Pakdaman Michael N, Austin Matthew J, Bannykh Serguei, Pressman Barry D
Department of Imaging, Cedars Sinai S. Mark Taper Foundation Imaging Center, Los Angeles, USA.
Department of Pathology and Laboratory Medicine, Cedars Sinai Medical Center, Los Angeles, USA.
J Radiol Case Rep. 2017 May 31;11(5):1-6. doi: 10.3941/jrcr.v11i5.2981. eCollection 2017 May.
Hemangioblastomas are rare vascular tumors most often found in the posterior fossa and cervical spinal cord and commonly associated with von Hippel-Lindau Disease. We report a case of sporadic hemangioblastoma in a patient without von Hippel-Lindau Disease. Imaging characteristics included a solid, suprasellar mass that was homogeneously enhancing. These findings most resembled a pituicytoma or choroid glioma because of the close association with the infundibulum and the homogeneous avid enhancement. Microscopically, the neoplasm was seen to be composed of vascular channels associated with foamy stromal cells, containing clear cytoplasmic vacuoles. Microscopic and immunohistochemical findings were consistent with hemangioblastoma. Hemangioblastomas are a rare form of vascular tumor most commonly associated with von-Hippel Lindau disease. Our finding of non-cystic hemangioblastoma arising from the infundibulum demonstrates that, while rare, hemangioblastomas should be considered on the differential diagnosis for an avidly enhancing suprasellar mass.
血管母细胞瘤是一种罕见的血管性肿瘤,最常发生于后颅窝和颈髓,通常与冯·希佩尔-林道病相关。我们报告一例无冯·希佩尔-林道病的散发性血管母细胞瘤患者。影像学特征包括一个实性的鞍上肿块,呈均匀强化。由于与漏斗紧密相连且均匀明显强化,这些表现最类似于垂体细胞瘤或脉络丛胶质瘤。显微镜下,肿瘤由与泡沫状基质细胞相关的血管通道组成,基质细胞含有透明的胞质空泡。显微镜及免疫组化结果符合血管母细胞瘤。血管母细胞瘤是一种罕见的血管性肿瘤,最常与冯·希佩尔-林道病相关。我们发现起源于漏斗的非囊性血管母细胞瘤表明,尽管罕见,但对于明显强化的鞍上肿块进行鉴别诊断时应考虑血管母细胞瘤。