Donovan Daniel Joseph, Thavapalan Varoon
Department of Surgery, University of Hawaii-John A. Burns School of Medicine, Honolulu, Hawaii.
Department of Neurological Surgery, Thomas Jefferson University Hospitals, Philadelphia, Pennsylvania.
Surg J (N Y). 2016 May 26;2(2):e31-e36. doi: 10.1055/s-0036-1584166. eCollection 2016 Apr.
Pediatric meningeal tumors are rare, but those in the region of the sylvian fissure without dural attachment are extremely rare, with only 24 previously reported cases in the world literature. In this series, we report two additional cases of sylvian fissure meningioma without dural attachment and one case of perisylvian meningioangiomatosis in the medial temporal lobe. All three patients presented with complex partial seizures, but the diagnosis was delayed in each case because the symptoms were misinterpreted to be behavioral rather than epileptic. The seizures were eventually confirmed with electroencephalogram, and subsequent imaging showed enhancing masses within the sylvian fissure region that were at least partially calcified in all three cases. Each patient underwent craniotomy. In the first case, gross total resection was achieved, and in the second case, a small residual portion of tumor was densely calcified and adherent to the middle cerebral artery branches. Both of these were World Health Organization (WHO) grade I meningiomas. The third patient underwent biopsy and limited resection of meningioangiomatosis. No dural attachments were noted in any of the tumors, but one of the meningiomas was intraparenchymal in location, surrounding the sylvian fissure in both the frontal and temporal lobes, which has been described in only a small number of these cases previously. The patients underwent pre- and postsurgical neuropsychiatric testing and did not experience any significant cognitive deficits. At 10-year follow-up, the patient who had gross total resection of the tumor has had no recurrence and is seizure-free without anticonvulsant medications. The incompletely resected intraparenchymal meningioma in the second patient recurred after 5 years, however, and at repeat surgery was found to have transformed to a WHO grade II tumor. Radiation therapy was delivered and the tumor has been stable for 2 years, but the patient continues to have occasional seizures despite medication. The patient with meningioangiomatosis has had no further growth and has excellent control of seizures but remains on medication. We review the clinical presentation of these rare tumors and discuss the treatment, outcomes, and possible relationship of meningiomas to meningioangiomatosis.
小儿脑膜瘤较为罕见，但位于外侧裂区域且无硬脑膜附着的脑膜瘤极为罕见，世界文献中此前仅报道过24例。在本系列研究中，我们报告了另外2例无硬脑膜附着的外侧裂脑膜瘤以及1例颞叶内侧的外侧裂周围脑膜血管外皮细胞瘤。所有3例患者均表现为复杂部分性发作，但由于症状被误诊为行为问题而非癫痫发作，导致每例患者的诊断均被延迟。最终通过脑电图确诊为癫痫发作，随后的影像学检查显示外侧裂区域有强化肿块，所有3例患者的肿块均至少部分钙化。每位患者均接受了开颅手术。第一例患者实现了肿瘤全切，第二例患者肿瘤的一小部分残留致密钙化并与大脑中动脉分支粘连。这两例均为世界卫生组织（WHO）I级脑膜瘤。第三例患者接受了脑膜血管外皮细胞瘤的活检及有限切除。所有肿瘤均未发现硬脑膜附着，但其中1例脑膜瘤位于脑实质内，在额叶和颞叶均环绕外侧裂，此前仅有少数此类病例有过描述。患者在手术前后均接受了神经精神测试，未出现任何明显的认知缺陷。在10年的随访中，肿瘤全切的患者未复发，未服用抗惊厥药物也无癫痫发作。然而，第二例患者未完全切除的脑实质内脑膜瘤在5年后复发，再次手术时发现已转变为WHO II级肿瘤。患者接受了放射治疗，肿瘤已稳定2年，但尽管服用药物，患者仍偶尔发作癫痫。患有脑膜血管外皮细胞瘤的患者肿瘤未进一步生长，癫痫发作得到良好控制，但仍需服药。我们回顾了这些罕见肿瘤的临床表现，并讨论了其治疗、预后以及脑膜瘤与脑膜血管外皮细胞瘤之间可能的关系。
