DOI: 10.1007/s00259-016-3521-3Pages: 598-610

18F-fluorodeoxyglucose positron emission tomography-computed tomography in the management of adult multisystem Langerhans cell histiocytosis

1. Université Paris Diderot

2. Assistance Publique-Hôpitaux de Paris, Centre National de Référence de l’Histiocytose Langerhansienne, Service de Pneumologie, Hôpital Saint-Louis

3. Assistance Publique-Hôpitaux de Paris, Service de Médecine Nucléaire, Hôpital Saint-Louis

4. Assistance Publique-Hôpitaux de Paris, Service de Radiologie, Hôpital Saint-Louis

5. Assistance Publique-Hôpitaux de Paris, Service de Biostatistique et Information Médicale, Hôpital Saint-Louis

6. Biostatistics and Clinical Epidemiology Research Team, U1153 CRESS

Correspondence to:
Abdellatif Tazi
Tel: 33 1 42 49 96 18
Email: abdellatif.tazi@aphp.fr

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Abstract

Purpose

The standard evaluation of multisystem Langerhans cell histiocytosis (LCH) includes a clinical evaluation, laboratory tests and a skeleton/skull X-ray survey, with chest high-resolution computed tomography (HRCT) in the case of pulmonary involvement. Preliminary reports suggest that 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) may be useful for evaluating patients with LCH.

Methods

Fourteen consecutive adult patients with multisystem LCH were included in this retrospective study, and were evaluated using standard procedures and 18F-FDG PET-CT. The two sets of findings were compared both at baseline and during follow-up. Serial HRCT and pulmonary function tests were used to evaluate outcome in patients with lung involvement.

Results

At the baseline evaluation, PET-CT identified every LCH localization found with the standard evaluation (except a mild cecum infiltration). PET-CT showed additional lesions in seven patients, mostly involving bones, and differentiated inactive from active lesions. Thyroid 18F-FDG uptake was identified in three cases. No pituitary stalk 18F-FDG uptake was observed in patients with pituitary LCH. Only 3/12 (25 %) patients with pulmonary LCH displayed moderate pulmonary 18F-FDG uptake. During follow-up, variations (≥50 % of maximum standardized uptake) in bone 18F-FDG uptake intensity were correlated with disease state and response to treatment. The absence of lung 18F-FDG uptake did not preclude lung function improvement after treatment.

Conclusions

Except for cases with pulmonary and pituitary involvement, 18F-FDG PET-CT could replace the standard evaluation for staging of adult patients with multisystem LCH. Serial PET-CT scans are useful for evaluating treatment responses, particularly in cases with bone LCH involvement.

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  • Accepted: Sep 7, 2016
  • Online: Sep 20, 2016

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