An aggressive, unresected pineoblastoma in an adult woman: the role of exclusive radiotherapy - a case report and literature review.

IF 1.2 Q4 ONCOLOGY
ecancermedicalscience Pub Date : 2025-05-15 eCollection Date: 2025-01-01 DOI:10.3332/ecancer.2025.1909
Salem Ouaddane Alami, Fatima Zahra Abdelli, Samia Khalfi, Fatima Zahra Farhane, Zineb Alami, Touria Bouhafa
{"title":"An aggressive, unresected pineoblastoma in an adult woman: the role of exclusive radiotherapy - a case report and literature review.","authors":"Salem Ouaddane Alami, Fatima Zahra Abdelli, Samia Khalfi, Fatima Zahra Farhane, Zineb Alami, Touria Bouhafa","doi":"10.3332/ecancer.2025.1909","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>The pineal gland is a small, endocrine structure located in the posterior cranial fossa, playing a critical role in regulating sleep-wake cycles. Pineal parenchymal tumours (PPTs) are rare, accounting for less than 1% of central nervous system malignancies. These tumours include pineocytomas (benign), intermediate-grade tumours (PPTs) and aggressive pineoblastomas (PBL), which represent the most dangerous subtype. PBL are fast-growing, high-grade tumours often classified as grade IV. This paper presents a rare case of unresected PBL in a young woman, leading to bilateral blindness and the role of exclusive radiotherapy (RT) as a treatment modality.</p><p><strong>Objective: </strong>This case report aims to explore the effectiveness of exclusive RT in treating an unresected PBL in a young patient, emphasising the tumour's aggressiveness and the therapeutic challenges it presents.</p><p><strong>Case presentation: </strong>A 35-year-old woman with no significant medical history presented with progressively worsening headaches and visual acuity loss. Imaging revealed a pineal region tumour, confirmed as PBL through stereotactic biopsy. The patient had bilateral papilledema and developed hydrocephalus due to tumour growth, leading to pressure on surrounding structures. Due to the patient's refusal of chemotherapy (CT), RT was chosen as the sole treatment option.RT was delivered in two phases: a craniospinal dose of 36 Gy and a boost of 18 Gy to the tumour. The patient tolerated the treatment well, with only mild nausea and fatigue. Follow-up imaging at 3 months showed a 38% reduction in tumour size and improvement in hydrocephalus, although the patient remained bilaterally blind. At 24 months post-treatment, the tumour remained stable, suggesting a positive long-term outcome with exclusive RT.</p><p><strong>Discussion: </strong>PBL are aggressive tumours with a high potential for leptomeningeal dissemination. Surgery, when feasible, is considered the primary treatment for pediatric cases, but in adults, data are sparse. In this case, the patient's refusal of CT made RT the only viable option. Recent studies suggest that RT alone can improve survival, particularly when combined with craniospinal irradiation and tumour boosts. However, the role of surgery and CT in adult PBL remains debated, with mixed results in the literature regarding their impact on overall survival.While some studies indicate no significant survival benefit from extensive tumour resection, others suggest that total resection can improve prognosis. The case highlights that exclusive RT can be a life-saving treatment for patients who cannot undergo surgery or CT, despite the tumour's aggressive nature. Further research is needed to establish standardised protocols for managing adult PBL.</p><p><strong>Conclusion: </strong>This case demonstrates the potential of exclusive RT as an effective treatment for PBL in patients who cannot undergo surgery. Despite the patient's permanent blindness, RT led to a significant reduction in tumour size and stabilisation of the disease. More research is necessary to define the most effective treatment strategies for PBL, particularly in adults, where surgical options may be limited.</p>","PeriodicalId":11460,"journal":{"name":"ecancermedicalscience","volume":"19 ","pages":"1909"},"PeriodicalIF":1.2000,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12185867/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"ecancermedicalscience","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.3332/ecancer.2025.1909","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/1/1 0:00:00","PubModel":"eCollection","JCR":"Q4","JCRName":"ONCOLOGY","Score":null,"Total":0}
引用次数: 0

Abstract

Introduction: The pineal gland is a small, endocrine structure located in the posterior cranial fossa, playing a critical role in regulating sleep-wake cycles. Pineal parenchymal tumours (PPTs) are rare, accounting for less than 1% of central nervous system malignancies. These tumours include pineocytomas (benign), intermediate-grade tumours (PPTs) and aggressive pineoblastomas (PBL), which represent the most dangerous subtype. PBL are fast-growing, high-grade tumours often classified as grade IV. This paper presents a rare case of unresected PBL in a young woman, leading to bilateral blindness and the role of exclusive radiotherapy (RT) as a treatment modality.

Objective: This case report aims to explore the effectiveness of exclusive RT in treating an unresected PBL in a young patient, emphasising the tumour's aggressiveness and the therapeutic challenges it presents.

Case presentation: A 35-year-old woman with no significant medical history presented with progressively worsening headaches and visual acuity loss. Imaging revealed a pineal region tumour, confirmed as PBL through stereotactic biopsy. The patient had bilateral papilledema and developed hydrocephalus due to tumour growth, leading to pressure on surrounding structures. Due to the patient's refusal of chemotherapy (CT), RT was chosen as the sole treatment option.RT was delivered in two phases: a craniospinal dose of 36 Gy and a boost of 18 Gy to the tumour. The patient tolerated the treatment well, with only mild nausea and fatigue. Follow-up imaging at 3 months showed a 38% reduction in tumour size and improvement in hydrocephalus, although the patient remained bilaterally blind. At 24 months post-treatment, the tumour remained stable, suggesting a positive long-term outcome with exclusive RT.

Discussion: PBL are aggressive tumours with a high potential for leptomeningeal dissemination. Surgery, when feasible, is considered the primary treatment for pediatric cases, but in adults, data are sparse. In this case, the patient's refusal of CT made RT the only viable option. Recent studies suggest that RT alone can improve survival, particularly when combined with craniospinal irradiation and tumour boosts. However, the role of surgery and CT in adult PBL remains debated, with mixed results in the literature regarding their impact on overall survival.While some studies indicate no significant survival benefit from extensive tumour resection, others suggest that total resection can improve prognosis. The case highlights that exclusive RT can be a life-saving treatment for patients who cannot undergo surgery or CT, despite the tumour's aggressive nature. Further research is needed to establish standardised protocols for managing adult PBL.

Conclusion: This case demonstrates the potential of exclusive RT as an effective treatment for PBL in patients who cannot undergo surgery. Despite the patient's permanent blindness, RT led to a significant reduction in tumour size and stabilisation of the disease. More research is necessary to define the most effective treatment strategies for PBL, particularly in adults, where surgical options may be limited.

成年女性一例侵袭性未切除的松果体母细胞瘤:单纯放疗的作用- 1例报告及文献回顾。
松果体是一个小的内分泌结构,位于颅后窝,在调节睡眠-觉醒周期中起关键作用。松果体实质肿瘤(PPTs)是罕见的,占不到1%的中枢神经系统恶性肿瘤。这些肿瘤包括松果体细胞瘤(良性)、中度肿瘤(PPTs)和侵袭性松果体母细胞瘤(PBL),这是最危险的亚型。PBL是一种生长迅速的高级别肿瘤,通常被分类为IV级。本文报道了一名年轻女性未切除PBL的罕见病例,导致双侧失明,并采用排他放疗(RT)作为治疗方式。目的:本病例报告旨在探讨独家RT治疗年轻患者未切除PBL的有效性,强调肿瘤的侵袭性及其带来的治疗挑战。病例介绍:一名35岁女性,无明显病史,表现为头痛逐渐加重,视力下降。影像显示一松果体区肿瘤,立体定向活检证实为PBL。患者双侧乳头水肿,肿瘤生长导致脑积水,导致周围结构受压。由于患者拒绝化疗(CT),因此选择RT作为唯一的治疗方案。RT分为两个阶段:36 Gy的颅脊髓剂量和18 Gy的肿瘤增强剂量。病人对治疗耐受良好,只有轻微的恶心和疲劳。3个月的随访成像显示肿瘤大小缩小38%,脑积水改善,尽管患者仍保持双侧失明。在治疗后24个月,肿瘤保持稳定,表明排他性rt的长期结果是积极的。讨论:PBL是侵袭性肿瘤,具有脑膜轻散的高潜力。手术,在可行的情况下,被认为是儿科病例的主要治疗方法,但在成人中,数据很少。在这种情况下,患者拒绝CT使得RT成为唯一可行的选择。最近的研究表明,单独放疗可以提高生存率,特别是当与颅脊髓照射和肿瘤刺激相结合时。然而,手术和CT在成人PBL中的作用仍然存在争议,文献中关于它们对总生存率的影响的结果不一。虽然一些研究表明广泛切除肿瘤没有显著的生存益处,但其他研究表明完全切除可以改善预后。该病例强调,尽管肿瘤具有侵袭性,但对于无法接受手术或CT的患者来说,独家RT可能是一种挽救生命的治疗方法。需要进一步的研究来建立管理成人PBL的标准化方案。结论:该病例证明了排他性放射治疗对于不能接受手术的PBL患者是一种有效的治疗方法。尽管患者的永久性失明,RT导致肿瘤大小的显著减少和疾病的稳定。需要更多的研究来确定PBL最有效的治疗策略,特别是在成人中,手术选择可能有限。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 求助全文
来源期刊
CiteScore
3.80
自引率
5.60%
发文量
138
审稿时长
27 weeks
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术官方微信