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Diagnosis and Treatment of Pineal Region Tumors in Adults: A EURACAN Overview.

作者信息

Lombardi Giuseppe, Poliani Pietro Luigi, Manara Renzo, Berhouma Moncef, Minniti Giuseppe, Tabouret Emeline, Razis Evangelia, Cerretti Giulia, Zagonel Vittorina, Weller Michael, Idbaih Ahmed

机构信息

Department of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Via Gattamelata 64, 35128 Padua, Italy.

Pathology Unit, Department of Molecular and Translational Medicine, University of Brescia, P.le Spedali Civili 1, 25121 Brescia, Italy.

出版信息

Cancers (Basel). 2022 Jul 27;14(15):3646. doi: 10.3390/cancers14153646.


DOI:10.3390/cancers14153646
PMID:35954310
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9367474/
Abstract

Pineal region tumors are rare intracranial tumors, accounting for less than 1% of all adult intracranial tumor lesions. These lesions represent a histologically heterogeneous group of tumors. Among these tumors, pineal parenchymal tumors and germ cell tumors (GCT) represent the most frequent types of lesions. According to the new WHO 2021 classification, pineal parenchymal tumors include five distinct histotypes: pineocytoma (PC), pineal parenchymal tumors of intermediate differentiation (PPTID), papillary tumor of the pineal region (PTPR), pinealoblastoma (PB), and desmoplastic myxoid tumor of the pineal region, SMARCB1-mutant; GCTs include germinoma, embryonal carcinoma, yolk sac tumor, choriocarcinoma, teratoma, mixed GCTs. Neuroradiological assessment has a pivotal role in the diagnostic work-up, surgical planning, and follow-up of patients with pineal masses. Surgery can represent the mainstay of treatment, ranging from biopsy to gross total resection, yet pineal region tumors associated with obstructive hydrocephalus may be surgically managed via ventricular internal shunt or endoscopic third ventriculostomy. Radiotherapy remains an essential component of the multidisciplinary treatment approach for most pineal region tumors; however, treatment volumes depend on the histological subtypes, grading, extent of disease, and the combination with chemotherapy. For localized germinoma, the current standard of care is chemotherapy followed by reduced-dose whole ventricular irradiation plus a boost to the primary tumor. For pinealoblastoma patients, postoperative radiation has been associated with higher overall survival. For the other pineal tumors, the role of radiotherapy remains poorly studied and it is usually reserved for aggressive (grade 3) or recurrent tumors. The use of systemic treatments mainly depends on histology and prognostic factors such as residual disease and metastases. For pinealoblastoma patients, chemotherapy protocols are based on various alkylating or platinum-based agents, vincristine, etoposide, cyclophosphamide and are used in association with radiotherapy. About GCTs, their chemosensitivity is well known and is based on cisplatin or carboplatin and may include etoposide, cyclophosphamide, or ifosfamide prior to irradiation. Similar regimens containing platinum derivatives are also used for non-germinomatous GCTs with very encouraging results. However, due to a greater understanding of the biology of the disease's various molecular subtypes, new agents based on targeted therapy are expected in the future. On behalf of the EURACAN domain 10 group, we reviewed the most important and recent developments in histopathological characteristics, neuro-radiological assessments, and treatments for pineal region tumors.

摘要
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/720a/9367474/aaa6f202bae0/cancers-14-03646-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/720a/9367474/73f45e018947/cancers-14-03646-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/720a/9367474/aaa6f202bae0/cancers-14-03646-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/720a/9367474/73f45e018947/cancers-14-03646-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/720a/9367474/aaa6f202bae0/cancers-14-03646-g002.jpg

相似文献

[1]
Diagnosis and Treatment of Pineal Region Tumors in Adults: A EURACAN Overview.

Cancers (Basel). 2022-7-27

[2]
Histopathology of tumors of the pineal region.

Future Oncol. 2010-5

[3]
Pineal/germ cell tumors and pineal parenchymal tumors.

Childs Nerv Syst. 2023-10

[4]
Brain and Spinal Tumors Originating from the Germ Line Cells.

Adv Exp Med Biol. 2023

[5]
Pathology of pineal region tumors.

J Neurooncol. 2001-9

[6]
Consensus guidelines for the management of pineal region tumours for low- and middle-income countries.

J Pak Med Assoc. 2024-3

[7]
The Third Eye Sees Double: Cohort Study of Clinical Presentation, Histology, Surgical Approaches, and Ophthalmic Outcomes in Pineal Region Germ Cell Tumors.

World Neurosurg. 2021-6

[8]
Survival and Malignant Transformation of Pineal Parenchymal Tumors: A 30-Year Retrospective Analysis in a Single-Institution.

Brain Tumor Res Treat. 2023-10

[9]
[Endoscopic treatment strategy of obstructive hydrocephalus induced by pineal region tumors].

Zhonghua Yi Xue Za Zhi. 2015-3-24

[10]
Simultaneous single-trajectory endoscopic biopsy and third ventriculostomy in pediatric pineal region tumors.

Acta Neurol Belg. 2021-12

引用本文的文献

[1]
Machine learning decision support model construction for craniotomy approach of pineal region tumors based on MRI images.

BMC Med Imaging. 2025-5-27

[2]
A rare case of pineal astrocytoma with hemorrhagic changes: Diagnostic and surgical challenges.

Radiol Case Rep. 2025-4-10

[3]
Recurrent pineal tumor in a young adult male: Challenges in diagnosis and multimodal treatment management.

Radiol Case Rep. 2025-3-27

[4]
Papillary Tumor of the Pineal Region Identified by DNA Methylation Leads to the Incidental Finding of Germline Mutation Associated with PTEN Hamartoma Tumor Syndrome: A Case Report and Systematic Review.

Curr Oncol. 2025-3-17

[5]
Papillary Tumor of the Pineal Region Treated With Surgery and Postoperative Radiotherapy: A Case Report.

Cureus. 2025-1-25

[6]
Pineal region tumors: prognostic stratification based on magnetic resonance imaging features-a retrospective cohort study.

Quant Imaging Med Surg. 2025-1-2

[7]
Desmoplastic myxoid tumor of the pineal region, SMARCB1 mutant: illustrative case.

J Neurosurg Case Lessons. 2024-11-25

[8]
Purines and purinergic receptors in primary tumors of the central nervous system.

Purinergic Signal. 2024-10-1

[9]
SNO-EANO-EURACAN consensus on management of pineal parenchymal tumors.

Neuro Oncol. 2024-12-5

[10]
Papillary Tumor of the Pineal Gland: Series of Four Clinical Cases.

Cureus. 2024-5-29

本文引用的文献

[1]
Long term outcomes following surgery for pineal region tumors.

J Neurooncol. 2022-2

[2]
Metastases in the Pineal Region: A Systematic Review of Clinical Features, Management Strategies, and Survival Outcomes.

World Neurosurg. 2022-3

[3]
Incidence, Prognostic Factors, and Survival Trend in Pineal Gland Tumors: A Population-Based Analysis.

Front Oncol. 2021-11-19

[4]
Surgical treatment of symptomatic pineal cysts without hydrocephalus-meta-analysis of the published literature.

Acta Neurochir (Wien). 2022-1

[5]
Phase II trial of response-based radiation therapy for patients with localized germinoma: a Children's Oncology Group study.

Neuro Oncol. 2022-6-1

[6]
In and around the pineal gland: a neuroimaging review.

Clin Radiol. 2022-2

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Prognosticating survival of pineal parenchymal tumors of intermediate differentiation (PPTID) by grade.

J Neurooncol. 2021-11

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The 2021 WHO Classification of Tumors of the Central Nervous System: a summary.

Neuro Oncol. 2021-8-2

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Pineal Gland Tumors: A Review.

Cancers (Basel). 2021-3-27

[10]
Paediatric atypical teratoid/rhabdoid tumour of the pineal region mimicking a meningioma: a case report and literature review.

Br J Neurosurg. 2023-12

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