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Background: Pineal cysts are a rare lesion of the pineal gland. Pineal cysts are benign lesions, generally asymptomatic, and are usually an incidental discovery on MRI performed for other problems. The management of pineal cysts in children remains a matter for debate. Here, we report our own retrospective paediatric cases that have been surgically treated and review the paediatric literature on this topic., Methods: This is a retrospective monocentric study. All patients operated by the senior author (CM) for a benign pineal cyst from 2000 to 2021 were included. All other pineal region cystic lesions were excluded. Medical and surgical data were extracted from the hospital medical database., Results: Twelve patients were included. The clinical symptomatology was characterized by headaches in seven patients, visual troubles in two patients, precocious puberty in one patient, signs of intracranial hypertension in two patients, seizures associated with headache in one patient, and headaches associated with behavioural troubles in another patient. No major post-operative complications were observed in this series. It is to noted that surgery was performed because a suspicion of a true pineal parenchymal tumour has been made. Histopathological study came back with the diagnosis of pineal cyst., Conclusions: Pineal cyst is rare. If the radiological diagnosis is clear, no surgery is advocated except in cases associated with hydrocephalus and rapid growth. In case of a suspicion of a true pineal parenchymal tumour, a surgery may be needed to confirm the diagnosis. Lastly, we stress that only cystic lesions of the pineal gland itself should be considered as pineal cyst., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Two major approaches exist for the surgical removal of pineal region tumors: the supracebellar infratentorial and the sub-occipital transtentorial., Methods: We present the Lyon's technique of the sub-occipital transtentorial approach for pineal region tumors and our tricks to avoid complications. The principle is to expose the pineal region under the occipital lobe and not through the interhemispheric fissure., Conclusions: The sub-occipital transtentorial approach is a direct, extra cerebral, safe, and effective way to access tumors of the pineal region., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Introduction: Pineal tumors are rare and their incidence is of 1% among all pediatric tumors of the central nervous system. Patient survival depends on the histology, the extension of the surgical removal, and the efficacy of the complementary treatment (chemotherapy and cranio-spinal irradiation), as well as the age of the patient., Materials and Methods: In this study, we analyzed 151 pediatric patients treated for pineal tumors from 1997 to 2020 in Lyon, France. All patients were recorded in the French Register of Pineal tumors, which has been centralized and maintained in Lyon since 2010., Results: Our analysis shows that benign tumors have an overall positive prognosis with total surgical removal. Concerning pineal parenchymal tumors, pinealoblastomas have a poor prognosis , especially in children less than three years old. A new pathological classification system allows for a better stratification of patient risk within different groups of patients with pineal tumors. It is also important to note that the identification of DICER 1 syndrome in families with pinealoblastomas warrant further medical investigation. Patients with Germ Cell Tumors have more favorable outcomes, with a global survival rate of 87 % and a pure germinoma survival rate of almost 97%. When analyzing the prognosis of pineal gland gliomas, otherwise known as tectal plate gliomas, pilocytic astrocytomas had a promising prognosis. Otherwise, prognosis of other tectal plate gliomas are related to the grade of malignancy and the efficacy of complementary treatment. Lastly, papillary tumors need a complete removal for the best chance of survival, and Atypical teratoid/ rabdoid tumors (AT/TR) still have a bad prognosis, regardless of surgical resection., Conclusions: Our results show that, with regards to pediatric pineal region tumors, there are still areas in prognostic indicators that need to be improved. Similarly, these pathologies need to be treated via a multidisciplinary approach to improve a patient's survival rate and their quality of life., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Pineal tumors are rare and include a large variety of entities. Germ cell tumors are relatively frequent and often secreting lesions. Pineal parenchymal tumors include pineocytomas, pineal parenchymal tumor of intermediate differentiation, pineoblastomas and papillary tumors of the pineal region. Other lesions including astrocytomas and meningiomas as well as congenital malformations i.e. benign cysts, lipomas, epidermoid and dermoid cysts, which can also arise from the pineal region. Imagery is often non-specific but detailed analysis of the images compared with the hormone profile can narrow the spectrum of possible diagnosis., (Copyright © 2015. Published by Elsevier Masson SAS.)

Endoscopy has entered into the armamentarium of pineal and pineal region tumor treatment. The technique permits not only to control hydrocephalus but also to obtain tissue samples for histological diagnosis. In this paper, we explain the utility of endoscopy for the treatment of pineal tumors and as well as report some personal considerations regarding this topic., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

The pineal gland has interested humans from millenniums. In this paper we review back in the history and the evolution of the pineal gland surgery. Originally, this surgery used to carry a high rate of morbidity and mortality. Nowadays the development of the anesthetic, radiological, surgical and intensive care techniques have been responsible of an improvement of the surgical results and better quality of life. It is always interesting to know from where we come., (Copyright © 2014. Published by Elsevier Masson SAS.)

Background and Purpose: Circumventricular organs (CVOs) are a diverse group of specialised structures characterized by peculiar vascular and position around the third and fourth ventricles of the brain. In humans, these organs are present during the fetal period and some become vestigial after birth. Some, such as the pineal gland (PG), subcommissural organ (SCO) and organum vasculosum of the lamina terminalis (OVLT), which are located around the third ventricle, might be the site of origin of periventricular tumours. In contrast to humans, CVOs are present in the adult rat and can be dissected by laser capture microdissection (LCM)., Methods: In this study, we used LCM and microarrays to analyse the transcriptomes of three CVOs, the SCO, the subfornical organ (SFO) and the PG and the third ventricle ependyma of the adult rat, in order to better characterise these organs at the molecular level. Furthermore, an immunohistochemical study of Claudin-3 (CLDN3), a membrane protein involved in forming cellular tight junctions, was performed at the level of the SCO., Results: This study highlighted some potentially new or already described specific markers of these structures as Erbb2 and Col11a1 in ependyma, Epcam and CLDN3 in the SCO, Ren1 and Slc22a3 in the SFO and Tph, Anat and Asmt in the PG. Moreover, we found that CLDN3 expression was restricted to the apical pole of ependymocytes in the SCO., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Background: The cerebellum is a fundamental structure of the central nervous system. However, in humans, its anatomo-functional organization and the processes through which this organization adapts in response to injuries remain widely unknown., Methods: Motor and somatosensory evoked potentials were used to map functional representations in the posterior cerebellum of patients with extra- and intracellebellar injuries. Extracerebellar patients had injuries outside the cerebellum (e.g. pineal region, quadrigeminal plate) while intracerebellar patients had injuries within the cerebellum. Data were collected in 20 extracerebellar patients for motor representations. Only preliminary data were gathered for somatosensory representations and intracerebellar patients., Results: In extracerebellar patients, electrical stimulation induced muscle contractions in the ipsilateral hemibody. These representations were somatotopically organized with large overlaps between the face and upper limb in the superior posterior cerebellum and the upper and lower limb in the inferior posterior cerebellum. Neck muscles were represented in the oculomotor vermis. In intracerebellar patients, preliminary data seem to indicate that motor plasticity is achieved by recruiting the contralesional (healthy) cerebellar hemisphere., Conclusions: Although still ongoing, this project could eventually lead to an improvement of the surgical treatment of patients with lesions of the posterior fossa, by improving our knowledge of cerebellar organization and the process of post-lesional plasticity., (Copyright © 2014. Published by Elsevier Masson SAS.)

The experience of the French National Register of pineal tumours and the experience of the Lyon School are reported. Data were collected from 26 French neurosurgical centres from 1989 in Lyons and in other centres from 1997. For radiological and pathological studies, 517 cases were considered while only 452 with sufficient clinical and follow-up data were retained for further analysis. These data highlight the importance of the initial diagnosis for a therapeutic strategy that can be completely different in cases of pineal or germ cell tumours. As regards the latter, chemotherapy and radiotherapy can avoid surgery. Pineocytomas are benign lesions and their complete removal guarantees the cure. Pineal parenchymal tumours with intermediate differentiation (PTT-ID) require complete removal and complementary treatment in cases of histological abnormalities with approximately 66% of patients who remain alive. Pineoblastomas have a poor prognosis and the rate of survival in the French National Register is only 33% of cases. Germinomas can be treated with chemotherapy or radiotherapy alone and generally the indication for surgical removal is limited to residual tumours. The rate of survival is 64% at 10 years. For papillary tumours of the pineal region (PTPR) complete removal is a good prognostic factor. Radiotherapy seems to be effective in cases of residual tumours. The experience and results with radiosurgery treatment have been limited in the French national and Lyon experience., (Copyright © 2014. Published by Elsevier Masson SAS.)

Unlabelled: The surgical experience of the sub-occipital approach for treatment of pineal gland and pineal region tumors is reported. This approach was originally proposed by Jamieson and modified by Lapras who by changing the shape of the bone flap obtained the elevation of the occipital lobe which consequently resulted in a better exposition of this deep region. The reason why this approach became the basis for their treatment is particularly related to the personal experience of Lapras who reported his fantastic experience of surgery in this deep area and demonstrated the advantages of the sub-occipital transtentorial approach., Material: Out of 277 patients operated from 1982 to 2012 in Lyon for a pineal tumor, 233 were treated by a sub-occipital approach: 153 males, 125 females, 75 patients of pediatric age. The majority of patients were operated on in a sitting position which represents a surgical specialty of the anesthesiological school of Lyon., Results: Complete tumor removal was possible in 135 patients (58%) and partial in 60 patients (26%). For 38 patients, it was not possible to establish the quality of surgical resection. The incidence of hemianopsia decreased to less that 2% of cases while the incidence of severe pneumocephaly which requires a prolonged intensive care hospitalization was less than 4% of cases. Mortality related to this surgical approach in the Lyonnais school series was 0% during this period., Conclusion: In our experience the sub-occipital transtentorial approach seems to us the best approach for pineal tumors because it permits a large exposition of the pineal region favoring the removal of the tumor with a lateral extension and also for tumors extending low into the posterior cranial fossa. In fact, this is our preferred approach because it has been used in a large majority of cases. However, surgeons have to be familiar with other possible approaches to obtain the best result in terms of removal and also to decrease the rate of sequelae to improve the quality of life of these patients., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

The present paper investigates the clinical picture and the different clinical signs that reveal pineal region tumors or appear during the course of the follow-up. Biological malignancy and tumor extension determine the semiology and its setting up mode. Typical endocrine signs, dominated by abnormal puberty development, are frequently a part of the clinical scene. Bifocal or ectopic localization in the hypothalamic-pituitary region is accompanied by other endocrine signs such as ante- or post-pituitary insufficiencies which occur several months or even years after the first neurological signs appear. Due to a mass syndrome and obstructive hydrocephalus, intracranial hypertension signs are frequent but unspecific. A careful ophthalmologic examination is essential to search upward gaze paralysis and other signs of the Parinaud's tetrad or pentad. Midbrain dysfunction, including extrinsic aqueduct stenosis, are also prevalent. Except for abnormal pubertal signs, hyper-melatoninemia (secretory tumors) or a-hypo-melatoninemia (tumors destructing pineal) generally remains dormant. Some patients present sleep problems such as narcolepsy or sleepiness during the daytime as well as behavioral problems. This suggests a hypothalamic extension rather than a true consequence of melatonin secretion anomalies. Similarly, some patients may present signs of a "pinealectomized" syndrome, including (cluster) headaches, tiredness, eventually responsive to melatonin., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Deeply located beneath the corpus callosum and surrounding by crucial veins, the pineal and tectal structures still challenge the surgeon. Either anterior or posterior, many surgical approaches have been developed to reach the pineal region. Most popular are likely the posterior sub-occipital or occipito-parietal transtentorial routes. Others, primarily transcallosal or supracerebellar, may be indicated depending of the extension of the tumors while the transcortical routes (frontal, parietal or atrial) have been almost given up. Our purpose in this article is give a practical overview of how to do and what are the respective indications of all these alternatives approaches developed for pineal tumors., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

In order to determine sources and metabolism of melatonin in human cerebrospinal fluid (CSF), melatonin and 6-sulfatoxymelatonin (aMT6S) concentrations were measured in CSF sampled during neurosurgery in both lateral and third ventricles in patients displaying movement disorder (Parkinson's disease, essential tremor, dystonia or dyskinesia) and compared with their plasma levels. Previous determinations in nocturnal urine had showed that the patients displayed melatonin excretion in the normal range, compared with healthy controls matched according to age. A significant difference in melatonin concentration was observed between lateral and third ventricles, with the highest levels in the third ventricle (8.75±2.75pg/mL vs. 3.20±0.33pg/mL, P=0.01). CSF aMT6s levels were similar in both ventricles and of low magnitude, less than 5pg/mL. They were not correlated with melatonin levels or influenced by the area of sampling. Melatonin levels were significantly higher in third ventricle than in the plasma, whereas there was no difference between plasma and lateral ventricle levels. These findings show that melatonin may enter directly the CSF through the pineal recess in humans. The physiological meaning of these data remains to be elucidated., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

The knowledge of the incidence of pineal tumours is important not only for diagnostic care but also for its therapeutic programme. We reviewed the incidence of pineal tumours reported in literature in an attempt to establish if a difference existed between pineal gland tumours and the pineal region tumours as different authors use both expressions to indicate the same thing. The rate of frequency of these tumours is useful to guide the therapeutic choice for patients as the decisional tree is completely different for either germ cell tumours, pineal gland tumours or pineal gliomas and eventually papillary tumours of the pineal region. According to the French Register of pineal tumours, true pineal tumours represent: 27% pineal parenchymal tumours (PPT), 27% germ cell tumours, 17% gliomas, 8% papillary tumours. True pineal gland tumours are represented by: pineocytomas - (13%), pineal parenchymal tumours with intermediary differentiation PTT-ID - (66%) and pinealoblastomas - (21%). There was no statistical difference found between the French register and the Lyon series concerning histological diagnosis. It seemed to us important to discover its true incidence by comparing the data published in the literature and to stress the utility of the French Register for these uncommon tumours not only for recording new histological cases but also to document clinical symptomatology, therapeutic programmes, length of follow-up and clinical results for each patient treated. A better understanding of their natural history and improved evaluation of different treatments and their complications should contribute to improve clinical results., (Copyright © 2014. Published by Elsevier Masson SAS.)

Unlabelled: The infratentorial supracerebellar approach is most widely used for pineal tumors. We report our own experience and technical considerations using this approach., Material: From 1982 to 2010, we operated on 232 patients with pineal region tumors. Of these, 201 patients were operated on using a suboccipital transtentorial approach while 31 patients were operated on using a supracerebellar infratentorial approach. The median age of the patients ranged between 8 months and 74 years. There were 19 children and 12 adults. All patients presented with elevated intracranial pressure. There were 6 pinealocytomas, 3 papillary tumors, 7 germinomas, 2 benign teratomas, 4 pineal cysts and 9 gliomas. Adjuvant post-surgical therapy consisted of chemo-radiotherapy in 4 patients, 2 with germinomas and 2 with a grade II/III gliomas. Radiotherapy was performed in the other twelve patients (5 germinomas and 7 gliomas)., Results and Complications: All patients are still alive at a median follow-up of eight years. Twelve of the 19 children are attending normal school classes for their age, 5 are attending classes for special needs children and 2 are not yet of school age at the last follow-up. Seven of the 12 adults are working normally, three are working part-time at the same job and two have retired but are able to lead a normal life. Postoperative complications included symptomatic diffuse cerebellar edema (one patient) completely resolved with a mild residual cerebellar syndrome; double vision secondary to IV nerve palsy (one patient); transitory Parinaud's syndrome (2 patients) and cerebellar gait (2 patients) nearly completely recovered at respectively six and twelve months., Conclusion: The supracerebellar infratentorial approach seems to be a safe and effective choice in the treatment of pineal region tumors. In our experience, it permits complete tumor resections with acceptable morbidity and all neurosurgeons should master this approach in order to adapt their surgical choice according to size, extent and the relationship of the lesion with the surrounding anatomical structures., (Copyright © 2014. Published by Elsevier Masson SAS.)

Tumors of the pineal region (TPR) include different entities: germ cell tumors (GCT), pineal parenchymal tumors (PPT), meningiomas, and glial tumors. Except for GCT, there are no peripheral markers and histopathological diagnosis needs biopsy or surgery. We studied daily melatonin variations in twenty-nine patients with TPR and five with tectal plate glioma (TPG), used as controls, before and/or after surgery. Before surgery, a melatonin nycthemeral rhythm was observed in patients with TPG and TPR (one cyst, three PPT, one papillary tumor of the pineal region, two meningiomas, six gliomas). Melatonin rhythm was dramatically reduced for undifferentiated or invasive tumors. After surgery, the absence of melatonin variation in some cases could be the consequence of pineal damage by surgery. The contribution of determination of melatonin profiles to the diagnosis of TPR remains limited but of interest. The evidence for melatonin deficiency could justify melatonin administration to prevent the postpinealectomy syndrome.

Pineal parenchymal tumours (PPT) are rare neoplasms and there have been few in vitro studies. Their capacity for synthesizing and secreting melatonin has been only partially examined. We investigated the presence of messenger RNA (mRNA) encoding tryptophan hydroxylase (TPH), arylalkylamine N-acetyltransferase (AANAT), hydroxyindol-O-methyltransferase (HIOMT), three enzymes involved in melatonin synthesis, and c-myc, a tumoural marker, in 10 PPT, one papillary tumour of the pineal region (PTPR), cell cultures derived from four PPTs and from three other tumours of the pineal region, and in normal pineal gland. Moreover, protein expression of TPH was investigated in three PPT and PTPR. Quantitative real-time reverse transcriptase-polymerase chain reaction and immunohistochemistry were used and the melatonin production by tumoural cells in vitro was analysed by radioimmunoassay. We showed that all the tumoural tissues and cells contained c-myc mRNA. mRNAs encoding TPH, AANAT and HIOMT were detected in all PPT, suggesting that tumour cells can synthesize melatonin. Only PPT expressed TPH protein. Cultured cells lost expression of transcripts throughout passages even if ultrastructural study revealed the presence of characteristic organelles in these tumoural cells. Nevertheless, the basal secretion of melatonin observed in one PPT culture is in favour of a maintained melatonin production and secretion by tumoural pinealocytes, but melatonin production was not stimulated by a beta noradrenergic agonist. Moreover, PTPR never expressed mRNA encoding TPH, AANAT and HIOMT. Our results may contribute to a better understanding of the biology of PTT and PTPR and may help to the diagnosis of these rare tumours.

Several types of tumors are known to originate from the pineal region, among them pineal parenchymal tumors (PPTs) and papillary tumors of the pineal region (PTPRs), probably derived from the subcommissural organ. As a result of their rarity, their histologic diagnosis remains difficult. To identify molecular markers, using CodeLink oligonucleotide arrays, gene expression was studied in 3 PPTs (2 pineocytomas and one pineoblastoma), 2 PTPRs, and one chordoid glioma, another rare tumor of the third ventricle. Because PTPR and chordoid glioma may present ependymal differentiation, gene expression was also analyzed in 4 ependymomas. The gene patterns of the 3 PPTs fell in the same cluster. The pineocytomas showed high expression of TPH, HIOMT, and genes related to phototransduction in the retina (OPN4, RGS16, and CRB3), whereas the pineoblastoma showed high expression of UBEC2, SOX4, TERT, TEP1, PRAME, CD24, POU4F2, and HOXD13. Using reverse transcriptase-polymerase chain reaction on 13 PPTs, we demonstrated that PRAME, CD24, POU4F2, and HOXD13 might be candidates for grading PPT with intermediate differentiation. PTPRs, classified with chordoid glioma and separately from ependymomas, showed high expression of SPEDF, KRT18, and genes encoding proteins reported to be expressed in the subcommissural organ, namely ZFH4, RFX3, TTR, and CGRP. Our results highlight the usefulness of gene expression profiling for classify tumors of the pineal region and identify genes with potential use as diagnostic markers.