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Review
. 2024 Dec 5;26(12):2159-2173.
doi: 10.1093/neuonc/noae128.

SNO-EANO-EURACAN consensus on management of pineal parenchymal tumors

Anthony P Y Liu  1   2 , Bryan K Li  3   4 , Alexandre Vasiljevic  5   6 , Michael C Dewan  7 , Benita Tamrazi  8 , Birgit Ertl-Wagner  9   10   11 , Jordan R Hansford  12 , Elke Pfaff  13   14   15 , Martin Mynarek  16   17 , Ho-Keung Ng  18 , Derek S Tsang  19 , Nicholas G Gottardo  20 , Amar Gajjar  21 , Eric Bouffet  2 , Christelle Dufour  22   23 , Barry Pizer  24 , David Schiff  25 , Michael D Jenkinson  26   27 , Giuseppe Lombardi  28 , Patrick Y Wen  29 , Martin J van den Bent  30 , Annie Huang  2   3   31   32
Affiliations
Review

SNO-EANO-EURACAN consensus on management of pineal parenchymal tumors

Anthony P Y Liu et al. Neuro Oncol. .

Abstract

Pineal parenchymal tumors are rare neoplasms for which evidence-based treatment recommendations are lacking. These tumors vary in biology, clinical characteristics, and prognosis, requiring treatment that ranges from surgical resection alone to intensive multimodal antineoplastic therapy. Recently, international collaborative studies have shed light on the genomic landscape of these tumors, leading to refinement in molecular-based disease classification in the 5th edition of the World Health Organization (WHO) classification of tumors of the central nervous system. In this review, we summarize the literature on diagnostic and therapeutic approaches, and suggest pragmatic recommendations for the clinical management of patients presenting with intrinsic pineal region masses including parenchymal tumors (pineocytoma, pineal parenchymal tumor of intermediate differentiation, and pineoblastoma), pineal cyst, and papillary tumors of the pineal region.

Keywords: clinical treatment guidelines; papillary tumor of pineal region; pineal cyst; pineal parenchymal tumors; risk-stratification.

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Conflict of interest statement

JRH declares honoraria from Bayer and Alexion Pharmaceuticals and Institutional funding from Servier International for work on their Scientific Advisory Board unrelated to this work. GL declares consulting or advisory role funding from AbbVie, Bayer, Novartis, Orbus Therapeutics, BrainFarm, Celgene, Cureteq, Health4U, Braun, Janssen, BioRegio STERN, Servier, and Novocure; and travel funding from Roche and Bayer. All other authors declare no conflict of interest.

Figures

Figure 1.
Figure 1.
Histopathologic features of pineal parenchymal tumors and papillary tumors of the pineal region (PTPRs) in relation to the normal pineal gland. Characteristic lobular architecture of the normal pineal gland with strong expression of neurofilament proteins (NFP), pinealocytes with CRX transcription factor expression and negative Ki-67 stain are shown in relation to pineal cysts—with typical 3 layer walls of piloid astrogliosis, disrupted pineal parenchyma, and thickened leptomeninges, and pineocytoma (PC) - with strong typical NFP expression in large and irregular fibrillary pseudorosettes, CRX positivity and low Ki-67 index, and pineal parenchymal tumor of intermediate differentiation (PPTID) and pineoblastoma (PB)—higher grade lesions with diminished NFP expression and respective low-intermediate and high Ki-67 indices. PTPR are distinct tumors resembling ependymoma with expression of ciliogenesis transcription factor FOX-J1 and evidence of epithelial differentiation with cytokeratin 18 (CK18) immunopositivity and no CRX expression.
Figure 2.
Figure 2.
Magnetic resonance imaging (MRI) features of pineoblastoma. Coronal T2-weighted (A) and Axial FLAIR MRI showing pineal region tumor with marked hydrocephalus and transependymal CSF seepage. Axial gradient (C) and T1-MRI with contrast (D) showing scattered susceptibility of “exploded calcification” and avid contrast enhancement in the tumor.
Figure 3.
Figure 3.
Cerebrospinal fluid diversion and biopsy in a pineoblastoma patient. Illustrative photos showing (A) visualization of the floor of the third ventricle and dorsally located PPTs with right frontal, endoscopic transforaminal approach, with (B) third ventriculostomy for hydrocephalus, followed by posteriorly directed scope toward PPT for (D) endoscopic diagnostic sampling of tumor tissue.
Figure 4.
Figure 4.
Demographic, clinical, and genomic features of pineoblastoma subtypes and pineal parenchymal tumors of intermediate differentiation (used with permission from Springer Nature).
See this image and copyright information in PMC

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