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一名患有造釉细胞瘤型颅咽管瘤的年轻成年人发生瘤内出血的罕见病例。

A rare case of intratumoral hemorrhage in a young adult with adamantinomatous craniopharyngioma.

作者信息

Agustina Ana, Ruhimat Undang, Mahmudah Raisa, Sutiono Agung Budi, Primastari Etis

机构信息

Department of Radiology, Faculty of Medicine, University of Padjadjaran, Dr. Hasan Sadikin General Hospital, Jl. Pasteur No. 38, Pasteur, Sukajadi, Bandung City, West Java, 40161, Indonesia.

Department of Neurosurgery, Faculty of Medicine, University of Padjadjaran, Dr. Hasan Sadikin General Hospital, Jl. Pasteur No. 38, Pasteur, Sukajadi, Bandung City, West Java, 40161, Indonesia.

出版信息

Radiol Case Rep. 2024 Nov 14;20(1):761-766. doi: 10.1016/j.radcr.2024.10.044. eCollection 2025 Jan.

Abstract

Craniopharyngiomas are rare, slow growing tumors arising along the craniopharyngeal duct. The incidence of craniopharyngioma was 0.13 per 100,000 persons per year. Intratumoral hemorrhage is a serious complication that can mimic pituitary tumor apoplexy, a life-threatening condition. In this report, We presented a case of a 20-year-old male with 3 days of decreased consciousness, with a history of worsening headaches, vomiting, blurry vision, bitemporal hemianopia, and right-sided limb weakness. MRI findings revealed a mixed cystic and solid suprasellar mass with blooming artifacts and fluid-fluid levels on SWI strongly suggest craniopharyngiomas with intratumoral hemorrhage. Trans-petrosal surgery was performed, the lesion appeared intraoperatively as a firm, elastic mass with a hemorrhagic component. Further histopathological testing confirmed the diagnosis of craniopharyngioma with adamantinomatous subtype, which typically occurs in children and adults over 45. While in this patient's diagnosis at age 20 falls outside the usual range. This study highlights the possibility of adamantinomatous craniopharyngioma occurring in young adulthood and the role of imaging in diagnosing craniopharyngioma, including its detailed characteristics and the presence of intratumoral hemorrhage for early management and better patient outcome.

摘要

颅咽管瘤是沿颅咽管发生的罕见、生长缓慢的肿瘤。颅咽管瘤的发病率为每年每10万人中有0.13例。瘤内出血是一种严重并发症,可酷似垂体瘤卒中,这是一种危及生命的情况。在本报告中,我们介绍了一例20岁男性患者,其意识下降3天,有头痛加重、呕吐、视力模糊、双颞侧偏盲和右侧肢体无力的病史。MRI检查结果显示鞍上有一个混合性囊性和实性肿块,磁敏感加权成像(SWI)上有磁敏感伪影和液-液平面,强烈提示为伴有瘤内出血的颅咽管瘤。进行了经岩骨手术,术中病变表现为一个质地硬、有弹性且有出血成分的肿块。进一步的组织病理学检查确诊为釉质型颅咽管瘤,这种类型通常发生在儿童和45岁以上的成年人中。而该患者20岁确诊,不在常见范围内。本研究强调了年轻成年人发生釉质型颅咽管瘤的可能性以及影像学在诊断颅咽管瘤中的作用,包括其详细特征和瘤内出血情况,以便早期管理并改善患者预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/271e/11609111/aa5d154d5e66/gr1.jpg

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