Intracranial Solitary Fibrous Tumor/Hemangiopericytoma A Clinicoradiological Poorly Recognized Entity-An Institutional Experience.

IF 0.9 4区 医学 Q4 CLINICAL NEUROLOGY
Nibedita Sahoo, Debahuti Mohapatra, Souvagya Panigrahi, Anasuya Lenka, Prateek DAS, Satya S Mohapatra
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引用次数: 0

Abstract

Aim: To present a series of four cases of solitary fibrous tumor/hemangiopericytoma (SFT/HPC) of the brain, which mimicked other central nervous system (CNS) tumors both clinically and radiologically.

Material and methods: This is a retrospective study over a period of two and a half years. Out of the 156 operated cases of brain tumors, four patients (2.56%) were diagnosed with SFT/HPC. The clinicoradiological details with the surgical procedure were retrieved from the archived hospital records.

Results: All cases were males, of which three were in their 5th decade while one was a 14-month-old baby. Two cases were primary and the rest were recurrent. The location of tumors was extra-axial left cerebellotentorial, clivaldural-based, left cerebellar, and in the left frontoparietal region, respectively. The clinical impression was meningioma in three cases, while it was primitive neuroectodermal tumor (PNET) /atypical teratoid/rhabdoid tumor (ATRT) in one case. With the detailed histomorphology and immunohistochemistry, the final diagnosis was anaplastic hemangiopericytoma (WHO grade III) for all the cases. During our followup, one patient died with the disease, while the rest are doing well.

Conclusion: SFT/HPC should be kept in the differential diagnosis of all dura-based hypervascular masses, especially in recurrent cases, due to its aggressiveness and high recurrence rate.

颅内单发纤维性肿瘤/血管扩张瘤:临床放射学识别率低的实体瘤--机构经验。
目的:颅内单发纤维性肿瘤(SFTs)和血管细胞瘤(HPCs)是罕见的非脑膜上皮间质肿瘤,具有NGF1-A结合蛋白2(NAB2)和信号转导和转录激活因子(STAT6.)的融合。WHO中枢神经系统(CNS)肿瘤分类(2016年)强调,NAB2/STAT6融合的分子确认或STAT6的免疫组化核表达是诊断SFT/HPC的必备条件。在此,我们介绍了4例脑部SFT/HPC的系列病例,这些病例在临床和影像学上都与其他中枢神经系统肿瘤相似:这是一项为期两年半的回顾性研究。在 156 例脑肿瘤手术病例中,有 4 例患者(2.56%)被诊断为 SFT/HPC。研究结果显示,所有病例均为男性,其中 3 例为 SFT/HPC:结果:所有病例均为男性,其中三人已年过5旬,一人是14个月大的婴儿。肿瘤位置分别位于轴外左侧小脑脑室、硬脑膜基底、左侧小脑和左侧额顶区。通过详细的组织形态学和免疫组化检查,所有病例的最终诊断均为无弹性血管细胞瘤(WHO III 级)。在我们的随访过程中,有一名患者因病去世,其余患者的情况良好:结论:由于SFT/HPC具有侵袭性和高复发率,因此应将其保留在所有硬脑膜基底高血管肿块的鉴别诊断中,尤其是复发病例。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Turkish neurosurgery
Turkish neurosurgery 医学-临床神经学
CiteScore
1.50
自引率
12.50%
发文量
126
审稿时长
2 months
期刊介绍: Turkish Neurosurgery is a peer-reviewed, multidisciplinary, open access and totally free journal directed at an audience of neurosurgery physicians and scientists. The official language of the journal is English. The journal publishes original articles in the form of clinical and basic research. Turkish Neurosurgery will only publish studies that have institutional review board (IRB) approval and have strictly observed an acceptable follow-up period. With the exception of reference presentation, Turkish Neurosurgery requires that all manuscripts be prepared in accordance with the Uniform Requirements for Manuscripts Submitted to Biomedical Journals.
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