Langerhans cell histiocytosis

Case contributed by Ryan Thibodeau
Diagnosis certain

Presentation

Persistent right arm pain and paresthesia that did not resolve with conservative measures.

Patient Data

Age: 8 years
Gender: Female
x-ray

Best appreciated on the lateral view, there is flattening of the C6 vertebral body without angular kyphosis at this level.

There is loss of vertebral body disc height of C6, particularly at the anterior aspect of the anterior body. The remaining vertebral bodies are within normal limits.

mri

There is marked loss of height of the C6 vertebral body. There is a few millimeter retropulsion, with mild canal compromise.

Case Discussion

This is a case of Langerhans cell histiocytosis. Approximately 2 years prior to these images, the patient was found to have a lesion of the left posterior element of L3. This lesion underwent curettage. Pathology demonstrated areas of inflammation with scattered eosinophils and histiocytes.

The patient was found to have vertebra plana of C6. Due to the patient's symptoms, she underwent partial corpectomy at C6 and anterior fusion at C5-7. Pathology from biopsies of the vertebral body, right foramina, and resected vertebral disc revealed a cellular giant cell rich lesion with aggregates of Langerhans cells associated with reactive inflammation. The presence of aggregates of Langerhans cells was confirmed by immunohistochemical staining, which demonstrated these cells to be strongly positive for CD1a and for S-100 protein. These findings were compatible with Langerhans cell histiocytosis.

The patient finished 6 months of treatment with vinblastine, prednisone, and 6-mercaptopurine. Overall, the patient did well with the therapy and has not had recurrence of her symptoms.

Co-authors:
Alannah McCann
Travis Bevington, MD

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