두경부를 침범한 랑게르한스 세포 조직구증의 치료

Abstract

Objective: The author presents a retrospective analysis of 11 patients with Langerhans cell histiocytosis (LCH) of head and neck to describe its optimal treatment strategies. Methods: This study involved five male and six female patients with a median age of 11 years. Sites of involvement included calvarium (9 cases), temporal bone (1 case), and cervical node (1 case). Ten patients had a solitary symptomatic lesion in the time of presentation. The localized LCH of calvarium (eosinophilic granuloma) was treated by surgery alone (8 cases) or surgery plus radiotherapy (1 case). An infant presented with multifocal disease received systemic chemotherapy. The patient who had extensive temporomastoid lesion with aural mass underwent radical mastoidectomy and cranial irradiation due to incomplete resection. Results: The time from initial symptom to diagnosis ranged from 10 days to four months (mean, 54 days). In cases with calvarial involvement, a painful lump with tenderness was the most common symptom at admission and a typical punched out lesion was seen on skull radiographs. No recurrence of symptoms or relapse of radiological evidence was observed during follow up, except for one infant who died of progressive multisystemic LCH. Conclusion: It is suggested that complete surgical excision is the treatment of choice for unifocal cranial LCH, leaving radiotherapy for possible relapse. Systemic chemotherapy can also plays an important role in the control of more extensive diseases in children.