Langerhans cell histiocytosis of fibula

Case contributed by Dr Brian Gilcrease-Garcia

Presentation

Painful swelling of lower extremity, walking with limp.

Patient Data

Age: 2 years
Gender: Female
X-ray

Tibia-fibula radiograph

Lytic lesion with somewhat permeative appearance centered in the medullary canal of the midshaft fibula. The lesions features ill defined margins (wide zone of transition). Periostitis surrounding the bone appears in a lamellated pattern, which nearly give the fibular diaphysis an expansile appearance. Extensive soft tissue swelling associated with this lesion.

Histopathology

Microscopic description

The provided slides show aggregates of histiocytes mixed with abundant eosinophils and lymphocytes. Nuclear grooves are also seen in some of the histiocytes. The provided immunostains show that these clusters of histiocytes are positive for CD1a. The morphology and immunoprofile support the diagnosis of Langerhans cell histiocytosis.

Diagnosis

Langerhans cell histiocytosis

Case Discussion

Despite its somewhat atypical location in the fibula, the radiographic appearance and demographic are classic for Langerhans cell histiocytosis.

There are multiple features which connote that this is an aggressive lesion:

  • wide zone of transition
  • lamellated pattern of periostitis - suggests that physiologic process of bone healing cannot "keep up" with the tissue destruction encited by the lesion
  • associated soft tissue swelling

In the absence of signs or symptoms of infection to suggest osteomyelitis, the differential for an aggressive-appearing lesion in a child includes metastasis (e.g. neuroblastoma), blue round cell tumor, and primary osseous lymphoma.

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Case information

rID: 60177
Published: 22nd Jun 2018
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included
Institution: Washington University in Saint Louis

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