Zhou Liangxue, Luo Linli, Hui Xuhui, Chen Haifeng, Yu Bin, Guo Gang, You Chao
Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, 610041, Sichuan, China.
Childs Nerv Syst. 2010 Dec;26(12):1813-7. doi: 10.1007/s00381-010-1270-3. Epub 2010 Aug 18.
Rathke's cleft cyst (RCC) is a congenital, benign, epithelial tumor and mainly occurs in sellar region and occasionally in suprasellar region; ectopic RCC is exceedingly rare. We report an uncommon RCC in cerebellopontine angle (CPA) associated with RCC apoplexy and investigated the possible hypothesis of its origin. A 12-year-old female student was admitted to hospital for 3-month history of vertigo, headache, nausea, and vomiting and aggravated for 1 week. Magnetic resonance imaging (MRI) revealed a space-occupying lesion with short T1 and long T2 signals in the left CPA and an intracystic floating nodule with hypointensity on T1- and T2-weighted imaging. The patient underwent the total tumor removal via the retrosigmoid approach with a good recovery. Primary RCC was confirmed by pathology. Follow-up MRI showed no recurrence 3.5 years after craniotomy. Primary RCC can occur in CPA and present special neuroimaging features associated with RCC apoplexy. We presumed that a mimicking mechanism of ectopic craniopharyngioma in CPA leads to the formation in the present case. Microsurgical resection is the optimal strategy for management. Further research and longer follow-up are helpful to better understanding the pathogenesis and development history of RCC in CPA.
拉克氏裂囊肿（RCC）是一种先天性良性上皮性肿瘤，主要发生于鞍区，偶尔见于鞍上区；异位RCC极为罕见。我们报告了一例发生于桥小脑角（CPA）的罕见RCC，伴有RCC卒中，并探讨了其可能的起源假说。一名12岁女学生因眩晕、头痛、恶心和呕吐3个月入院，症状加重1周。磁共振成像（MRI）显示左侧CPA有一个T1加权像呈短T1、T2加权像呈长T2信号的占位性病变，以及一个在T1加权像和T2加权像上呈低信号的囊内漂浮结节。患者通过乙状窦后入路接受了肿瘤全切术，恢复良好。病理证实为原发性RCC。术后3.5年的随访MRI显示无复发。原发性RCC可发生于CPA，并呈现出与RCC卒中相关的特殊神经影像学特征。我们推测，本例中CPA区异位颅咽管瘤的一种模拟机制导致了其形成。显微手术切除是最佳治疗策略。进一步的研究和更长时间的随访有助于更好地了解CPA区RCC的发病机制和发展历程。
