Dr.Soubhagya S B Patil

Prof. Dr.Chinmayee J Thrishulamurthy, Dr.Bv Shwetha

Abstract

STUDY DESIGN: A case report
PURPOSE: langerhans cell histiocytosis ( LCH) is a rare disease seen more commonly in childhood
METHODS: Here we report a case of LCH in a 13 months old female child who presented to us with a left upper eyelid swelling and underwent an incisional biopsy, immunohistochemistry evaluation and was referred to paediatric oncologist for systemic evaluation
RESULTS: The histopathological examination revealed Langerhans histiocytes. The immunohistochemistry of the lesion was positive for S100, CD1a, CD68 (focal) which confirmed the diagnosis. PET CT suggested FDG enhancing soft tissue density mass lesion involving left temporal lobe extending to left orbit, infratemporal fossa causing destruction of left lateral orbital wall and mass effect on globe and an FDG uptake in biliary canaliculi suggesting systemic involvement of LCH. The child was treated with vinblastine and steroids
CONCLUSION: LCH is a disorder with aggressive course and good response to treatment