Cranial unifocal Langerhans cell histiocytosis in children.
OBJECTIVE
The present study presents 22 cases of pediatric patients harboring an eosinophilic granuloma (EG) of the skull.


METHODS
Twenty-two patients (13 males, 9 females; mean age, 7.5 years; range, 3-14 years) with a suspected diagnosis of a cranial EG were enrolled in the study. They all had been preoperatively evaluated by skull x-ray and computed tomography, whereas 10 patients additionally underwent magnetic resonance imaging. To rule out a multifocal disease, scintigraphy was performed in all cases preoperatively. Surgical excision of the lesions was performed, and EG was proven histopathologically.


RESULTS
There was a male predominance. Frontal bone was the most common affected bone. One patient had a multifocal disease. Total excision of the lesion was performed in 19 of 22 patients. No patient received postoperative radiotherapy. In the remaining 3 cases because of the periorbital localization of the EG and the subsequent risk of disfigurement, only a biopsy was performed. These patients were treated with sulfamethoxazole and trimethoprim for 6 months. All 3 lesions were resolved. The follow-up examinations ranged from 6 months to 17 years, with a mean follow-up of 6.2 years, and no tumor recurrence was noted.


CONCLUSIONS
We conclude that EG is a benign disease. At the time of diagnosis, a bone scan should be performed to rule out a multifocal disease. Surgical resection is the treatment of choice. Nevertheless, for lesions in which excision can cause cosmetic defects, administration of sulfamethoxazole and trimethoprim after biopsy appeared to be an effective alternative treatment.
Alexiou
George A
GA
Department of Neurosurgery, Children's Hospital "Agia Sofia", Athens, Greece.
Mpairamidis
Evriviadis
E
Sfakianos
George
G
Prodromou
Neofytos
N
D000293:Adolescent; D000890:Anti-Infective Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D004803:Eosinophilic Granuloma / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D005260:Female; D005624:Frontal Bone; D006801:Humans; D008297:Male; D010294:Parietal Bone; D011379:Prognosis; D011877:Radionuclide Imaging; D012886:Skull / Q000000981:diagnostic imaging; D013420:Sulfamethoxazole / Q000627:therapeutic use; D013701:Temporal Bone; D014057:Tomography, X-Ray Computed; D014295:Trimethoprim / Q000627:therapeutic use
D000890:Anti-Infective Agents; D014295:Trimethoprim; D013420:Sulfamethoxazole