Kim Hansol, Joo Jin-Deok, Kim Young-Hoon, Kim Chae-Yong
Seoul National University College of Medicine, Seoul, Republic of Korea.
Seoul National University College of Medicine, Seoul, Republic of Korea Department of Neurosurgery, Seoul National University Bundang Hospital, Seongnam, Republic of Korea.
BMJ Case Rep. 2014 Nov 26;2014:bcr2014207149. doi: 10.1136/bcr-2014-207149.
Haemangioblastomas (HBLs) are rare central nervous system tumours accounting for only 1.2-2.5% of all intracranial tumours. While most HBLs occur sporadically, 36-40% of cases are associated with von Hippel-Lindau (VHL) syndrome. Owing to its benign nature, sporadic cases are usually detected only when symptoms occur due to mass effect. Thus, the natural history of HBLs has only been studied in association with VHL syndrome. We present a case of sporadic HBL with a rapid evolution of its small nodule into an enlarging mural nodule with a large pseudocyst that resulted in increased intracranial pressure. Craniotomy for complete tumour removal was performed and the patient fully recovered. This case implies a regular follow-up of HBL might be mandatory even in patients without VHL.
血管母细胞瘤(HBLs)是罕见的中枢神经系统肿瘤,仅占所有颅内肿瘤的1.2 - 2.5%。虽然大多数HBLs为散发性,但36 - 40%的病例与冯·希佩尔 - 林道(VHL)综合征相关。由于其良性性质,散发性病例通常仅在因占位效应出现症状时才被发现。因此,HBLs的自然病史仅在与VHL综合征相关的情况下进行过研究。我们报告一例散发性HBL病例,其小结节迅速演变为一个增大的壁结节,并伴有一个大的假性囊肿,导致颅内压升高。进行了开颅手术以完全切除肿瘤,患者完全康复。该病例表明,即使对于没有VHL的患者,定期随访HBL可能也是必要的。