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老年罕见的小脑蚓部毛细胞型星形细胞瘤伴复发性自发性出血:一例报告并文献复习

Rare vermian pilocytic astrocytoma with recurrent spontaneous hemorrhage in the elderly: A case report and review of literature.

作者信息

Francis Campbell Chukwuebuka, Kanaya Kohei, Nagamine Kohei, Goto Tetsuya, Horiuchi Tetsuyoshi, Ohaegbulam Samuel Chukwunonyerem

机构信息

Department of Neurosurgery, Memfys Hospital, Transekulu, Enugu, Nigeria.

Department of Neurosurgery, Shinshu University Hospital, Matsumoto, Japan.

出版信息

Surg Neurol Int. 2024 Mar 15;15:90. doi: 10.25259/SNI_978_2023. eCollection 2024.

DOI:10.25259/SNI_978_2023
PMID:38628526
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11021065/
Abstract

BACKGROUND

Pilocytic astrocytoma (PA) is a benign glial tumor predominately seen in pediatrics and early adolescence with associated overall good outcomes. Very few cases of elderly PA have been reported in the literature, and they are known to display unique anatomic, histologic, and genetic peculiarities distinct from pediatric disease. We report a rare case of vermian PA in an octogenarian with recurrent spontaneous intratumoral hemorrhage as a presenting symptom. Furthermore, a review of the literature on the peculiarities of PA in the elderly will be discussed.

CASE DESCRIPTION

An 81-year-old woman presented with features suggestive of repeated posterior fossa hemorrhages characterized by headaches, diplopia, and alteration in sensorium occurring about 5 months apart. Brain neuroimaging showed a cerebellar vermian tumor with features suggestive of repeated intratumoral bleeding. She had an initial ventriculoperitoneal shunting for acute hydrocephalus and subsequently had a suboccipital craniotomy and subtotal tumor excision due to morbid adherence to the brainstem. The histologic diagnosis was PA with Ki-67 <1% and negative for isocitrate dehydrogenase-1. There was a slow but progressive clinical improvement, and she has remained symptom-free for 4 years on follow-up.

CONCLUSION

PA in the elderly is a rare disease with distinct histologic and genetic peculiarities. This case review showed one of the oldest cases of cerebellar vermian PA presenting with recurrent spontaneous intratumoral hemorrhage, an extremely rare occurrence in benign glioma. Although complete surgical excision is recommended, partial resection is advocated for morbidly adherent tumors. Overall prognosis is worse in elderly PA.

摘要

背景

毛细胞型星形细胞瘤(PA)是一种良性神经胶质瘤,主要见于儿童和青少年早期,总体预后良好。文献中报道的老年PA病例极少,已知其在解剖学、组织学和遗传学方面具有与儿科疾病不同的独特特征。我们报告一例罕见的老年小脑蚓部PA病例,以反复自发性肿瘤内出血为首发症状。此外,还将讨论有关老年PA特点的文献综述。

病例描述

一名81岁女性,表现出提示后颅窝反复出血的特征,症状包括头痛、复视和意识改变,发作间隔约5个月。脑部神经影像学检查显示小脑蚓部有一肿瘤,具有提示反复肿瘤内出血的特征。她最初因急性脑积水接受了脑室腹腔分流术,随后因肿瘤与脑干紧密粘连,接受了枕下开颅手术及肿瘤次全切除术。组织学诊断为PA,Ki-67<1%,异柠檬酸脱氢酶-1阴性。患者临床症状缓慢但持续改善,随访4年无症状。

结论

老年PA是一种罕见疾病,具有独特的组织学和遗传学特征。本病例回顾展示了最年长的小脑蚓部PA病例之一,以反复自发性肿瘤内出血为表现,这在良性胶质瘤中极为罕见。尽管建议完整手术切除,但对于紧密粘连的肿瘤主张部分切除。老年PA的总体预后较差。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b58c/11021065/bc34da38143e/SNI-15-90-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b58c/11021065/14fc53c1242e/SNI-15-90-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b58c/11021065/4fb18b569f39/SNI-15-90-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b58c/11021065/bc34da38143e/SNI-15-90-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b58c/11021065/14fc53c1242e/SNI-15-90-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b58c/11021065/4fb18b569f39/SNI-15-90-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b58c/11021065/bc34da38143e/SNI-15-90-g003.jpg

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本文引用的文献

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IBRO Neurosci Rep. 2022 Oct 7;13:388-392. doi: 10.1016/j.ibneur.2022.10.001. eCollection 2022 Dec.
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毛细胞型星形细胞瘤:一般特征、临床特征和分子特征的综述。
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Rapid Recurrence and Anaplastic Transformation of a Pilocytic Astrocytoma in an Elderly Patient: Case Report and Review of the Literature.老年患者毛细胞星形细胞瘤的快速复发和间变:病例报告及文献复习。
World Neurosurg. 2020 Oct;142:441-449. doi: 10.1016/j.wneu.2020.06.173. Epub 2020 Jul 4.
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Superficial Siderosis Associated with Long-Term Recurrence of Pilocytic Astrocytoma in an Elderly Person.老年人伴长期复发的毛细胞星形细胞瘤相关的脑表铁沉积症。
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