STANDARD CLINICAL PRACTICE RECOMMENDATIONS FOR PEDIATRIC-ONSET LANGERHANS CELL HISTIOCYTOSIS - Version 1.2 - October 30, 2020

Oct 22, 2026

Guidelines for diagnosis, clinical work-up, and treatment of children and adolescents with Langerhans cell histiocytosis (LCH) with a focus on pediatric-onset disease. The document emphasizes responsible use, stating that ESCP guidance is not a clinical trial protocol and must be applied alongside other relevant medical information and local clinical governance. It outlines background and rationale, patient group diagnostic criteria (including histopathology and molecular pathology), imaging approaches, pre-treatment and relapse evaluation, clinical classification, treatment stratification, treatment details, assessment methods, and summary of known adverse events.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages41

Reading Time1 h 43 min

AccessFree to download

Background and Rationale

Background

Patient Group

Diagnostic Criteria

Histopathology

Molecular pathology

Imaging

Radiography

Computed tomography (CT)

MRI

Functional imaging

Diagnostic evaluation and clinical classification pre-treatment and at relapse

Medical history

Physical examination

Laboratory tests and imaging

Definition of organ involvement

Clinical classification

Treatment stratification

Treatment Details

Treatment

Treatment of localized LCH

Treatment of multifocal skeletal and multisystem LCH

Assessments

Response categories of the LCH Working Group of the Histiocyte Society

Disease activity score

Summary of known adverse events associated with treatment recommendation

Steroids (Prednisone, Prednisolone)

Vinblastine

STANDARD CLINICAL PRACTICE RECOMMENDATIONS FOR PEDIATRIC-ONSET LANGERHANS CELL HISTIOCYTOSIS DISCLAIMER: These ESCP guidance documents were produced by the relevant tumour group or specialist committee as recommendations on current best practice. The ESCP guidance documents are not clinical trial protocols. The interpretation and responsibility of the use of ESCP guidance documents lies fully with the user who retains full responsibility for the use of these guidance documents and his actions and (treatment) decisions based thereon, such as, but without limitation thereto: checking and prescribing certain doses, checking prescriptions, etc. A user should never base its decision solely on the content of these guidance documents and should always check any other relevant medical information that is available and make appropriate use of all relevant medical information. These guidance documents have been made publicly available by SIOP Europe – the European Society of Paediatric Oncology and the European Reference Network for Paediatric Oncology (ERN PaedCan) . It is the responsibility of the user who downloads these documents to make sure that: · their use within the Paediatric Clinical Unit / Hospital is approved according to the local clinical governance procedures. · appropriate document control measures are in place to ensure that the most up to date locally approved versions are considered. · any anomalies or discrepancies within the documents are immediately brought to the attention of the relevant special interest group chair and the European Clinical Study Group who has developed the ESCP guidance document. Every care has been taken whilst preparing these documents to ensure that they are free of errors. Nonetheless, SIOP Europe and ERN PaedCan cannot be held liable for possible errors or mistakes in these guidance documents, nor can SIOP Europe and ERN PaedCan be held liable for any kind of damage resulting out of the use of these guidance documents. INTRODUCTORY PAGES • Guidelines for diagnosis, clinical work-up, and treatment of children and adolescents with Langerhans cell histiocytosis • Guidelines for pediatric-onset LCH • Version 1.2 , Oct 30 , 2020 This document has been developed by: the European Consortium for Histiocytosis (ECHO), WP Treatment of Pediatric-onset LCH: Jean Donadieu (France), Jan-Inge Henter (Sweden), Thomas Lehrnbecher (Germany), Milen Minkov (Austria), Elena Sieni (Italy), Vassilios Papadakis (Greece), Karel Svojgr (Czech Republic), Cor van den Bos (The Netherlands), Johannes Visser (UK) . Corresponding authors: Karel Svojgr (Young SIOPE member) & Milen Minkov (President of ECHO) Planned review date: Oct 2023 Table of contents 1. Background and Rationale......................................................................................3 1.1 Background .......................................................................................................3 2. Patient Group ............................................................................................................3 2.1 Diagnostic Criteria .............................................................................................4 2.1.1 Histopathology ...............................................................................................5 2.1.2 Molecular pathology.......................................................................................5 2.2 Imaging ..............................................................................................................6 2.2.1 Radiography ...................................................................................................6 2.2.2 Computed tomography (CT) ..........................................................................6 2.2.3 MRI .................................................................................................................7 2.2.4 Functional imaging ........................

FAQs

What is the scope of these ESCP guidance documents for pediatric-onset Langerhans cell histiocytosis?

They provide recommendations for diagnosis, clinical work-up, and treatment of children and adolescents with Langerhans cell histiocytosis, including pediatric-onset disease. They also cover how to evaluate patients before treatment and at relapse.

Are these guidance documents clinical trial protocols?

No. The document explicitly states that ESCP guidance documents are not clinical trial protocols, and decisions should not be based solely on the guidance.

What diagnostic and assessment areas are covered?

The guidelines include diagnostic criteria such as histopathology and molecular pathology, imaging modalities (radiography, CT, MRI, and functional imaging), clinical classification, and assessment tools such as response categories and a disease activity score.

Oct 22, 2026

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