Multifocal intraparenchymal Langerhans' cell histiocytosis in a child: case report and review of the literature

Abstract

Objective To improve the understanding of intracranial Langerhans' cell histiocytosis (LCH) in children.Method A 4 - year - old boy diagnosed with multifocal intraparenchymal LCH and the consequent procedure of treatments were reported,as well as some comparable literatures were reviewed.Results The patient complained firstly of nausea and vomiting with a progressing headache.Multifocal intracranial lesions with mass effect concomitant with an arachnoid cyst were found in imaging examination.After a series of laboratory examinations,the right frontal mass ( which caused symptoms of intracranial hypertension) was surgically excised.Histological examinations confirmed the diagnosis of intracranial Langerhans' cell histiocytosis (ILCH).The patient' s intracranial hypertension symptoms were alleviated and the remaining foci were treated by LCH - directed standard chemotherapy.At the 6 - month follow - up visit,no recurrence of the excised lesion was found,and no change in the size of other lesions was seen.Conclusion Supratentorial intracerebral lesions with mass effect and enhancement have rarely been described; in this report,the histological features of and therapeutic options for such a case are discussed.Surgery for the intracranial lesions with mass effect followed with LCH - directed standard chemotherapy was recommended. Key words: Langerhans'cell histiocytosis; Intracranial multifocal lesions; Child