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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.
Review the high-quality, interactive digital Aperio slides at http://virtualacp.com/JCPCases/jclinpath-2018-205016/ and consider your diagnosis
What is your diagnosis?
Erdheim-Chester disease (ECD)
Follicular dendritic cell sarcoma (FDCS)
Histiocytic sarcoma (HS)
Langerhans cell histiocytosis (LCH)
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