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Eosinophilic granuloma - skull

Case contributed by Frank Gaillard
Diagnosis certain

Patient Data

Age: 15 years
Gender: Female

Skull x-ray

x-ray

A well defined lytic lesion is seen in the parietal bone on the right with bevelled edges. It involves both the inner and outer table, although the outer table is affected more than the inner table. 

Brain

mri

Pre-and post contrast scans were performed. The skull defect measures 18 mm in transverse dimension and the lesion measures 14.5 mm in height. There is destruction of both inner and outer tables.  The calvarium adjacent to the lesion is not expanded. The lesion has an extensive subgaleal component and intracranially abuts the dura which is thickened and demonstrates increased enhancement. The lesion has stippled central low T2 signal with peripheral high T2 signal. There is mild increased T1 signal and peripheral enhancement with irregular central non enhancement which may represent central necrosis. The lesion demonstrates probable peripheral restricted diffusion/low ADC values (not shown). 

No other calvarial lesions are seen. No definite intra-axial abnormality. The infundibulum and cavernous sinuses have a normal appearance (not shown).

The patient went on to have a local resection. 

MICROSCOPIC DESCRIPTION:

Paraffin sections show densely hypercellular tissue fragments.These show a mixture of large mononuclear cells, multinucleated cells and eosinophils. The mononuclear cells have reniform vesicular nuclei and a moderate amount of pale cytoplasm. Similar nuclear features are noted in the multinucleated cells. Eosinophils are dispersed as single cells as well as diffuse sheets intermingling with the mononuclear and multinucleated cells. No mitotic figures are identified and there is no necrosis. No bone tissue is included in the specimens submitted. The features are of Langerhans cell histiocytosis (eosinophilic granuloma).

Distribution of LCH in bone

Diagram

Distribution of Langerhans cell histiocytosis in bone. Layout and distribution: Frank Gaillard 2012, Line drawing of skeleton: Patrick Lynch 2006, Creative Common NC-SA-BY

Case Discussion

This case illustrates the typical appearances of an eosinophilic granuloma of the skull. 

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