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理解与管理中间分化型松果体实质肿瘤:从病理学到辅助治疗的深入探索

Understanding and Managing Pineal Parenchymal Tumors of Intermediate Differentiation: An In-Depth Exploration from Pathology to Adjuvant Therapies.

作者信息

Bianconi Andrea, Panico Flavio, Lo Zito Bruna, Do Trinh Andrea, Cassoni Paola, Ricardi Umberto, Garbossa Diego, Cofano Fabio, Mantovani Cristina, Bertero Luca

机构信息

Neurosurgery Unit, Department of Neuroscience, University of Turin, 10126 Turin, Italy.

Radiation Oncology Unit, Department of Oncology, University of Turin, 10126 Turin, Italy.

出版信息

J Clin Med. 2024 Feb 23;13(5):1266. doi: 10.3390/jcm13051266.

Abstract

BACKGROUND

Pineal parenchymal cell tumors constitute a rare group of primary central nervous system neoplasms (less than 1%). Their classification, especially the intermediate subtype (PPTIDs), remains challenging.

METHODS

A literature review was conducted, navigating through anatomo-pathological, radiotherapy, and neurosurgical dimensions, aiming for a holistic understanding of these tumors.

RESULTS

PPTIDs, occupying an intermediate spectrum of malignancy, reveal diverse histological patterns, mitotic activity, and distinct methylation profiles. Surgical treatment is the gold standard, but when limited to partial removal, radiotherapy becomes crucial. While surgical approaches are standardized, due to the low prevalence of the pathology and absence of randomized prospective studies, there are no shared guidelines about radiation treatment modalities.

CONCLUSION

Surgical removal remains pivotal, demanding a personalized approach based on the tumor extension. This review underscores the considerable variability in treatment approaches and reported survival rates within the existing literature, emphasizing the need for ongoing research to better define optimal therapeutic strategies and prognostic factors for PPTIDs, aiming for further and more detailed stratification among them.

摘要

背景

松果体实质细胞肿瘤是一组罕见的原发性中枢神经系统肿瘤(不到1%)。它们的分类,尤其是中间型亚型(PPTIDs),仍然具有挑战性。

方法

进行了一项文献综述,从解剖病理学、放射治疗和神经外科等方面进行研究,旨在全面了解这些肿瘤。

结果

PPTIDs处于恶性程度的中间范围,呈现出多样的组织学模式、有丝分裂活性和独特的甲基化谱。手术治疗是金标准,但当只能部分切除时,放射治疗就变得至关重要。虽然手术方法是标准化的,但由于该病理学的发病率低且缺乏随机前瞻性研究,关于放射治疗方式没有共同的指南。

结论

手术切除仍然至关重要,需要根据肿瘤的扩展情况采取个性化方法。这篇综述强调了现有文献中治疗方法和报告的生存率存在相当大的差异,强调需要持续研究以更好地确定PPTIDs的最佳治疗策略和预后因素,以期在它们之间进行更深入、更详细的分层。

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