Oral Langerhans cell histiocytosis

AIM: Our aim was to compare the different methods of treatment available for Langerhans cell histiocytosis (LCH) in the oral maxillofacial region. A classification based on clinical and immunohistochemical data is proposed and a grading for disease severity is provided.

PATIENTS AND METHODS: The records and clinical data of 12 patients were evaluated retrospectively. The patients ages ranged from 20 months to 47 years. Nine had manifestations in the maxillo-facial region, of which six had a single oral manifestation only, with the three remaining ones having multiple manifestations in this region. Three patients had additional manifestations in the upper thorax (coming to a total of twelve patients). Eleven patients were surgically treated with one having been treated previously with a steroid-therapy. One patient had been treated with chemotherapy. The longest duration of follow-up was 16 years. Immunohistochemical stains for antigen-CD1a, antigen-S-100 and antigen-Ki-67 were performed. A proposal for staging the manifestations in the oromaxillo-facial-region was made. The intensity of the antigen-Ki-67 stains were evaluated.

RESULTS: One patient presented a relapse only implying that surgical treatment was very effective in the eradication and cure of the disorder. Eleven patients exhibited no signs of recurrence. It is felt that the number of antigen-Ki-67-positive cells is related to the activity of the disease.

CONCLUSION: Langerhans cell histiocytosis should be treated surgically. Only in very severe cases should surgical treatment be complimented by either radiotherapy or chemotherapy. Especially in disseminated cases, chemotherapy seems to improve the outcome. Antigen-Ki-67 as a proliferation marker is suggested as a grading parameter for local tumor behaviour.