Rushing Elisabeth J, Cooper Patrick B, Quezado Martha, Begnami Maria, Crespo Ana, Smirniotopoulos James G, Ecklund James, Olsen Cara, Santi Mariarita
Department of Neuropathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
J Neurooncol. 2007 Dec;85(3):297-305. doi: 10.1007/s11060-007-9411-6. Epub 2007 Jun 14.
Subependymomas are rare ependymal neoplasms. To date, a large clinicopathologic study of these benign neoplasms treated with modern neurosurgical techniques has not been reported.
Eighty-three cases of subependymoma were retrieved from the files of the Armed Forces Institute of Pathology. Clinicopathological features were reviewed; chromogenic in situ hybridization analysis for chromosome 22 was performed (n = 8), and patient follow-up was obtained (n = 34). Overall, the patients included 68 males and 15 females, 1.5 to 85 years of age (mean, 51.0 years). Twenty-seven cases were discovered at autopsy and the remaining were surgical specimens (n = 56). Tumors arose in the posterior fossa (n = 43), lateral ventricles (n = 37), spinal cord (2) and only one arose in the temporal horn. Tumors ranged in size from 2.0 mm to 60 mm in greatest dimension (mean, 23.0 mm). Eighteen-percent (15/83) of subependymomas exhibited a mixed histologic pattern; that is, subependymoma together with another glial tumor. The most common mixture (13/15) was subependymoma and ependymoma. Surgical excision was used in all symptomatic patients; 10 patients received radiation. Four patients developed a recurrence due to incomplete excision. All patients were without evidence of disease at the last follow-up: alive (n = 28) or dead (n = 8).
Age is the only variable found to be significantly associated with survival. Currently, surgical methods result in an excellent long-term clinical outcome. Subependymomas do not appear to be associated with NF2 mutations.
室管膜下瘤是一种罕见的室管膜肿瘤。迄今为止,尚未有关于采用现代神经外科技术治疗这些良性肿瘤的大型临床病理研究报告。
从武装部队病理研究所的档案中检索出83例室管膜下瘤病例。回顾临床病理特征;对22号染色体进行显色原位杂交分析(n = 8),并对患者进行随访(n = 34)。总体而言,患者包括68名男性和15名女性,年龄在1.5岁至85岁之间(平均51.0岁)。27例在尸检时发现,其余为手术标本(n = 56)。肿瘤发生于后颅窝(n = 43)、侧脑室(n = 37)、脊髓(2例),仅1例发生于颞角。肿瘤最大直径范围为2.0毫米至60毫米(平均23.0毫米)。18%(15/83)的室管膜下瘤呈现混合组织学模式;即室管膜下瘤与另一种神经胶质肿瘤并存。最常见的混合类型(13/15)是室管膜下瘤和室管膜瘤。所有有症状的患者均采用手术切除;10例患者接受了放疗。4例患者因切除不完全而复发。在最后一次随访时,所有患者均无疾病证据:存活(n = 28)或死亡(n = 8)。
年龄是唯一被发现与生存显著相关的变量。目前,手术方法可带来优异的长期临床结果。室管膜下瘤似乎与NF2突变无关。