Langerhans cell histiocytosis

Case contributed by Dr Ian Bickle

Presentation

Toddler who was generally unwell.

Patient Data

Age: 3
Gender: Male
X-ray
Lucent lesions in the skull and right ilium.  L3 vertebral plana.

SKULL LESION BIOPSY

GROSS

The specimen is received in formalin, labeled with patient data and designated "lt parietal skull lesion". It consists of a few pieces of haemorrhagic tissue 1.5x1x0.4cm in aggregate. (

MICROSOPY

The specimen comprises sheets of histiocytic cells admixed with eosinophils, neutrophils, plasma cells and multinucleated giant cells. The histiocytic cells frequently have indented nuclei, single nucleoli and abundant pale cytoplasm. Necrosis and haemorrhage are also present.

Immunohistochemistry shows the histiocytic cells positively stains with CD1a and S100.

DIAGNOSIS
LEFT PARIETAL BONE OF SKULL, CURETTAGE: LANGERHAN CELL HISTIOCYTOSIS.

Case Discussion

Multiple bone lesions in keeping with Langerhans cell histiocytosis.  The skull lesions show the classical bevelled edges. A single skull lesion would be term eosinophilic graunuloma.

Vertebra plana is another feature, although a differential for this in isolation exists.

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Case information

rID: 23907
Case created: 17th Jul 2013
Last edited: 26th Feb 2016
Inclusion in quiz mode: Included

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