Algahtani Hussein, Shirah Bader, Bajunaid Mohammed, Subahi Ahmad, Al-Maghraby Hatim
King Abdulaziz Medical City / King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia.
King Abdullah International Medical Research Center / King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia.
Gulf J Oncolog. 2019 Sep;1(31):72-77.
Langerhans cell histiocytosis (LCH) is a rare neoplasm that is caused by an uncontrolled proliferation of Langerhans cells. The clinical presentation of LCH is heterogeneous and can manifest as single or multiple osteolytic lesions, skin ulcerations, and involvement of single or multiple systems. Central nervous system (CNS) involvement is reported in 3.4-57% of patients with multisystem disease. In this article, we present the case of a young man with single system involvement (bone) of LCH who presented with seizures, headache, papilledema, and tinnitus. His magnetic resonance imaging (MRI) of the brain findings were reported as a normal study. The subtle signs of CNS involvement were missed by the radiologist. However, the high index of suspicion resulted in early diagnosis and treatment. The presence of empty sella turcica in neuroimaging could be the first sign of intracranial disease with chronic intracranial hypertension associated with LCH. This is especially correct if previous computed tomography (CT) scan of the brain was normal with normal appearance of the pituitary gland and the sella. Neuroimaging films should be reviewed by an expert neuroradiologist. In patients with new neurological symptoms who were diagnosed previously with LCH, intracranial disease has to be excluded. The workup in such case should include an MRI of the brain, CT of the brain and temporal bones, bone scan, cerebrospinal fluid analysis, ophthalmological assessment, and measurement of intracranial pressure. In patients with LCH who present with symptoms and signs of raised intracranial pressure, the term idiopathic intracranial hypertension should not be applied until an intracranial disease has been excluded totally. Keywords: Langerhans Cell Histiocytosis; Central Nervous System Involvement; Neuroimaging; Intracranial Hypertension.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的肿瘤，由朗格汉斯细胞不受控制的增殖引起。LCH的临床表现具有异质性，可表现为单发性或多发性溶骨性病变、皮肤溃疡以及单系统或多系统受累。据报道，多系统疾病患者中3.4% - 57%会出现中枢神经系统（CNS）受累。在本文中，我们介绍了一名LCH单系统受累（骨骼）的年轻男性病例，他出现了癫痫发作、头痛、视乳头水肿和耳鸣。他的脑部磁共振成像（MRI）检查结果报告为正常。放射科医生遗漏了CNS受累的细微迹象。然而，高度的怀疑指数导致了早期诊断和治疗。神经影像学检查中出现空蝶鞍可能是与LCH相关的慢性颅内高压颅内疾病的首个迹象。如果之前的脑部计算机断层扫描（CT）显示垂体和蝶鞍外观正常且脑部正常，情况尤其如此。神经影像学胶片应由神经放射学专家进行复查。对于先前诊断为LCH且出现新的神经系统症状的患者，必须排除颅内疾病。此类病例的检查应包括脑部MRI、脑部和颞骨CT、骨扫描、脑脊液分析、眼科评估以及颅内压测量。对于出现颅内压升高症状和体征的LCH患者，在完全排除颅内疾病之前，不应使用特发性颅内高压这一术语。关键词：朗格汉斯细胞组织细胞增多症；中枢神经系统受累；神经影像学；颅内高压
