Langerhans cell histiocytosis

Case contributed by Ian Bickle
Diagnosis certain

Presentation

Toddler who was generally unwell.

Patient Data

Age: 3 years
Gender: Male
x-ray

Lucent lesions in the skull and right ilium.  L3 vertebra 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 hemorrhagic tissue 1.5 x 1 x 0.4 cm 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 hemorrhage are also present.

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

DIAGNOSIS
LEFT PARIETAL BONE OF SKULL, CURETTAGE: LANGERHANS 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 termed eosinophilic granuloma.

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

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