Cranial unifocal langerhans cell histiocytosis in a female child: a difficult case with S-100 and CD1a immunonegativity

Abstract

A 13-years old female child was carried to Dr. Sardjito General Hospital, Yogyakarta by her mother with chief
complaint of a mass on her forehead. Since eight months prior to her visiting, she had a mass on her forehead which
became larger slowly without tenderness and had no fever. Clinical examination revealed a well circumscribed
mass, 3 cm in diameter, fixed, with rubbery consistency. The skull X-ray revealed a punch out lesion in frontal bone.
The head CT scanning revealed a destruction of frontal bone. Clinical diagnosis of dermoid cyst was determined,
excision and curettage was performed. Gross examination showed 2.5 cc fragmented tissue, brownish yellow, with
rubbery consistency. A diagnosis of benign histiocytosis (Langerhans cell histiocytosis or non-Langerhans cell
histiocytosis) of frontal bone was determined based on morphological and immunohistochemical examination. The
aim of this presented article was to report a rare case of cranial unifocal Langerhans cell histiocytosis in a female
child with S-100 and CD1a immunonegativity, and to discuss how to determine its diagnosis based on literature
review.
Key words: Langerhans cell histiocytosis - juvenile xanthogranuloma – reticulohistiocytoma - eosinophilic granuloma
– S100 – CD1a