Tosaka Masahiko, Sato Koji, Amanuma Makoto, Higuchi Tetsuya, Arai Motohiro, Aishima Kaoru, Shimizu Tatsuya, Horiguchi Keishi, Sugawara Kenichi, Yoshimoto Yuhei
Department of Neurosurgery, Gunma University Graduate School of Medicine.
Neurol Med Chir (Tokyo). 2015;55(1):89-94. doi: 10.2176/nmc.cr.2012-0362. Epub 2014 Mar 27.
Superficial siderosis is a rare condition caused by hemosiderin deposits in the central nervous system (CNS) due to prolonged or recurrent low-grade bleeding into the cerebrospinal fluid (CSF). CNS tumor could be one of the sources of bleeding, both pre- and postoperatively. We report an extremely rare case of superficial siderosis associated with purely third ventricle craniopharyngioma, and review previously reported cases of superficial siderosis associated with CNS tumor. A 69-year-old man presented with headache, unsteady gait, blurred vision, and progressive hearing loss. Brain magnetic resonance (MR) imaging with gadolinium revealed a well enhanced, intraventricular mass in the anterior part of the third ventricle. T2*-weighted gradient echo (GE) MR imaging revealed a hypointense rim around the brain particularly marked within the depth of the sulci. Superficial siderosis was diagnosed based on these findings. The tumor was diffusely hypointense on T2*-weighted GE imaging, indicating intratumoral hemorrhage. The lateral ventricles were dilated, suggesting hydrocephalus. [(18)F]fluorodeoxyglucose positron emission tomography revealed increased uptake in the tumor. The whole brain surface appeared dark ocher at surgery. Histological examination showed the hemorrhagic tumor was papillary craniopharyngioma. His hearing loss progressed after removal of the tumor. T2*-weighted GE MR imaging demonstrated not only superficial siderosis but also diffuse intratumoral hemorrhage in the tumor. Superficial siderosis and its related symptoms, including hearing loss, should be considered in patients with hemorrhagic tumor related to the CSF space. Purely third ventricle craniopharyngioma rarely has hemorrhagic character, which could cause superficial siderosis and progressive hearing loss.
浅表性铁沉积症是一种罕见的疾病，由于脑脊液（CSF）中长期或反复的低度出血导致含铁血黄素在中枢神经系统（CNS）沉积所致。中枢神经系统肿瘤可能是术前和术后出血的来源之一。我们报告一例极其罕见的与单纯第三脑室颅咽管瘤相关的浅表性铁沉积症病例，并回顾先前报道的与中枢神经系统肿瘤相关的浅表性铁沉积症病例。一名69岁男性出现头痛、步态不稳、视力模糊和进行性听力丧失。钆增强脑磁共振（MR）成像显示第三脑室前部有一个强化良好的脑室内肿块。T2 *加权梯度回波（GE）MR成像显示脑周围有低信号环，在脑沟深处尤为明显。根据这些发现诊断为浅表性铁沉积症。在T2 *加权GE成像上肿瘤呈弥漫性低信号，提示肿瘤内出血。侧脑室扩张，提示脑积水。[（18）F]氟脱氧葡萄糖正电子发射断层扫描显示肿瘤摄取增加。手术时整个脑表面呈暗赭色。组织学检查显示出血性肿瘤为乳头状颅咽管瘤。肿瘤切除后他的听力丧失进展。T2 *加权GE MR成像不仅显示浅表性铁沉积症，还显示肿瘤内弥漫性出血。与脑脊液间隙相关的出血性肿瘤患者应考虑浅表性铁沉积症及其相关症状，包括听力丧失。单纯第三脑室颅咽管瘤很少具有出血特征，这可能导致浅表性铁沉积症和进行性听力丧失。
