Tong Yi, Sirhan Denis, Cortes Maria
Department of Radiology, McGill University Health Center, Montreal, Quebec, Canada.
Department of Neurosurgery, Montreal Neurological Hospital and Institute, Montreal, Quebec, Canada.
Indian J Radiol Imaging. 2021 Apr;31(2):499-509. doi: 10.1055/s-0041-1734335. Epub 2021 Sep 3.
Our case report presents the first case of suprasellar hemangioblastoma diagnosed preoperatively with dynamic computed tomography angiography (four-dimensional [4D] CTA) in a patient without Von Hippel-Lindau (VHL) disease. We illustrate the imaging characteristics of these exceedingly rare tumors and discuss the role of 4D CTA in confirming this diagnosis and guiding surgical management. Finally, we present a literature review of imaging findings, differential diagnosis, management, and prognosis. A 39-year-old woman known for diabetes mellitus type II and dyslipidemia presented with headache, bitemporal hemianopsia, and mild hyperprolactinemia. Initial diagnosis of suprasellar meningioma separate from pituitary gland was revised to definitive diagnosis of suprasellar hemangioblastoma after 4D CTA. Suprasellar hemangioblastomas are extremely rare, often associated to VHL disease. They present as enhancing as suprasellar mass with prominent intra- and peritumoral vascular flow-voids on magnetic resonance imaging. 4D CTA confirms their vascular nature, demonstrates characteristic rapid shunting with feeding arteries, and enlarged draining veins, and is important in guiding surgical management.
我们的病例报告展示了首例在无冯·希佩尔-林道(VHL)病的患者中通过动态计算机断层血管造影术(四维[4D]CTA)术前诊断出的鞍上血管母细胞瘤。我们阐述了这些极其罕见肿瘤的影像学特征,并讨论了4D CTA在确诊该疾病及指导手术治疗中的作用。最后,我们对影像学表现、鉴别诊断、治疗及预后进行了文献综述。一名患有II型糖尿病和血脂异常的39岁女性,出现头痛、双颞侧偏盲和轻度高催乳素血症。最初诊断为与垂体分离的鞍上脑膜瘤,经4D CTA检查后确诊为鞍上血管母细胞瘤。鞍上血管母细胞瘤极为罕见,常与VHL病相关。它们在磁共振成像上表现为鞍上强化肿块,瘤内及瘤周有明显的血管流空。4D CTA证实了其血管性质,显示出供血动脉的特征性快速分流及引流静脉增粗,对指导手术治疗很重要。