method: A total of 36 patients enrolled diagnosed with LCH and treated in our center from 1st Jan, 2014 to 30th Jun, 2016. Result: Nineteen patients underwent the detection of BRAF mutation, with a positive rate of 21.1%. The overall response rate was 100%, only 16.7% achieved complete response. The overall regression rate of osseous lesions was 100%. Regression of central nervous system involvement was also favorable. After a median follow-up of 44 months, the estimated event-free survival was 48.9 months, the overall survival rate was 97.2%. The risk organ involvement showed strong prognostic value, EFS was 34.1 or 54.6 months (p = 0.001) in groups with/without risk organ involvement respectively. Neutropenia and thrombocytopenia were the most common adverse effects. Conclusion: The regimen of methotrexate and cytosine arabinoside (MA) is effective and safe in treating adult LCH patients, and timely preventions may be considered for the high incidence of hematological adverse effects. Trial registration: Trial No. NCT02389400 on Clinicaltrials.gov, registered on 10th Mar. 2015.

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Langerhans cell histiocytosis: Differences and similarities in long-term outcome of paediatric and adult patients at a single institutional centre

Abstract: The OS was similar in both groups despite clinical differences between paediatric and adult patients, and higher reactivation and death rates in adults.

Cited by 17 publications

References 53 publications

Non-odontogenic tumors of the facial bones in children and adolescents: role of multiparametric imaging

Non-odontogenic tumors of the facial bones in children and adolescents: role of multiparametric imaging

High efficacy of the MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis, a 20 year experience

The combination of methotrexate and cytosine arabinoside in newly diagnosed adult Langerhans cell histiocytosis: a prospective phase II interventional clinical trial

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