Bukhari Syed Sarmad, Bari Muhammad Ehsan, Ahmad Zubair, Din Nasir Ud
Department of Neurosurgery, Aga Khan University Hospital, Karachi, Sindh, Pakistan.
Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Sindh, Pakistan.
Surg Neurol Int. 2021 Jan 20;12:24. doi: 10.25259/SNI_477_2020. eCollection 2021.
Hemangioblastomas are benign neoplasms that consist of stromal cells and small blood vessels. They are highly vascular tumors and can arise throughout the central nervous system. This study aims to provide an overview of our experience with this rare tumor's presentation, radiology, histopathology, and outcomes as literature regarding this pathology is sparse from our country.
The study is a retrospective review of cases that were histopathology proven cases of spinal cord hemangioblastomas. The clinical characteristics of these patients were examined, and their presentation was recorded. The radiology was also reviewed to describe classic appearance on magnetic resonance imaging. A detailed review of immunohistochemistry was also performed and outcome was described.
A total of 25 cases of spinal hemangioblastomas were found in our records in the period of 2001-2019. There were 20 males (80%) and only 5 female patients (20%). Gross tumor fragments ranged in size from 0.24 cm to 10.5 cm (mean 3.28 ± 2.65). Histologically, tumor was composed of nests of large stromal cells with clear to vacuolated cytoplasm separated by thin-walled capillaries. Focal intratumoral hemorrhage was noted. No significant cytological atypia or mitotic figures were noted. Immunohistochemical stains were performed to confirm the diagnosis and exclude other tumors. Inhibin was tested in 20 cases and it was positive in 16 cases (80%). Neuron-specific enolase was positive in 6/8 cases. Cluster of differentiation (CD) CD68 was positive in 6/6 cases and vimentin in 4/4 cases. Glial fibrillary acidic protein (GFAP) and epithelial membrane antigen were performed in 14 and 8 cases, respectively, and all were negative. Cytokeratin AE1/AE3 was negative in 13/13 cases. CD34 highlighted vasculature in the 8 cases in which it was performed and was negative in tumor cells. Follow-up was available in 17 out of 25 cases and ranged from 12 months to 216 months (mean 61.8 ± 60.6 months). Recurrence occurred in 2 out of 17 (11.7%) patients for whom follow-up information was available.
Our experience shows that spinal cord hemangioblastomas can be surgically removed in most cases with a low risk of recurrence. Most patients in our study were male and unlike other studies, none of our cases showed GFAP positivity.
血管母细胞瘤是由基质细胞和小血管组成的良性肿瘤。它们是高度血管化的肿瘤，可发生于整个中枢神经系统。本研究旨在概述我们对这种罕见肿瘤的临床表现、放射学、组织病理学及治疗结果的经验，因为我国关于该病理学的文献较少。
本研究是对经组织病理学证实的脊髓血管母细胞瘤病例的回顾性分析。检查这些患者的临床特征并记录其表现。还对放射学进行回顾以描述磁共振成像的典型表现。同时对免疫组织化学进行详细分析并描述治疗结果。
在我们2001年至2019年的记录中，共发现25例脊髓血管母细胞瘤病例。其中男性20例（80%），女性仅5例（20%）。肿瘤大体标本大小从0.24厘米至10.5厘米不等（平均3.28±2.65厘米）。组织学上，肿瘤由大的基质细胞巢组成，胞质清亮至空泡状，被薄壁毛细血管分隔。可见局灶性瘤内出血。未发现明显的细胞学异型性或核分裂象。进行免疫组织化学染色以确诊并排除其他肿瘤。20例检测了抑制素，其中16例（80%）呈阳性。神经元特异性烯醇化酶在6/8例中呈阳性。分化簇（CD）CD68在6/6例中呈阳性，波形蛋白在4/4例中呈阳性。分别对14例和8例进行了胶质纤维酸性蛋白（GFAP）和上皮膜抗原检测，结果均为阴性。细胞角蛋白AE1/AE3在13/13例中呈阴性。8例进行CD34检测，其突出显示了血管系统，肿瘤细胞呈阴性。25例中有17例有随访资料，随访时间从12个月至216个月不等（平均61.8±60.6个月）。17例有随访信息的患者中有2例（11.7%）复发。
我们的经验表明，大多数情况下脊髓血管母细胞瘤可通过手术切除，复发风险较低。我们研究中的大多数患者为男性，与其他研究不同的是，我们的病例均未显示GFAP阳性。
