Primary head and neck Langerhans cell histiocytosis in children.

OBJECTIVE

To evaluate the experience of a tertiary care children's hospital in the diagnosis, treatment, and long-term outcomes of patients with primary head and neck Langerhans' cell histiocytosis.

STUDY DESIGN AND SETTING

A retrospective study of patients who presented with Langerhans' cell histiocytosis that primarily affected head and neck sites between January 1, 1986, and December 31, 2004, at Children's Mercy Hospital.

RESULTS

Twenty-two patients were diagnosed and treated for LCH at our hospital during this time period. Seventeen (77%) patients had head and neck involvement; 14 (64%) of these patients had primary head and neck LCH. Lesions of the head and neck are complicated and involve multiple structures including the calvarium and skull base. Overall outcomes were good with 10 of 14 patients without disease at time of last follow-up. Recurrence is common and involved 50% of our patients.

CONCLUSIONS

Langerhans' cell histiocytosis commonly affects the head and neck. These lesions are complex and require prudent evaluation and treatment. Due to the complex nature of head and neck lesions, primary chemotherapy is the treatment of choice.

SIGNIFICANCE

Because of the frequent head and neck involvement of this disease, otolaryngologists need to be familiar with its presentation, work-up, and treatment.

EBM RATING

C-4.