Kim Ju-Hwi, Jang Woo-Youl, Jung Tae-Young, Moon Kyung-Sub, Jung Shin, Lee Kyung-Hwa, Kim In-Young
Department of Neurosurgery, Chonnam National University Hwasun Hospital and Medical School, Hwasun, Jeollanam-do, South Korea.
Department of Pathology, Chonnam National University Hwasun Hospital and Medical School, Hwasun, Jeollanam-do, South Korea.
World Neurosurg. 2018 Aug;116:333-336. doi: 10.1016/j.wneu.2018.06.060. Epub 2018 Jun 19.
In this study, we report a case of solitary Langerhans cell histiocytosis (LCH) without skull or systemic lesions including reviewing the initial magnetic resonance (MR) findings of cerebral LCH.
A 36-year-old male patient presented with generalized tonic-clonic type seizure. Brain MR imaging showed a 3-cm mass lesion involving the right frontal lobe. It showed isointensity on T1-weighted images and hyperintensity on T2-weighted images. After gadolinium enhancement, the mass showed heterogeneous enhancement with perilesional edema and additional adjacent leptomeningeal enhancement. Special MR techniques focused on the enhanced lesion showed increased cerebral blood volume on perfusion images, no diffusion restriction on diffusion-weighted images, and a necrotic spectrum on MR spectroscopy. At surgery, we found a yellow mass and yellow tissue debris in the sulci adjacent to the mass and removed them. The final histopathologic diagnosis was LCH in the frontal lobe. The patient underwent scheduled adjuvant chemotherapy with cytarabine for 6 months and has been regularly followed up without any neurologic problem for 3 years.
In conclusion, additional sulcal enhancement around the mass lesion might be a clue MR imaging feature of intracerebral LCH, and special MR imaging techniques such as perfusion imaging, diffusion-weighted imaging, and MR spectroscopy could be helpful in differential diagnosis.
在本研究中，我们报告了一例无颅骨或全身病变的孤立性朗格汉斯细胞组织细胞增多症（LCH）病例，包括回顾脑LCH的初始磁共振（MR）表现。
一名36岁男性患者出现全身强直阵挛性癫痫发作。脑部MR成像显示一个3厘米的肿块病变，累及右侧额叶。在T1加权图像上呈等信号，在T2加权图像上呈高信号。钆增强后，肿块显示不均匀强化，伴有病灶周围水肿和额外的相邻软脑膜强化。针对强化病变的特殊MR技术显示灌注图像上脑血容量增加，扩散加权图像上无扩散受限，磁共振波谱显示坏死谱。手术中，我们在肿块相邻的脑沟中发现一个黄色肿块和黄色组织碎片并将其切除。最终组织病理学诊断为额叶LCH。患者接受了为期6个月的阿糖胞苷预定辅助化疗，并且已经定期随访3年，没有任何神经问题。
总之，肿块病变周围额外的脑沟强化可能是脑LCH的一个提示性MR成像特征，而灌注成像、扩散加权成像和磁共振波谱等特殊MR成像技术有助于鉴别诊断。
