1.A study of pathological characteristics and BRAF V600E status in Langerhans cell histiocytosis of Vietnamese children
Thu Dang Anh PHAN ; Bao Gia PHUNG ; Tu Thanh DUONG ; Vu Anh HOANG ; Dat Quoc NGO ; Nguyen Dinh The TRINH ; Tung Thanh TRAN
Journal of Pathology and Translational Medicine 2021;55(2):112-117
Background:
Langerhans cell histiocytosis (LCH) is more common in children than adults and involves many organs. In children, the BRAF V600E mutation is associated with recurrent and high-risk LCH.
Methods:
We collected paraffin blocks of 94 pediatric LCH patients to detect BRAF V600E mutation by sequencing. The relationship between BRAF V600E status and clinicopathological parameters were also critically analyzed.
Results:
BRAF V600E mutation exon 15 was detected in 45 cases (47.9%). Multiple systems LCH showed a significantly higher BRAF V600E mutation rate than a single system (p=.001). No statistical significance was evident for other clinical characteristics such as age, sex, location, risk organs involvement, and CD1a expression.
Conclusions
In Vietnamese LCH children, the proportion of BRAF V600E mutational status was relatively high and related to multiple systems.
2.Pathological brain lesions in girls with central precocious puberty at initial diagnosis in Southern Vietnam
Quynh Thi Vu HUYNH ; Ban Tran HO ; Nguyen Quoc Khanh LE ; Tung Huu TRINH ; Luu Ho Thanh LAM ; Ngan Thi Kim NGUYEN ; Shih-Yi HUANG
Annals of Pediatric Endocrinology & Metabolism 2022;27(2):105-112
Purpose:
Cranial magnetic resonance imaging (MRI) is recommended to identify intracranial lesions in girls with central precocious puberty (CPP). Yet, the use of routine MRI scans in girls with CPP is still debatable, as pathological findings in girls 6 years of age or older with CPP are limited. Therefore, we aimed to identify the prevalence of brain lessons in CPP patients stratified by age group (0–2, 2–6, and 6–8 years).
Methods:
This retrospective cross-sectional study recruited 257 girls diagnosed with CPP for 6 years (2010–2016). MRI was used to detect brain abnormalities. Levels of luteinizing hormone, follicle-stimulating hormone, and sex hormones in blood samples were measured.
Results:
Most girls had no brain lesions (82.9%, n=213), and of the minor proportion of girls with CPP that exhibited brain lesions (17.1%, n=44), 32 girls had organic CPP. Pathological findings were detected in 33.3% (2 of 6) of girls aged 0–2 years, 15.6% (5 of 32) of girls aged 2–6 years, and 3.6% (8 of 219) of girls aged 6–8 years. Hypothalamic hamartoma and tumors in the pituitary stalk were the most common pathological findings. The likelihood of brain lesions decreased with age. Girls with organic CPP were more likely to be younger (6.1±2.4 vs. 7.3±1.3 years, p<0.01) than girls with idiopathic CPP.
Conclusion
Older girls appeared to have a lower prevalence of organic CPP. Clinicians should cautiously use cranial MRI for girls aged 6–8 years with CPP.