Rosette-Forming Glioneuronal Tumor in the Pineal Region: A Series of 6 Cases and Literature Review

Author:

Lin Chun-Chieh12ORCID,Mansukhani Mahesh M1,Bruce Jeffrey N3ORCID,Canoll Peter1ORCID,Zanazzi George24

Affiliation:

1. From the Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USA

2. Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire, USA

3. Department of Neurosurgery, Columbia University Irving Medical Center, New York, New York, USA

4. Norris Cotton Cancer Center, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire, USA

Abstract

Abstract Resected lesions from the pineal region are rare specimens encountered by surgical pathologists, and their heterogeneity can pose significant diagnostic challenges. Here, we reviewed 221 pineal region lesions resected at New York-Presbyterian Hospital/Columbia University Irving Medical Center from 1994 to 2019 and found the most common entities to be pineal parenchymal tumors (25.3%), glial neoplasms (18.6%), and germ cell tumors (17.6%) in this predominantly adult cohort of patients. Six cases of a rare midline entity usually found exclusively in the fourth ventricle, the rosette-forming glioneuronal tumor, were identified. These tumors exhibit biphasic morphology, with a component resembling pilocytic astrocytoma admixed with variable numbers of small cells forming compact rosettes and perivascular pseudorosettes. Targeted sequencing revealed a 100% co-occurrence of novel and previously described genetic alterations in the mitogen-activated protein kinase (MAPK) and phosphoinositide 3-kinase (PI3K) signaling pathways, suggesting a synergistic role in tumor formation. The most common recurrent mutation, PIK3CA H1047R, was identified in tumor cells forming rosettes and perivascular pseudorosettes. A review of the literature revealed 16 additional cases of rosette-forming glioneuronal tumors in the pineal region. Although rare, this distinctive low-grade tumor warrants consideration in the differential diagnosis of pineal region lesions.

Funder

Shelanski Award

Publisher

Oxford University Press (OUP)

Subject

Cellular and Molecular Neuroscience,Neurology (clinical),Neurology,General Medicine,Pathology and Forensic Medicine

Reference36 articles.

1. Incidence of pineal tumours. A review of the literature;Mottolese;Neurochirurgie,2015

2. Pathology of tumors of the pineal region;De Girolami;Rev Neurol (Paris),2008

3. A rosette-forming glioneuronal tumor of the fourth ventricle: Infratentorial form of dysembryoplastic neuroepithelial tumor?;Komori;Am J Surg Pathol,2002

4. Rosette-forming glioneuronal tumor: An illustrative case and a systematic review;Wilson;Neurooncol Adv,2020

5. FGFR1 mutations in Rosette-forming glioneuronal tumors of the fourth ventricle;Gessi;J Neuropathol Exp Neurol,2014

Cited by 7 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3