Caldarelli M, Colosimo C, Di Rocco C
Department of Neurosurgery, Section of Pediatric Neurosurgery, Catholic University School of Medicine, Rome, Italy.
Surg Neurol. 2001 Aug;56(2):97-105. doi: 10.1016/s0090-3019(01)00542-0.
Epidermoid and dermoid cysts are rare intracranial space-occupying lesions, which account for about 1% of all intracranial tumors; they are even rarer in the pediatric age group. Among the various locations, that inside the brain stem is quite exceptional (only 12 cases reported to date in the literature). We report two additional cases occurring in children, underlining their clinical characteristics, the difficulties faced in the diagnostic work-up, and the surgical treatment adopted (which consisted of removal of the cyst contents and partial resection of the cyst membrane, because of its adherence to the surrounding nervous and vascular structures).
We report two cases of intraaxial dermoid/epidermoid cysts observed within the last 5 years. Both patients complained of cervico-nuchal pain, with a remittent/intermittent character in one of them; this patient also exhibited transient 6th and 7th cranial nerve deficit. One lesion was approached through the floor of the fourth ventricle, the second one through the ventrolateral aspect of the brainstem.
Subtotal tumor resection was achieved in both cases. Both patients had a smooth postoperative course without permanent neurological deficits. In the first patient residual tumor remains stable on follow-up MRI. In the second one, early tumor regrowth necessitated a second operation, after which the residual tumor has remained stable.
Based on our experience we suggest that a cautious surgical approach ("conservative" resection) to these lesions is the best choice. In fact, attempts at radical removal carry unacceptably high morbidity and mortality rates.
表皮样囊肿和皮样囊肿是罕见的颅内占位性病变，约占所有颅内肿瘤的1%；在儿童年龄组中更为罕见。在各个部位中，脑干内的此类囊肿相当特殊（迄今为止文献中仅报道了12例）。我们报告另外两例发生在儿童身上的病例，强调其临床特征、诊断检查中面临的困难以及所采用的手术治疗方法（由于囊肿壁与周围神经和血管结构粘连，手术包括切除囊肿内容物并部分切除囊肿壁）。
我们报告过去5年内观察到的两例轴内皮样/表皮样囊肿病例。两名患者均主诉颈枕部疼痛，其中一名患者疼痛呈缓解/间歇性；该患者还出现了短暂的第6和第7颅神经功能缺损。一个病变通过第四脑室底部入路，另一个通过脑干腹外侧入路。
两例均实现了肿瘤次全切除。两名患者术后病程顺利，无永久性神经功能缺损。在第一名患者中，随访MRI显示残留肿瘤稳定。在第二名患者中，肿瘤早期复发需要进行二次手术，术后残留肿瘤保持稳定。
根据我们的经验，我们建议对这些病变采取谨慎的手术方法（“保守”切除）是最佳选择。事实上，试图进行根治性切除会带来不可接受的高发病率和死亡率。
