Langerhans cell histiocytosis

Case contributed by RMH Neuropathology


Forehead lump.

Patient Data

Age: 25 years
Gender: Male

On the lateral x-ray image a focal cortical osteolytic lesion is seen. No sclerotic rim.  

CT images show a midline hyperdense soft tissue mass focally eroding both the internal and the external frontal bone layers. The lesion has both an intracranial component which remains extra-dural and a extracranial subgaleal component. There is destruction of both inner and outer frontal bone tables.   


MRI shows again the destruction of both inner and outer frontal bone tables in the midline region, where a soft tissue mass extends with a subgaleal component and intracranially abutting the dura. The lesion has a intermediate T1 signal with a moderate to marked central contrast-enhancement, there are also peripheral small cystic components with a fluid-fluid level inside. 



MICROSCOPIC DESCRIPTION: Paraffin sections show a densely hypercellular lesion. This is composed predominantly of large cells with irregular round, oval and indented nuclei some with conspicuous nucleoli and variable amount of pale cytoplasm. These are arranged in diffuse sheets and are admixed with large numbers of eosinophils. Many multinucleated giant cells are noted. An occasional mitotic figure is identified. The tumor cells are S-100 and CD1a positive. They are AE1/3 negative. The features are of Langerhans cell histiocytosis (eosinophilic granuloma). DIAGNOSIS: Frontal scalp subgaleal lesion: Langerhans cell histiocytosis (eosinophilic granuloma).

Case Discussion

This case illustrates an eosinophilic granuloma of the skull. Langerhans cell histiocytosis is a rare multi-system disease, with unknown etiology and a wide and heterogeneous clinical spectrum and variable extent of involvement. 

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