Chest computed tomography findings for a cohort of children with pulmonary Langerhans cell histiocytosis

Abstract

Objective This study was undertaken to describe the spectrum of lung computed‐tomography (CT) findings in children with pulmonary Langerhans cell histiocytosis (PLCH) and to evaluate for this population the CT‐scan nodule and cyst scores proposed by adult pulmonologists at diagnosis and during follow‐up. Methods Among 175 children with PLCH identified in the French national population‐based Langerhans cell histiocytosis cohort, 60 were retrospectively selected by the availability of CT for a central review by three pediatric radiologists. These 60 patients are representative of childhood PLCH for almost all clinical aspects, except a lower percentage of risk organ involvement (38% vs 54%; P = 0.05). Results The 60 children's chest CT scans (n = 218) were reviewed. At diagnosis, 63% of them had nodules, 53% had cysts, and 29% had both. The percentages of patients with nodules or cysts increased from diagnosis to peak disease activity, respectively, from 63% to 73% and from 53% to 66%. The costophrenic angle was involved in 71%. Patients with pneumothorax (25%) had a higher median cyst score. Alveolar consolidation was observed in 34%. Patients with low CT‐scan nodule and cyst scores had no long‐term pulmonary sequelae. Conclusions Well‐known characteristics of adult PLCH (nodules and cysts) were observed in children. The chest CT scores proposed by adult pulmonologists could easily be applied to childhood PLCH. Lesions in children, unlike those in adults, are frequently located near the costophrenic angles. Alveolar consolidation might be considered an atypical feature of childhood PLCH.

Clinicogenomic associations in childhood Langerhans cell histiocytosis: an international cohort study

“…Two of 3 patients with BRAF rearrangements had SS-bone LCH without progression or relapse during their follow-up (5.4 or 27.5 years); the remaining patient with a BICD2 :: BRAF fusion had MS-RO − disease with uncommon large tumors in both lungs ( Figure 4 G) instead of small nodules and cysts typical for pulmonary LCH. 54 , 55 , 56 This patient had progressive disease despite 2 lines of chemotherapy and recently received MEK inhibition (trametinib) with a complete metabolic response after 6 months of treatment. The ARAF and KRAS mutations were confined to patients with SS-bone LCH.…”

Pulmonary manifestations of Erdheim–Chester disease: clinical characteristics, outcomes and comparison with Langerhans cell histiocytosis

“…Therefore, we applied the CT score system of pulmonary alveolar proteinosis (PAP), 20 which is characterized by interlobular septal thickening and ground-glass opacities being similar with ECD to some extent to grade the severity of our ECD patients instead of the widely used CT score scale for pulmonary LCH. 27,28 The CT lesions of ECD patients are uniformly distributed, while for LCH patients, the lesions were mainly distributed in the anterior, apical, and peripheral segments of the lungs. Furthermore, restrictive disorder was more predominant in ECD than in LCH patients, suggesting that ECD patients with pulmonary involvement may have more intense ILD lesions.…”

“…The ILD scale is not pertinent and LCH is more likely a bronchiolitis, while the typical CT findings of pulmonary ECD are interstitial lung diseases, including interlobular septal thickening and opacities. Therefore, we applied the CT score system of pulmonary alveolar proteinosis (PAP), 20 which is characterized by interlobular septal thickening and ground‐glass opacities being similar with ECD to some extent to grade the severity of our ECD patients instead of the widely used CT score scale for pulmonary LCH 27,28 . The CT lesions of ECD patients are uniformly distributed, while for LCH patients, the lesions were mainly distributed in the anterior, apical, and peripheral segments of the lungs.…”

“…Although there is a possibility that the lung lesions observed might have not been manifestations of ECD, lung involvement was confirmed only when extrapulmonary ECD was pathologically confirmed and with compatible chest CT findings without alternative explanation. Furthermore, we did not apply the recognized CT scoring system on our LCH patients, 27,28 which will be used in our future study on LCH.…”

Dural involvement in central nervous system langerhans cells histiocytosis (LCH) on FDG PET/CT: Case report and review of CNS manifestations of LCH on PET/CT

“…In addition to detailed clinical assessment and laboratory panels, various imaging modalities such as magnetic resonance imaging (MRI), ultrasound, CT, X-ray, and bone scintigraphy are utilized both for initial staging of LCH and as well as treatment response assessment [ 3 , [15] , [16] , [17] , [18] ]. Certain imaging modalities offer diagnostic advantages when evaluating specific organ systems.…”