Multifocal Intraparenchymal Langerhans’ Cell Histiocytosis Concomitant With an Arachnoid Cyst in a Child

Abstract

Langerhans' cell histiocytosis is a disease usually found in children and characterized by idiopathic proliferation of histiocytes in the reticuloendothelial system. Intracranial Langerhans' cell histiocytosis presenting as multifocal intraparenchymal lesions is very rare. In this article, the authors report on a 4-year-old boy diagnosed with multifocal intraparenchymal Langerhans' cell histiocytosis concomitant with an arachnoid cyst. After a series of laboratory examinations, the right frontal mass was surgically excised. Histological examinations confirmed the diagnosis of intracranial Langerhans' cell histiocytosis. The patient's intracranial hypertension symptoms were alleviated, and the remaining foci were treated by Langerhans' cell histiocytosis-directed standard chemotherapy. At the 8-month follow-up visit, no recurrence of the excised lesion was found, and no change in the size of other lesions was seen. 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.

Prevalence and natural history of arachnoid cysts in adults

“…Case reports of such associations do not prove causation and, by suggesting that such a relationship is possible or even likely, these reports are misleading. Recent examples include cases of arachnoid cysts found in patients with intracranial aneurysms, 82 Langerhans cell histiocytosis, 92 parkinsonism, 89 panic disorder, 17 personality disorders, 9 conversion disorder, 19 depression, 11 psychosis, 44,53 and coital headache. 46 Individual case reports have also attempted to suggest a connection between arachnoid cysts and various syndromes such as Kabuki syndrome, 13,47 Chudley-McCullough syndrome, 96 oculoectodermal syndrome, 59 Apert syndrome, 103 Sturge-Weber syndrome, 26 Usher syndrome, 18 Aicardi syndrome, 16 Proteus syndrome, 5 neurofibromatosis, 61 Marfan syndrome, 95 and undefined syndromes including undescended testes 83 and absence of the tibia.…”