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A man in his mid-20s presented with a 2-month history of an enlarging left-sided skull swelling associated with focal pain. His medical history and systems enquiry were otherwise unremarkable. Specifically, he had no neurological deficits and respiratory, skin or constitutional symptoms. MRI brain scan revealed a lytic focus in the left parietal bone. Within this lytic lesion was an enhancing soft-tissue mass with central necrosis. The mass extended over the inner and outer surface of the skull vault and had both an extradural and a deep scalp component. There was no cerebral oedema, hydrocephalus or midline shift. The patient underwent a left parietal craniotomy and excision of the lytic parietal skull and extradural lesion. The histopathology of the skull lesion is shown in the scanned slide.
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Erdheim-Chester disease (ECD)
Follicular dendritic cell sarcoma (FDCS)
Histiocytic sarcoma (HS)
Langerhans cell histiocytosis (LCH)
Handling editor Iskander Chaudhry.
Contributors SDM conceived of the project and critically reviewed the manuscript. VC-LC wrote the manuscript. CLT provided the slide and provided input on the histopathology component. Y-LC critically reviewed the manuscript and edited the clinical component.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Commissioned; internally peer reviewed.