Langerhans cell histiocytosis

Case contributed by Dr Anar Kazimov

Presentation

Tender swelling of the right temporal soft-tissues.

Patient Data

Age: 7 years
Gender: Female
MRI

Soft tissue mass in the right temporalis muscle extending through the infratemporal fossa and infiltrating the squamous part and zygomatic process of the temporal bone. The mass has central cystic components and after intravenous gadolinium administration there is marked peripheral enhancement of the lesion. 

An enhancing extensive soft tissue mass is noted in the right temporal muscle with marked osteolysis of the right squamous temporal bone.

Case Discussion

Langerhans cell histiocytosis (LCH) arises from an abnormal proliferation of histiocytes, and most commonly presents as a solitary lesion in the skull. In this case, the mass demonstrates central cystic changes with enhancing soft tissue components and is associated with temporal bone destruction. The open biopsy confirmed LCH.

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