Paediatric Pathology Online
Pathology of Pediatric Scalp and Skull Lesions
Langerhans' cell histiocytosis commonly involves the head and neck, especially the skull.
Presentation and extent of disease is variable.
Microscopically, they consist of Langerhans cells admixed with eosinophils and macrophages.
The Langerhans' cell has an indented nucleus and pink cytoplasm, is S100 and CD1a positive, and ultrastructurally contains Birbeck granules.
Although the diagnosis may be suspected by the characteristic histology and strengthened by S100 positivity, demonstration of CD1a positivity or Birbeck granules is required for definite diagnosis.
The differential diagnosis includes juvenile xanthogranuloma and Rosai-Dorfman disease, which may be extranodal.
These conditions usually lack CD1 surface antigen and Birbeck granules.
Cranial fasciitis presents as a rapidly enlarging mass in the scalp of infants and young children.
It erodes skull bone and may extend to the dura.
It is non-neoplastic and composed of stellate and spindle cells, vimentin and smooth-muscle actin positive, in myxoid stroma.
There is some histological overlap with nodular fasciitis.
Hamartoma of the scalp with ectopic meningothelial elements consists of connective tissue with cords of plump epitheloid cells that appear to line vascular channels.
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