Arko Leopold, Berry Corbett T, Desai Anuj Sunil, Weaver Michael
Department of Neurosurgery, Temple University, Philadelphia, Pennsylvania, United States.
College of Medicine, Drexel University, Philadelphia, Pennsylvania, United States.
J Neurol Surg A Cent Eur Neurosurg. 2017 Mar;78(2):167-179. doi: 10.1055/s-0036-1585584. Epub 2016 Aug 24.
Epidermoid cysts are a rare intracranial tumor, mostly arising from the cerebellar pontine angle but sporadically arising from the cranial diploe. Intradiploic epidermoids were first described in 1838 and have since been reported in sporadic case reports and case series. Due to the scarcity of cases, no institution has a large enough case series to fully characterize this tumor. We review numerous case series to better describe intradiploic epidermoids. Using a search for several case reports and series were found. References from these articles were reviewed for further cases. A total of 169 cases of intradiploic epidermoids were reviewed. The average age of patients presenting with intradiploic epidermoids was 38.1 years (standard error of the mean: 1.56). Overall, 68.9% of these patients presented with localized swelling over the scalp, 32.3% with headaches, and 42.7% with other neurologic symptoms. The most common location of the tumor was the frontal bones, with the least common the sphenoid, zygomatic, and maxillary bone. Surgical resection was curative in most cases, with a 3.2% mortality. Recurrence rate was only 5.8%, with nearly all occurring before 1999. Of the recurrent cases, malignant transformation to squamous cell carcinoma was estimated at 44% (4/9). Most of these cases are thought to have occurred as a result of incomplete resection of a primary intradiploic epidermoid. To date, this is the largest review of intradiploic epidermoids reported in the literature. In the reported cases, most had a benign course. However, there are occasional malignant transformations, and some patients have neurologic sequelae from mass effect or tumor infiltration. This is a surgically curative tumor in most cases.
表皮样囊肿是一种罕见的颅内肿瘤，大多起源于桥小脑角，但偶发于颅骨板障。板障内表皮样囊肿于1838年首次被描述，此后在散发病例报告和病例系列中均有报道。由于病例稀少，没有机构拥有足够大的病例系列来全面描述这种肿瘤。我们回顾了大量病例系列以更好地描述板障内表皮样囊肿。通过检索发现了几篇病例报告和系列。对这些文章的参考文献进行了回顾以寻找更多病例。共回顾了169例板障内表皮样囊肿病例。出现板障内表皮样囊肿的患者平均年龄为38.1岁（平均标准误差：1.56）。总体而言，这些患者中有68.9%表现为头皮局部肿胀，32.3%表现为头痛，42.7%表现为其他神经症状。肿瘤最常见的部位是额骨，最不常见的是蝶骨、颧骨和上颌骨。大多数情况下手术切除可治愈，死亡率为3.2%。复发率仅为5.8%，几乎所有复发都发生在1999年之前。在复发病例中，估计有44%（4/9）恶变至鳞状细胞癌。这些病例大多被认为是由于原发性板障内表皮样囊肿切除不完全所致。迄今为止，这是文献中报道的关于板障内表皮样囊肿的最大规模回顾。在报道的病例中，大多数病程良性。然而，偶尔会有恶变，一些患者因占位效应或肿瘤浸润出现神经后遗症。在大多数情况下，这是一种手术可治愈的肿瘤。
