Park Chang Hyun, Lee Chang-Hyun, Hyun Seung Jae, Jahng Tae-Ahn, Kim Hyun-Jib, Kim Ki-Jeong
Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
J Korean Neurosurg Soc. 2012 Sep;52(3):221-7. doi: 10.3340/jkns.2012.52.3.221. Epub 2012 Sep 30.
Spinal cord hemangioblastomas are rare tumors. Despite their benign, slow-growing nature, they can cause severe neurological consequences. The purpose of this study was to evaluate variable factors, including clinical features, tumor findings, the extent of resection, and its recurrence or progression, which determine postoperative functional outcomes.
This study included sixteen patients at our institute who underwent microsurgical resection for sporadic spinal intramedullary hemangioblastomas and spinal intramedullary hemangioblastomas associated with von Hippel-Lindau (VHL) disease, between June 2003 and March 2012.
A total of 30 operations were performed. Total resection (TR) of the tumor was achieved in 10 patients, and subtotal resection (STR) was achieved in 6. Postoperatively, the initial presenting symptoms were improved in 18.7% of the patients and were unchanged in 56.3%, but 25% were worse. Stable postoperative neurological functions were found in 83% of patients with preoperative McCormick grade I, and TR was achieved in 75% of these patients. In the STR group, poorer neurological status was observed in one patient, despite multiple operations. There were no poorer outcomes in the four cases of VHL disease. Various factors were analyzed, but only a correlation between the pre- and postoperative neurological status was verified in the TR-group patients.
Preoperative focal neurological impairment and meticulous microsurgical manipulation may be predictors of favorable outcomes for solitary hemangioblastomas. In addition, the preservation of function is more important than the extent of resection in VHL disease.
脊髓血管母细胞瘤是罕见肿瘤。尽管其性质良性、生长缓慢,但可导致严重的神经后果。本研究的目的是评估各种可变因素,包括临床特征、肿瘤表现、切除范围及其复发或进展情况,这些因素决定术后功能结果。
本研究纳入了2003年6月至2012年3月期间在我院接受显微手术切除散发性脊髓髓内血管母细胞瘤和与冯·希佩尔-林道(VHL)病相关的脊髓髓内血管母细胞瘤的16例患者。
共进行了30次手术。10例患者实现了肿瘤全切(TR),6例实现了次全切(STR)。术后,18.7%的患者初始出现的症状得到改善,56.3%的患者症状未改变,但25%的患者症状恶化。术前McCormick I级的患者中83%术后神经功能稳定,其中75%实现了全切。在次全切组中,1例患者尽管接受了多次手术,但神经状态较差。4例VHL病患者未出现更差的结果。对各种因素进行了分析,但仅在全切组患者中验证了术前和术后神经状态之间的相关性。
术前局灶性神经功能损害和细致的显微手术操作可能是孤立性血管母细胞瘤预后良好的预测因素。此外,在VHL病中,功能的保留比切除范围更重要。