Balasubramanian Kishore, Kharbat Abdurrahman, Haider Ammar, Parikh Deep, Cuellar Iii Jaime, Call-Orellana Francisco, Palmisciano Paolo, Bin-Alamer Othman, Rajendran Sibi, Graffeo Christopher S, Zuccato Jeffrey A, Dunn Ian F
Department of Neurosurgery, University of Oklahoma College of Medicine, Oklahoma City, OK, USA.
College of Medicine, Texas A&M Health Science Center, Houston, TX, USA.
Neurosurg Rev. 2025 Sep 12;48(1):639. doi: 10.1007/s10143-025-03798-2.
Intradiploic epidermoid cysts (IECs) are rare cystic calvarial lesions. Due to their rarity, there is a clinical need to aggregate the collective experience reported in the literature to characterize typical clinical features, clinical predictors of patient outcomes, and management strategies used for this entity. Following PRI SMA guidelines, 127 patients from 94 studies (77 case reports, 17 case series) were included with individual patient data. Clinical data on demographics, clinical presentation, imaging features, management approaches, and outcomes were characterized, and predictors of outcome were evaluated. The cohort had a median age of 44 years (range = 18-81). Common presenting clinical features included headache (50%) and a palpable mass (48%), with half reporting antecedent head trauma. Lesions most frequently involved the occipital (28%), frontal (26%), and parietal bones (15%), with 13% localizing to multiple bones of the calvarium. Surgical resection was performed in all but one case, achieving a gross total resection (GTR) in 92% and complete cyst capsule removal in 87%. The rate of recurrence was significantly lower in those who received a GTR versus a subtotal resection (4% vs. 57%, p < 0.001) and in cases where the capsule was completely versus incompletely removed (2% vs. 66%, p < 0.001). IECs should be suspected in patients presenting with headaches or palpable skull masses with osteolytic non-enhancing and diffusion-restricting T2 hyperintense calvarial lesions. Complete surgical excision with total cyst capsule removal is the goal for IEC management. Close long-term monitoring remains essential, particularly in lesions that have been subtotally resected.
板障内表皮样囊肿（IECs）是一种罕见的颅骨囊性病变。由于其罕见性，临床上需要汇总文献中报道的集体经验，以明确其典型临床特征、患者预后的临床预测因素以及针对该病变所采用的管理策略。按照PRISMA指南，纳入了来自94项研究（77篇病例报告、17篇病例系列）的127例患者的个体患者数据。对人口统计学、临床表现、影像学特征、管理方法和预后的临床数据进行了特征描述，并对预后预测因素进行了评估。该队列的中位年龄为44岁（范围=18 - 81岁）。常见的临床表现包括头痛（50%）和可触及肿块（48%），其中一半患者报告有头部外伤史。病变最常累及枕骨（28%）、额骨（26%）和顶骨（15%），13%定位于颅骨的多块骨头。除1例患者外，所有患者均接受了手术切除，92%实现了全切除（GTR），87%实现了囊肿包膜完全切除。接受GTR的患者与次全切除的患者相比，复发率显著更低（4%对57%，p<0.001），囊肿包膜完全切除与未完全切除的病例相比也是如此（2%对66%，p<0.001）。对于出现头痛或可触及颅骨肿块且伴有颅骨溶骨性、无强化及扩散受限的T2高信号病变的患者，应怀疑为IECs。完整手术切除并完全去除囊肿包膜是IECs管理的目标。长期密切监测仍然至关重要，尤其是对于次全切除的病变。
