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ENT · Ear and audiology: Langerhans cell histiocytosis of the temporal bone
Sample itemA 3-year-old has chronic ear discharge unresponsive to antibiotics and a postauricular swelling. CT shows a punched-out lytic lesion of the mastoid, and a skull radiograph shows other lytic areas. What is the most likely diagnosis?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. Langerhans cell histiocytosis
Langerhans cell histiocytosis in a young child can involve the temporal bone, presenting as a refractory aural polyp or discharge and a postauricular swelling, with punched-out lytic skull lesions. Biopsy confirms it by CD1a positivity and Birbeck granules.
Option by option
A. Cholesteatoma
Cholesteatoma gives scalloped bone erosion with a retraction pocket, not multiple lytic skull lesions.
B. Rhabdomyosarcoma
Rhabdomyosarcoma causes a bleeding polyp with rapid facial palsy and a soft tissue mass, not multiple skull lesions.
C. Tuberculous mastoiditis
Tuberculous mastoiditis gives painless discharge, sequestra and multiple drum perforations.
D. Langerhans cell histiocytosis (correct)
Correct. Refractory otorrhoea with a punched-out mastoid lesion and other lytic skull defects suggests Langerhans cell histiocytosis.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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