Langerhans cell histiocytosis presenting as an isolated brain tumour: A case report

Langerhans cell histiocytosis (LCH) is a rare proliferative histiocyte disorder. It can affect any organ or system, especially the bone, skin, lung, and central nervous system (CNS). In the CNS, the hypothalamic-pituitary is predominantly affected, whereas the brain parenchyma is rarely affected. LCH occurring in the brain parenchyma can be easily confused with glioblastoma or brain metastases. Thus, multimodal imaging is useful for the differential diagnosis of these intracerebral lesions and detection of lesions in the other organs.

A 47-year-old man presented with a headache for one week and sudden syncope. Brain computed tomography (CT) and magnetic resonance imaging showed an irregularly shaped nodule with heterogeneous enhancement. On ¹⁸F-fluorodeoxyglucose (¹⁸F-FDG) positron emission tomography/CT, a nodule with ¹⁸F-FDG uptake and multiple cysts in the upper lobes of both lungs were noted, which was also confirmed by high-resolution CT. Thus, the patient underwent surgical resection of the brain lesion for further examination. Postoperative pathology confirmed LCH. The patient received chemotherapy after surgery. No recurrence was observed in the brain at the 12-mo follow-up.

Multimodal imaging is useful for evaluating the systemic condition of LCH, developing treatment plans, and designing post-treatment strategies.