title: Solitary Cranial Langerhans Cell Histiocytosis: Two case reports.
abstract: Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as a unisystem (unifocal or multifocal) or multisystem disease. We describe the clinical and histologic spectrum of LCH of the orbit and skull in our two cases. Both cases had unifocal erosive skull lesions with a history of trauma. Typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunohistochemistry. LCH has an excellent prognosis when treated with surgical resection, steroids and radiotherapy or chemotherapy. One of our patients is disease free at 7 year follow-up and one patient had regression of lesion on follow-up.
authors.forename: Prasanna
authors.initials: P
authors.lastname: Karki
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Hirano
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Yamahata
authors.forename: Shingo
authors.initials: S
authors.lastname: Fujio
authors.forename: Hajime
authors.initials: H
authors.lastname: Yonezawa
authors.forename: Koji
authors.initials: K
authors.lastname: Iida
authors.forename: Manoj
authors.initials: M
authors.lastname: Bohara
authors.forename: Tatsuki
authors.initials: T
authors.lastname: Oyoship
authors.forename: Ryosuke
authors.initials: R
authors.lastname: Hanaya
authors.forename: Kazunori
authors.initials: K
authors.lastname: Arita
mesh_terms: D018949:Antigens, CD1 / Q000032:analysis; D015415:Biomarkers / Q000032:analysis; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D006259:Craniocerebral Trauma / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D006646:Histiocytosis, Langerhans-Cell / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D007801:Langerhans Cells / Q000276:immunology / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D009916:Orbital Diseases / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D012074:Remission Induction; D009418:S100 Proteins / Q000032:analysis; D012886:Skull / Q000276:immunology / Q000473:pathology*; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D018949:Antigens, CD1; D015415:Biomarkers; C419393:CD1a antigen; D009418:S100 Proteins