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丘脑原发性颅内胆脂瘤。

Primary intracranial cholesteatoma in the thalamus.

作者信息

Golden Nyoman, Lauren Christopher, Saputra Angky, Wibawa Ida Bagus Gede Adiguna, Surya Kencana I Gusti Ketut Agung, Bangun Kristian Gerry Raymond Sinarta, Mahadewa Tjokorda Gde Bagus

机构信息

Neurosurgery Division, Department of Surgery, Faculty of Medicine, Udayana University, Ngoerah Hospital, Bali, Indonesia.

出版信息

Surg Neurol Int. 2025 May 16;16:187. doi: 10.25259/SNI_252_2025. eCollection 2025.

Abstract

BACKGROUND

Primary intracranial cholesteatomas are rare, benign lesions of keratinizing squamous epithelium, often arising from middle ear extension. However, their occurrence in the thalamus is exceptionally rare, with only one prior case reported.

CASE DESCRIPTION

A 48-year-old male presented with progressive headaches over 3 years and difficulty with upward gaze. Neurological examination revealed bilateral upward gaze palsy. Magnetic resonance imaging demonstrated an intra-axial cystic mass with a solid component in the right thalamus extending to the midbrain, causing obstructive hydrocephalus. A craniotomy with subtotal tumor resection was performed. Histopathology confirmed cholesteatoma. Postoperatively, the patient's symptoms improved, but hydrocephalus recurred, necessitating an endoscopic third ventriculostomy. Five months later, imaging showed an increase in residual mass size, but further, surgical intervention was declined due to financial constraints. The patient continued outpatient follow-up and maintained functional independence despite persistent symptoms.

CONCLUSION

Thalamic cholesteatomas are rare and diagnostically challenging. Their deep-seated location complicates surgical resection, often necessitating subtotal removal to preserve neurological function. Close postoperative monitoring is essential due to the high recurrence risk. Further research is needed to optimize diagnostic strategies and explore alternative treatment approaches for these rare lesions.

摘要

背景

原发性颅内胆脂瘤是一种罕见的角化鳞状上皮良性病变,常起源于中耳扩展。然而,它们在丘脑的发生极为罕见,此前仅报道过一例。

病例描述

一名48岁男性,3年来逐渐出现头痛,向上凝视困难。神经系统检查发现双侧向上凝视麻痹。磁共振成像显示右丘脑有一个轴内囊性肿块,有实性成分,延伸至中脑,导致梗阻性脑积水。进行了开颅肿瘤次全切除术。组织病理学证实为胆脂瘤。术后患者症状改善,但脑积水复发,需要进行内镜下第三脑室造瘘术。五个月后,影像学显示残余肿块大小增加,但由于经济限制,拒绝了进一步的手术干预。患者继续门诊随访,尽管症状持续,但仍保持功能独立。

结论

丘脑胆脂瘤罕见,诊断具有挑战性。其深部位置使手术切除复杂化,往往需要次全切除以保留神经功能。由于复发风险高,术后密切监测至关重要。需要进一步研究以优化诊断策略,并探索针对这些罕见病变的替代治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78cb/12134822/91b263126a7d/SNI-16-187-g001.jpg

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