Langerhans Cell Histiocytosis in the Pediatric Population: Treatment of Isolated Craniofacial Lesions

Langerhans cell histiocytosis (LCH) commonly affects the craniofacial skeleton and prognosis depends on location, extension, and recurrence of the disease. The aim of our study is to better define the treatment of single craniofacial lesions, as to date different treatment modalities have been suggested and recurrence rates for both unifocal and multifocal bony lesion range between 10% and 70%. Between 2000 and 2014, we retrospectively reviewed clinical findings, anatomic location, extent of the disease, therapy, and outcomes in 24 pediatric patients with histologically confirmed LCH. Seventeen patients (67%) had craniofacial involvement, of which 13 had single system involvement and 4 had multisystem involvement. Eight patients (33%) had no craniofacial involvement. Eleven patients affected by unifocal cranial lesions were treated with resection and reconstruction. One patient with a unifocal mastoid lesion was treated with chemotherapy alone (vinblastine and prednisone). Four patients with mandible lesions were treated with curettage alone. There were no recurrences in patients treated with excision alone. One patient (25%) treated with curettage recurred. Two patients with diffuse disease manifested organ dysfunction and diabetes insipidus. Chemotherapy was tolerated in 12 patients treated. Our findings suggest that resection of isolated LCH lesions of the cranium is safe and chemotherapy is effective and well tolerated for nonsurgical cases.