Langerhans cell histiocytosis

Case contributed by Dr Ian Bickle

Presentation

Reluctance to mobilize. Plain films performed elsewhere.

Patient Data

Age: 2 years
Gender: Female

Plain radiographs were performed elsewhere, and not available to review.

Report indicated an expansile lucent lesion in the tibial metaphysis.

MRI

6.2cm well defined expansile mildly T1 hyperintense, T2 hyperintense, rim enhancing mass-like lesion in the left tibial metaphadiaphysis extending to the mid-diaphysis. A 4mm track contiguous with this extends into the physis and just into the epiphysis.

Extensive multifocal cortical destruction, the largest single focus measuring 1.6cm.

Edema in the posterior soft tissues, but no extra-ossesou involvement.

No synchronous lesion.

MRI performed to assess for additional lesions given histology of tibial bone biopsy

MRI

T12 vertebral plana.

High signal lesion in the posterior aspect of the right acetabulum.

No other abnormality.

Photo

Surgical biopsy was performed of the tibia and a definitive diagnosis of Histiocytosis X was given.

Case Discussion

Langerhans cell histiocytosis (LCH) is a rare multi-system disease with a wide and heterogeneous clinical spectrum and variable extent of involvement.  The musculoskeletal system is the commonest site of presentation.

The vertebral plana and addition pelvic lesion in this case are consistent with the histology from the tibial bone biopsy.

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