skull base tumor

颅底肿瘤
  • 文章类型: Journal Article
    反义长链非编码RNA(AS-lncRNA)代表了一类新的RNA分子。近年来,已经发现AS-lncRNAs在各种生物过程中发挥关键作用,特别是在肿瘤的发作和进展中。颅底肿瘤,起源于大脑的底部,表现出与临床特征显着相关的AS-lncRNA的特异性表达模式。这使得AS-lncRNA成为有希望的候选肿瘤标志物。功能研究表明,AS-lncRNAs可以通过充当miRNA海绵并与RBPs相互作用来调节基因表达。因此,它们在肿瘤细胞周期中起关键作用,凋亡,血管生成,入侵,和转移过程。进一步探索肿瘤中AS-lncRNA的机制对于更深入地了解病因具有重要的理论意义。发病机制,和颅底肿瘤的RNA动力学。此外,AS-lncRNA可以作为早期诊断的分子标志物或潜在靶标。它们的潜力延伸到疗效评估,预后预测,和基因治疗,表明广泛的临床应用。总之,AS-lncRNA作为与颅底肿瘤的发病和进展有关的有前途的分子标志物出现。
    Antisense long non-coding RNA (AS-lncRNA) represents a novel class of RNA molecules. In recent years, it has been discovered that AS-lncRNAs play crucial roles in various biological processes, particularly in the onset and progression of tumors. Skull base tumors, originating from the base of the brain, exhibit specific expression patterns of AS-lncRNA which correlate significantly with clinical characteristics. This makes AS-lncRNA a promising candidate as a tumor marker. Functional studies have revealed that AS-lncRNAs can regulate gene expression by acting as miRNA sponges and interacting with RBPs. Consequently, they play pivotal roles in tumor cell cycle, apoptosis, angiogenesis, invasion, and metastasis processes. Further exploration into the mechanisms of AS-lncRNA in tumors holds substantial theoretical significance for deeper insights into the etiology, pathogenesis, and RNA dynamics of skull base tumors. Moreover, AS-lncRNA could serve as molecular markers or potential targets for early diagnosis. Their potential extends to efficacy assessment, prognosis prediction, and gene therapy, suggesting broad clinical applications. In summary, AS-lncRNA emerges as a promising molecular marker implicated in the onset and progression of skull base tumors.
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  • 文章类型: Journal Article
    副骨瘤并发颅内和眼眶延伸,颅骨穹窿增生,颅内黏液囊肿和炎性假瘤异常。一名68岁的男子抱怨左眼进行性眼球突出和复发性角膜结膜炎的长期历史,在向上凝视的限制下,被观察到。对比增强磁共振成像显示额窦病变延伸至左前窝和眼眶,具有颅内囊性成分和异质对比增强。头部计算机断层扫描证实了病变的双重钙化-囊性。左眶上翼点入路可完全切除黏液囊肿并钻孔颅内和眼眶骨瘤,包括鼻窦内成分。额窦被颅骨化,有一个皮瓣,由明胶海绵增强,反映在前颅底/眶顶。术后过程顺利;MRI描绘了眼球突出的分辨率。组织学检查有利于与颅内黏液囊肿相关的寄生虫骨瘤,额骨骨增生和炎性假瘤。
    Parasinusal osteoma complicated by intracranial and orbit extension, cranial vault hyperostosis, intracranial mucocele, and inflammatory pseudotumor is exceptional. A 68-year-old man presented with a long history of progressive proptosis and recurrent episodes of keratoconjunctivitis in the left eye, with restriction in upward gaze. Contrast-enhanced magnetic resonance imaging revealed a frontal sinus lesion extending to the left anterior fossa and orbit, featuring an intracranial cystic component and heterogeneous contrast enhancement. Head computed tomography confirmed the double calcific-cystic nature of the lesion. A left supraorbital-pterional approach allowed complete resection of mucocele and drilling of intracranial and orbital osteoma, including the intrasinusal component. The frontal sinus was cranialized, and a flap of pericranium, reinforced by Gelfoam sponge, was reflected on the anterior cranial base/orbital roof. The postoperative course was uneventful; magnetic resonance imaging depicted resolution of proptosis. Histological examination favored parasinusal osteoma associated with intracranial mucocele, frontal bone hyperostosis, and inflammatory pseudotumor.
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  • 文章类型: Journal Article
    目的:分析经手术治疗的颅底和颞骨软骨肉瘤的总体长期预后。
    方法:彻底评估了1983年至2024年间手术治疗的颅底和颞骨软骨肉瘤患者的病历。
    结果:在我们中心进行的5000多次颅底手术中,只有29例患者经组织病理学证实为颅底和颞骨软骨肉瘤。患者的平均年龄为45.6岁,男女比例为1.9:1。最常见的症状包括听力损失(58.6%),耳鸣(41.4%),复视(31%),发音困难(24.1%),吞咽困难(20.7%),眩晕(10.3%),和头晕(10.3%)。29例患者中最常见的病变位置如下:岩斜区域(34.5%),颈静脉孔(27.6%),岩尖(17.2%),中耳(13.8%),其他(3.4%)。TO,IFTA,IFTB,IFTC,POTS,和联合手术方法是常用的。总清除率和复发率分别为82.6%和13.8%。6例患者的随访时间超过5年,少于10年,而10例患者的随访时间超过10年。
    结论:颅底和颞骨软骨肉瘤是一种非常罕见的病理。由于其多个潜在的起源和组织学特异性,它给我们带来了巨大的挑战。总切除是颅底和颞骨软骨肉瘤的主要治疗方法。个性化决策应基于以下几个方面来考虑:肿瘤,病人,和外科医生的因素。术后放射治疗是II级和III级病变手术治疗的补充,以实现长期生存。
    OBJECTIVE: To analyze the overall long-term outcome of surgically treated skull base and temporal bone chondrosarcomas.
    METHODS: The medical records of patients with surgically treated skull base and temporal bone chondrosarcomas between 1983 and 2024 were thoroughly evaluated.
    RESULTS: Out of a total of over 5000 skull base surgeries performed at our center, only 29 patients had histopathologically confirmed chondrosarcomas of the skull base and temporal bone. The mean of patients age was 45.6, and the male-to-female ratio was 1.9:1. The most common symptoms included hearing loss (58.6%), tinnitus (41.4%), diplopia (31%), dysphonia (24.1%), dysphagia (20.7%), vertigo (10.3%), and dizziness (10.3%). The most frequent locations of lesions among the 29 patients are as follows: petroclival region (34.5%), jugular foramen (27.6%), petrous apex (17.2%), middle ear (13.8%), others (3.4%). TO, IFTA, IFTB, IFTC, POTS, and combined surgical approaches were commonly used. The rate of gross total removal and recurrence was 82.6% and 13.8% respectively. The follow-up duration of 6 patients was more than five years and less than ten years whereas ten patients had more than ten years of follow-up.
    CONCLUSIONS: Chondrosarcoma of the skull base and temporal bone is a very rare pathology. Due to its multiple potential sites of origin and histological specificity, it presents us with significant challenges. Gross total removal is the primary treatment for chondrosarcoma of the skull base and temporal bone. Personalized decision-making should be considered based on the following aspects: tumor, patient, and surgeon\'s factors. Postoperative radiation therapy is complementary to surgical treatment in grades II and III lesions to achieve long-term survival.
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  • 文章类型: Journal Article
    四个下颅神经的联合缺陷(CNIX,X,XI,和XII)最初是由法国医生Collet(1915)和Sicard(1917)在第一次世界大战期间描述的。这种罕见的神经系统临床表现缺乏关于其流行病学的系统证据,临床表现,治疗策略,和结果。我们根据系统评价和荟萃分析指南的首选报告项目,在Medline数据库上对Collet-Sicard综合征(CSS)进行了系统评价和荟萃分析。该研究共发表了84篇文章,其中最终保留了73份个案报告。平均年龄为53.7(±16)岁,男女比例为1.8/1。CSS首先由肿瘤引起(38.4%),其次是血管病因(28.8%),外伤(16.4%),和感染(6.8%),在其他人中。17例患者需要临时肠内营养(23.3%)。四个CN显示出完全或部分恢复的重要机会:CNIX为52.1%(p<0.001),CNX和CNXII的46.6%(p<0.001),CNXI为39.7%(p=0.002)。与感染(60%)相比,肿瘤原因显示出良好的CN恢复(7.1%)的机会显着降低,血管(52.4%),和创伤(41.7%)(p<0.001)。年龄较大(>53岁)与CN预后不佳无关(p=0.763)。大多数患者(71.2%)表现出良好的结果(格拉斯哥预后量表评分≥4)。所有死亡患者(6.8%)均患有颅底肿瘤。CSS是一种罕见的疾病,需要及时的临床和放射学诊断以及多学科管理。血管或感染相关的CSS似乎表现出相当好的预后,紧随其后的是创伤,而与肿瘤相关的CSS似乎患有更令人沮丧的预后。
    Combined deficit of the four lower cranial nerves (CN IX, X, XI, and XII) was originally described by French physicians Collet (1915) and Sicard (1917) during World War I. To date though, this rare neurological clinical picture lacks systematic evidence regarding its epidemiology, clinical presentation, treatment strategies, and outcome. We conducted a systematic review and meta-analysis concerning Collet-Sicard syndrome (CSS) on Medline database in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The research yielded 84 articles among which 73 individual case reports were eventually retained. Mean age was 53.7 (± 16) years old and the male-to-female ratio was 1.8/1. CSS was firstly caused by tumors (38.4%), following by vascular etiologies (28.8%), trauma (16.4%), and infection (6.8%), among others. Temporary enteral nutrition was required for 17 patients (23.3%). The four CN presented significant chances of complete or partial recovery: 52.1% for CN IX ( p  < 0.001), 46.6% for CN X and CN XII ( p  < 0.001), and 39.7% for CN XI ( p  = 0.002). Tumoral causes presented significantly lower chances of favorable CN recovery (7.1%) compared to infection (60%), vascular (52.4%), and trauma (41.7%) ( p  < 0.001). Older age (> 53 years old) was not associated with a dismal CN prognostic ( p  = 0.763). Most patients (71.2%) presented a favorable outcome (Glasgow Outcome Scale score ≥ 4). All the patients who died (6.8%) suffered from skull base tumors. CSS is a rare condition requiring prompt clinical and radiologic diagnostic and multidisciplinary management. Vascular or infectious-related CSS seem to present a rather good prognostic, closely followed by trauma, whereas tumoral-related CSS seem to suffer from a more dismal prognostic.
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  • 文章类型: Case Reports
    引言涉及中枢神经系统(CNS)的复发性急性淋巴细胞白血病(ALL)是导致发病率和死亡率的重要问题。鉴于中枢神经系统复发患者的不良预后,了解ALL涉及颅内复发的表现和治疗方式至关重要.这里,我们介绍了1例小儿复发性ALL的复杂病例.病例报告一名11岁的患者以广泛的颅底病变和软脑膜疾病的形式出现ALL的双重复发。对于颅底病变,她接受了化疗和放疗的非手术治疗,这导致病变的大小显著减少。然而,患者在完成治疗5个月后发现早期复发伴脑膜增强,导致脑积水.分流成功。目前,患者接受每月鞘内化疗,每2个月进行一次脑脊液取样和骨髓活检.讨论我们报告了化疗和放疗在减少广泛颅底病变的大小方面的重要作用,将患者从与手术相关的风险中拯救出来。这个病人初次复发,颅内受累的巨大颅底病变,是复发性ALL的不寻常表现。软脑膜疾病的额外早期复发进一步使这种情况变得独特,并且管理更加微妙。这里,我们展示了一种成功治疗患者的多学科方法,这可以帮助指导未来类似患者的管理。
    Introduction  Relapsed acute lymphoblastic leukemia (ALL) involving the central nervous system (CNS) is a significant issue that contributes to both morbidity and mortality. Given the poor outcomes in patients with CNS relapse, understanding how ALL involving intracranial relapse presents and is treated is critical. Here, we present a complex case of relapsed recurrent ALL in a pediatric patient. Case Report  An 11-year-old patient presented with double relapse of ALL in the form of an extensive skull base lesion and again with leptomeningeal disease. For the skull base lesion, she was treated nonsurgically with chemotherapy and radiation, which led to a remarkable reduction in the size of the lesion. However, she was found to have early recurrence with leptomeningeal enhancement resulting in hydrocephalus 5 months after completing therapy. A shunt was placed successfully. Currently, she is being managed with monthly intrathecal chemotherapy with cerebrospinal fluid sampling and bone marrow biopsies every 2 months. Discussion  We report the significant effect of chemotherapy and radiotherapy in reducing the size of the extensive skull base lesion, saving the patient from the risks associated with surgery. This patient\'s initial relapse, with a large skull base lesion that had intracranial involvement, is an unusual presentation of relapsed ALL. The additional early recurrence of leptomeningeal disease further makes this case unique and the management even more nuanced. Here, we demonstrate a multidisciplinary approach for the successful treatment of our patient, which can help guide the management of similar patients in the future.
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  • 文章类型: English Abstract
    Objective:To preliminarily study the practical value of Indocyanine green(ICG) molecular fluorescence imaging technology in nasal endoscopic tumor surgery. Methods:Five patients with tumors related to nasal sinuses, orbital wall and skull base in the Department of Otolaryngology head and Neck Surgery, General Hospital of Xinjiang Military Command from December 2022 to April 2023 were enrolled. Among them, 3 were benign tumors and 2 were malignant tumors. All patients underwent surgery under the guidance of ICG molecular fluorescence imaging. ICG was administered intravenously through cubital vein at a dose of 0.5 mg/kg 12 to 24 h before surgery. Tumors were labeled by fluorescence imaging during the operation. surgeons cleared the tumor tissue strictly according to the labeled range and depth, malignant tumors were further expanded and cleaned according to pathology results. Results:All 5 patients achieved accurate tumor localization with the aid of fluorescence imaging technology. Resections were performed with reference to fluorescent labeling boundaries, all patients achieved complete tumor cleanup or negative margins. Conclusion:For tumor-related surgery under nasal endoscopy, ICG molecular fluorescence imaging technology can not only achieve accurate real-time positioning, but also provide evidence for surgeons to judge tumor boundaries. Therefore, we believe that the technology should have certain practical value in nasal endoscopic tumor surgery.
    目的:初步探讨吲哚菁绿(indocyanine green,ICG)分子荧光成像技术在鼻内镜下肿瘤手术中的应用价值。 方法:以2022年12月—2023年4月间新疆军区总医院耳鼻咽喉头颈外科5例鼻腔鼻窦、眶壁、颅底相关肿瘤患者作为研究对象,其中良性肿瘤3例,恶性肿瘤2例。所有患者皆在ICG分子荧光成像技术指导下完成手术。术前12~24 h按0.5 mg/kg剂量经肘静脉静推ICG;术中通过荧光成像对肿瘤进行标记,术者严格按标记范围、深度清除肿物组织,恶性肿瘤根据病检结果做进一步扩大清理。 结果:5例患者在荧光成像技术辅助下,均实现了精准的肿瘤定位;参照荧光标记界限进行切除,对患者均做到了肿瘤的彻底清理或切缘阴性。 结论:针对鼻内镜下肿瘤相关手术,ICG分子荧光成像技术不仅能做到实时精准定位,而且能为术者判断肿瘤边界提供依据。因此认为该项技术在鼻内镜肿瘤手术中应具有一定的应用价值。.
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  • 文章类型: Journal Article
    鼻旁和前颅底(ASB)恶性肿瘤的肿瘤切除后重建额基底缺损仍然具有挑战性。重建无效会导致脑脊液漏,脑膜炎,和张力性气颅。
    这项研究的目的是分析有或没有植骨的前颅底重建术后并发症的发生率。
    在这项回顾性研究中,我们纳入了2013年10月至2022年12月期间鼻旁和/或前颅底恶性肿瘤切除术后的患者.分析了有关颅底重建类型的并发症。
    确定了11名患者(2名女性,9男,年龄(中位数,SD)64±14.1年(范围38-81)。鼻旁窦和鼻腔癌9例,嗅神经母细胞瘤2例。总生存期为22.5±28个月(范围:5-78),无进展生存期为17.0±20.3个月(范围:11-78).在三例病例中,使用分裂移植物进行了骨颅底重建。需要手术干预的术后并发症在骨重建组中占33%(1张力性气颅)和50%(3例脑脊液漏,一种感染)在非骨重建组中。
    结构骨碎片移植的结构增强可能为ASB提供额外的支持,并防止CSF渗漏或脑膨出。尤其是在扩展到中颅窝的晚期鼻窦恶性肿瘤的大(>10cm2)骨缺损中,应该考虑重建可能性的完整武器库。
    UNASSIGNED: The reconstruction of frontobasal defects following oncologic resections of paranasal and anterior skull base (ASB) malignancies remains challenging. Ineffective reconstruction could lead to cerebrospinal fluid leak, meningitis, and tension pneumocephalus.
    UNASSIGNED: Aim of this investigation was to analyse postoperative complication rates with or without bone graft for anterior skull base reconstruction.
    UNASSIGNED: In this retrospective study, we included patients following resection of paranasal and/or anterior skull base malignancies between October 2013 and December 2022. Complications were analysed with regards to the type of skull base reconstruction.
    UNASSIGNED: Eleven patients were identified (2 female, 9 male, age (median, SD) 64 ± 14.1 years (range 38-81). There were nine cases of paranasal sinus and nasal cavity carcinomas and two cases of olfactory neuroblastomas. Overall survival was 22.5 ± 28 months (range: 5-78), progression free survival was 17.0 ± 20.3 months (range: 11-78). Bone skull base reconstruction using a split graft was performed in three cases. Postoperative complications requiring surgical intervention were seen in 33% (one tension pneumocephalus) of cases in the bone reconstruction group and 50% (three patients with cerebrospinal fluid leak, one infection) in the non-bone reconstruction group.
    UNASSIGNED: The structural reinforcement of structural bone chip grafting might provide additional support of the ASB and prevent CSF leakage or encephalocele. Especially in large (>10 cm2) bone defects of advanced sinonasal malignancies extending into the middle cranial fossa, the full armamentarium of reconstruction possibilities should be considered.
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  • 文章类型: Journal Article
    背景:腺泡细胞癌(AcCC),唾液腺的罕见恶性肿瘤,经常复发和转移,特别是在颅底。传统的根治性切除术可以侵入性的颅底AcCC邻近颅神经和主要脉管系统,立体定向放射外科(SRS)作为替代方案的有效性尚不明确。
    方法:本病例报告详细介绍了SRS在复发性颅底AcCC中的应用。一名71岁的男性,有23年前的右下颌AcCC切除史,经历了涉及右海绵窦和鼻腔的肿瘤复发。他接受了鼻内镜手术,然后是针对不同肿瘤位置的SRS-海绵窦至翼腭窝,上颌窦,和clivus-各自的处方剂量为20Gy至40%至50%的等剂量线。在第一次颅底转移后,内镜手术后再进行局部SRS治疗,可获得12年无后遗症的生存期.
    结论:这是一份报告,表明颅底AcCC的SRS可以实现良好的局部控制,功能保存,和长期生存。考虑到病变倾向于多次局部复发,SRS可能适用于颅底AcCC。需要进一步的研究来验证治疗的疗效。
    BACKGROUND: Acinic cell carcinomas (AcCCs), rare malignancies of the salivary glands, often recur and metastasize, particularly in the skull base. Conventional radical resection can be invasive for skull base AcCCs adjacent to cranial nerves and major vasculature, and the effectiveness of stereotactic radiosurgery (SRS) as an alternative is not well established.
    METHODS: This case report details the application of SRS for recurrent skull base AcCCs. A 71-year-old male with a history of resection for a right mandibular AcCC 23 years earlier experienced tumor recurrence involving the right cavernous sinus and nasal cavity. He underwent endoscopic transnasal surgery followed by SRS targeting different tumor locations-the cavernous sinus to the pterygopalatine fossa, maxillary sinus, and clivus-each with a prescribed dose of 20 Gy to the 40% to 50% isodose line. After the first skull base metastasis, additional sessions of localized SRS after endoscopic surgery led to a 12-year survival without sequela.
    CONCLUSIONS: This is a report indicating that SRS for skull base AcCCs can achieve favorable local control, functional preservation, and long-term survival. SRS may be suitable for skull base AcCC given the lesion\'s tendency toward multiple local recurrences. Further investigation is needed to validate the treatment\'s efficacy.
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  • 文章类型: Case Reports
    转移性嗜铬细胞瘤/副神经节瘤(MPP)是一种罕见的内分泌肿瘤,起源于肾上腺外嗜铬细胞,例如交感神经和副交感神经的副神经节细胞。它通常会导致多个实体瘤,并表现出强烈的侵袭性,预后不良。据报道,5年生存率低于50%。尚未报道最初诊断时脑和腹膜后转移的病例。我们报告了一名41岁的男性患者,最初被诊断为大脑和腹膜后MPP,他在我们中心接受了多学科协作手术并同时切除了两个肿瘤。术后病理显示颅底肿瘤浸润性生长。患者选择接受酪氨酸激酶抑制剂舒尼替尼作为靶向治疗。术后3个月随访显示,患者恢复良好,无转移或复发迹象。我们提出了类似情况下的多学科手术,以加强治疗和术后管理。患者在术后随访期间表现出良好的预后,表明同时进行多学科手术可能为MPP患者带来更大的益处.
    Metastatic pheochromocytoma/paraganglioma (MPP) is a rare endocrine tumor that originates from extra-adrenal chromaffin cells such as the paraganglia cells of sympathetic and parasympathetic nerves. It usually causes multiple solid tumors and exhibits strong aggressiveness with poor prognosis, with a reported 5-year survival rate of less than 50%. Cases of brain and retroperitoneal metastases at the initial diagnosis have not yet been reported. We report a 41-year-old male patient initially diagnosed with MPP in the brain and retroperitoneum who underwent multi-disciplinary collaborative surgery and simultaneous removal of two tumors at our center. Postoperative pathology revealed infiltrative growth of a skull base tumor. The patient chose to receive the tyrosine kinase inhibitor sunitinib as a targeted treatment. A 3-month follow-up after surgery showed that the patient recovered well without signs of metastasis or recurrence. We present multi-disciplinary surgery under similar circumstances for enhanced treatment and postoperative management. The patient demonstrates a favorable prognosis during postoperative follow-up, indicating that simultaneous multidisciplinary surgery may offer greater benefits for MPP patients.
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  • 文章类型: Journal Article
    背景:大脑前动脉(ACA)的双侧视神经下起源是一种罕见的解剖学变异,可在前颅底手术中遇到。ACA起源于颈内动脉(ICA),位于眼动脉水平和内侧,在同侧视神经下方行进。在这里,作者讨论了解剖变体的不同配置,其患病率,以及导致这种异常的可变配置的假设。
    方法:一名67岁的妇女在一周的时间内表现为头晕恶化,并被发现患有视神经的大的左蝶骨脑膜瘤,海绵状,和鞍上延伸。肿瘤合并了左侧上滑膜ICA及其分支。她接受了左侧改良的眶骨开颅手术以切除肿瘤。早期识别异常的ACA解剖结构对于避免血管损伤至关重要。
    结论:虽然在血管病变的治疗过程中通常会遇到这种变体-即,颅内动脉瘤-在治疗任何前颅底病变时应牢记其存在。未能解释这种变异的存在可能导致潜在的术中并发症。
    BACKGROUND: A bilateral infraoptic origin of the anterior cerebral arteries (ACAs) is a rare anatomical variant that can be encountered during anterior skull base surgery. The ACAs arise from the internal carotid artery (ICA) at the level of the ophthalmic artery and course medially, traveling inferior to the ipsilateral optic nerves. Herein, the authors discuss the different configurations of the anatomical variant, its prevalence, and hypotheses leading to the variable configuration of this anomaly.
    METHODS: A 67-year-old woman presented with worsening dizziness over a week-long period and was found to have a large left sphenocavernous meningioma with optic, cavernous, and suprasellar extension. The tumor incorporated the left supraclinoid ICA and its branches. She underwent a left modified orbitozygomatic craniotomy for tumor resection. Early identification of the aberrant ACA anatomy was crucial in avoiding vascular injury.
    CONCLUSIONS: While this variant is typically encountered during the treatment of vascular pathologies-namely, intracranial aneurysms-its existence should be kept in mind during the treatment of any anterior skull base pathology. Failure to account for the presence of this variant may lead to potential intraoperative complications.
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