Langerhans Cell Histiocytosis: A Population-based Study of Anatomical Distribution and Treatment Patterns

Hu, Xianglin; Buhtoiarov, Ilia N.; Wang, Chunmeng; Sun, Zhengwang; Zhu, Qian; Huang, Wending; Yan, Wangjun; Sun, Yangbai

doi:10.1016/j.jbo.2022.100454

Cited by 2 publications

(2 citation statements)

References 27 publications

(26 reference statements)

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“…Eosinophilic granuloma (EG) is a disease in the spectrum of Langerhans cell histiocytosis (or histiocytosis X), usually developing as a single focal osteolytic lesion affecting children or young adults. Though the overall incidence in the general population is low (1–5 cases per million each year) [ 1 ], EG is not uncommon to encounter in neurosurgical practice since the most frequently affected skeletal district is the calvarium . Multiple cases have been reported in a series of pediatric calvarial masses [ 2 , 3 ].…”

Section: Introductionmentioning

confidence: 99%

Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature

Benato¹,

Riva²,

Raneri³

2023

Childs Nerv Syst

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Introduction Eosinophilic granuloma (EG) is the most common form of Langerhans cell histiocytosis, presenting as a single osteolytic lesion of the calvarium. Its diagnosis is based on typical clinical and radiological features. While surgical resection has been the standard treatment for EG, growing evidence favors watchful waiting, as unifocal calvarial lesions appear to frequently undergo spontaneous remission. However, histopathological confirmations of this hypothesis are still very limited. Methods Methods. Here, we report a case of EG with typical clinical and radiological features which, due to intervening circumstances, was resected in a delayed fashion. Moreover, we perform a systematic review of the literature on conservative management of EG. Results In our case, histological examination showed ongoing bone regeneration with no traces of the disease. Through our literature review, we found 47 cases of calvarial EG managed with watchful waiting. No active intervention was required in 43 cases (91%). Four patients (9%) received surgery or chemotherapy due to the persistence/progression of symptoms or family request. Three reports other than ours documented spontaneous disease remission in surgically resected EG upon histopathological examination. Conclusion Our report provides further evidence that watchful waiting can be a reasonable option in the management of single calvarial EG.

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Section: Introductionmentioning

confidence: 99%

Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature

Benato¹,

Riva²,

Raneri³

2023

Childs Nerv Syst

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“…Langerhans cell histiocytosis (LCH) is a rare myeloid precursor cell inflammatory neoplasia driven by mutations in the mitogen-activated protein kinase pathway ( 1 ). LCH usually occurs in children, and it is reported that the incidence rate of LCH is 4.46 cases per million children (<15 years old) every year, and the median age at diagnosis is 4 years old ( 2 , 3 ). From isolated eosinophilic granuloma to extensive diffuse disease with organ dysfunction, LCH shows a series of systemic involvement ( 4 ).…”

Section: Introductionmentioning

confidence: 99%

The baseline metabolism parameters of 18F‑FDG PET/CT as promising prognostic biomarkers in pediatric Langerhans cell histiocytosis

Lu,

Wei,

Wang

et al. 2023

Quant Imaging Med Surg

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Background Langerhans cell histiocytosis (LCH) is a rare myeloid precursor cell inflammatory neoplasia, which agonizes, maims, and even kills patients. Although clinical outcomes have steadily improved over the past decades, the progression/relapse rate of LCH remains high. The purpose of this study was to evaluate the prognostic value of the pre-treatment metabolism parameters of baseline 18 F-fluorodeoxyglucose positron emission tomography/computed tomography ( 18 F‑FDG PET/CT) in children with LCH. Methods This cross-sectional study retrospectively and consecutively included 37 children (24 males and 13 females; median age, 5.1 years; range, 2.4–7.8 years) with pre-treatment 18 F-FDG PET/CT from September 2020 to September 2022 in Nuclear Medicine Department, Beijing friendship hospital, Capital Medical University, Beijing, China. These patients were then all admitted to the hospital and diagnosed with LCH by biopsy, in Hematology Center, Beijing Children’s Hospital, Capital Medical University, Beijing, China. Five metabolism parameters of 18 F-FDG PET/CT were analyzed, including maximum standardized uptake, tumor-to-normal liver standard uptake value ratio, tumor-to-normal bone marrow standard uptake value ratio, sum of metabolic tumor volume (sMTV), and sum of total lesion glycolysis (sTLG) of all lesions. Patients were followed up for at least 1 year or until disease progression/relapse. Univariate and multivariate analyses of progression-free survival was performed. Results During follow-up, 11 (29.7%) patients had disease progression/relapse. Univariate analysis revealed that the risk organ involvement, the treatment response at the 5 th or 11 th week, pre-treatment sMTV, and sTLG were significantly associated with progression-free survival (P=0.024, 0.018, 0.006, 0.006, and 0.042, respectively). Multivariate COX analysis revealed that non-response at the 11 th week, pre-treatment sMTV >32.55 g/cm 3 , and sTLG >98.86 g (P=0.002, 0.020, 0.026, respectively) were risk factors for progression-free survival. Conclusions The baseline metabolism parameters of 18 F-FDG PET/CT could be promising imaging biomarkers for predicting prognosis in children with LCH.

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Langerhans Cell Histiocytosis: A Population-based Study of Anatomical Distribution and Treatment Patterns

Cited by 2 publications

References 27 publications

Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature

Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature

The baseline metabolism parameters of 18F‑FDG PET/CT as promising prognostic biomarkers in pediatric Langerhans cell histiocytosis

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