Langerhans cell histiocytosis of the parietal bone with epidural and extracranial expansion – case report and a review of the literature

Abstract

Langerhans cell histiocytosis is a rare neoplasm that belongs to the histiocytic and dendritic cell neoplasm group according to the 2008 WHO classification. It has been defined as neoplastic proliferation of Langerhans cells that express CD1a and S-100 proteins and have Birbeck granules on the ultrastructural examination. Clinical presentation and behaviour are heterogeneous and can range from a solitary lytic bone lesion with a favourable course to a fatal disseminated leukaemia-like form, with a wide spectrum of intermediate clinical presentations between these two extremes. Here, we present a case report of a solitary calvarial lesion in an adolescent boy along with a review of the literature. Presenting features, initial diagnostic evaluation and treatment protocol of a unifocal monosystemic calvarial location of LCH are presented.