Clinicopathological features of extraosseous Langerhans cell histiocytosis:report of five cases and review of literature
ZHANG Yun
ZHANG Guan-jun
WAGN Kai
LIAN Jie
DENG Yuan
Abstract：Objective To explore the cinicopathological features of extraosseous Langerhans cell histiocytosis(LCH).Methods From August 2015 to May 2023,5 cases of extraossous LCH diagnosed in the Department of Pathology,the First Affiliated Hospital of Xi'an Jiaotong University were collected.The clinicopathological features,immunophenotype,molecular detection,diagnosis and differential diagnosis of BRAF V600E were studied and followed up.Results Among the 5 cases,1 case was male and 4 cases were female.The age of birth was 6 hours to 68 years.The mean age was 30 years.Among them,1 case involved the left palate and thyroid gland,1 case involved the thymus gland,the other cases involved the single system,2 cases involved the skin,1 case involved the thyroid gland.Histologically,the coffee-like Langerhans cells were arranged in sheets and nests,with abundant cytoplasm,light staining and little nuclear atypia.The immunophenotypic tumor cells expressed CD1a,Langerin,S-100,and BRAF V600E protein focally.One patient had a BRAF V600E mutation,and the remaining four patients had no detectable BRAF V600E mutation.Two patients received chemotherapy after diagnosis,and two patients underwent surgical resection only.Treatment for the remaining 1 case was unknown.Four patients were followed up(one was lost to follow-up)with stable condition and no progress.Conclusion Histological morphology and immunophenotype are the gold criteria for the diagnosis of extra-osseous LCH,and BRAF V600E gene mutation can be used as auxiliary diagnostic criteria for LCH.
Keywords：Langerhans histiocytosisSkinThyroidThymus
Publication Date：2025-04-28
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：6( 384-389 )