Al-Saiari Sultan, Al-Orabi Khalid, Farag Ahmed, Brinji Zaina, Azzouz Azza, Mohammed Tahira, Mushtaq Deepa, Hamouda Waeel
Department of Neurosurgery, King Abdullah Medical City, Mecca, Western Province, Saudi Arabia.
Department of Radiology, King Abdullah Medical City, Mecca, Western Province, Saudi Arabia.
Surg Neurol Int. 2021 Feb 17;12:58. doi: 10.25259/SNI_622_2020. eCollection 2021.
Extra-axial cerebral cavernous hemangiomas particularly those found in the sellar region, are extremely rare. Their clinical manifestations and imaging characteristics can mimic those of a pituitary adenoma thus making preoperative diagnosis difficult. Few cases are reported in the literature. We present a case, along with a comprehensive review of the literature regarding specific aspects of diagnosis and management of all similarly reported rare cases.
We present the clinical, radiological, and operative data of a rare case of a large intrasellar cavernous hemangioma in a 49-year-old female patient presented with headache and diminution of vision, which was diagnosed intraoperatively during an endonasal endoscopic transsphenoidal approach. Subtotal debulking was performed with immediate postoperative clinical improvement. The patient was then referred for radiotherapy and maintained her clinical improvement since then.
Neurosurgeons should consider this rare pathology in the preoperative differential diagnosis of sellar tumors. Bright hyperintense T2 signal with or without signal voids associated with centripetal delayed contrast enhancement in magnetic resonance imaging images might raise the suspicion which can be further confirmed intraoperatively with frozen sections. Due the reported high vascularity and intraoperative profuse bleeding leading to high operative morbidities, piecemeal subtotal resection followed by radiosurgery may be considered today as the safest and most effective strategy.
脑外海绵状血管瘤，尤其是位于鞍区的，极为罕见。其临床表现和影像学特征可类似于垂体腺瘤，因此术前诊断困难。文献报道的病例很少。我们报告一例病例，并对所有类似报道的罕见病例在诊断和治疗方面的具体情况进行全面的文献综述。
我们展示了一名49岁女性患者的临床、放射学和手术数据，该患者患有罕见的鞍内大型海绵状血管瘤，表现为头痛和视力减退，在内镜经鼻蝶窦入路手术中被诊断。进行了次全切除，术后临床症状立即改善。该患者随后接受了放射治疗，此后病情持续改善。
神经外科医生在鞍区肿瘤的术前鉴别诊断中应考虑这种罕见的病理情况。磁共振成像中T2加权像上的高信号强度，无论有无信号缺失，并伴有向心性延迟强化，可能会引起怀疑，术中冰冻切片可进一步确诊。由于报道显示其血管丰富，术中大量出血导致手术并发症发生率高，如今可考虑采用分块次全切除，随后进行放射外科治疗，这是最安全、最有效的策略。
