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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.)

Background and Purpose: Pineal tumours account for 1% to 4% of brain tumours in adults and for around 10% in children. Except in a few cases where germ cell markers are elevated, accurate histological samples are mandatory to initiate the treatment. Open surgery still has a high morbidity and is often needless. Biopsies can either be obtained by endoscopic or stereotactic procedures., Methods: Following an extensive review of the literature (PubMed 1970-2013; keywords pineal tumour, biopsy; English and French), 33 studies were analysed and relevant data compared regarding the type of procedure, diagnosis rate, cerebrospinal fluid diversion type and rate, perioperative mortality, morbidity., Results: Endoscopic and stereotactic biopsies showed a diagnosis rate of 81.1% (20%-100%) and 93.7% (82%-100%), respectively. Endoscopic biopsies involved 21.0% of minor and 2.0% of major complications whereas stereotactic biopsies involved 6.4% of minor and 1.6% of major complications. The most frequently reported complication was haemorrhage for both endoscopic and stereotactic procedures, accounting for 4.8% and 4.3%, respectively. Mortality rate was low for both endoscopic and stereotactic procedures, equal to 0.4% and 1.3%, respectively. Local experience of stereotactic biopsies was also reported and corroborated the previous data., Conclusions: The difference between both procedures is not statistically significant (p>0.05) across large series (≥20patients). Nevertheless, tissue diagnosis appears less accurate with endoscopic procedures than with stereotactic procedures (81.1% versus 93.7%, weighted mean across all series). In our opinion, the neuroendoscopic approach is the best tool for managing hydrocephalus, whereas stereotactic biopsies remain the best way to obtain a tissue diagnosis with accuracy and low morbidity., (Copyright © 2014. 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.)

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 cases of pineal tumors, a germ cell tumor should always be suspected. As some of them are secreting tumors, tumoral markers (AFP and/or hGC) are an important part of the diagnostic process. Their positivity either in the serum and/or in the cerebrospinal fluid may lead to an accurate diagnosis, avoiding a potentially dangerous surgical biopsy. Follow-up of tumoral markers is useful during and after treatment in order to monitor response to chemotherapy or a remission status., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Background and Purpose: Pineal parenchymal tumours (PPTs) and pineal cysts represent one third of the pineal region lesions. PPTs are subdivided into pineocytoma (PC), pineoblastoma (PB) and PPT with intermediate differentiation (PPTID). We report morphological and immunochemical features which permit to grade these tumours., Methods: The description of histopathological features and grading is based on a large cooperative series and on the WHO 2007 classification., Results: PCs occur in adults between the third and the sixth decade of life. PBs typically occur in children. PPTIDs have a peak incidence in young adults between 20 and 40 years of age. There is no sex preference. PC is characterized by a uniform cell proliferation with large fibrillary pineocytomatous rosettes. PB is a high-density tumour composed of small blue cells with hyper-chromatic, round or carrot shaped nuclei. PPTIDs have lobulated or diffuse patterns. Grading is based on morphological features, count of mitoses and neurofilament protein (NFP) expression. PCs (grade I) have no mitosis and NFP is highly expressed in pineocytomatous rosettes. PBs (grade IV) are high mitotic tumours and present low or no expression of NFPs. PPTIDs are grade II when mitoses are fewer than 6 for 10 high-power fields and NFPs are expressed, and are grade III when mitoses are greater or equal to 6 or are fewer than 6 with NFPs lowly expressed. Pineal cysts may be differentiated from PPTs by the high expression of NFPs and no expression of Ki-67., (Copyright © 2014. Published by Elsevier Masson SAS.)

Germ cell tumors (GCTs) classically occur in gonads. However, they are the most frequent neoplasms in the pineal region. The pineal location of GCTs may be caused by the neoplastic transformation of a primordial germ cell that has mismigrated. The World Health Organization (WHO) recognizes 5 histological types of intracranial GCTs: germinoma and non-germinomatous tumors including embryonal carcinoma, yolk sac tumor, choriocarcinoma and mature or immature teratoma. Germinomas and teratomas are frequently encountered as pure tumors whereas the other types are mostly part of mixed GCTs. In this situation, the neuropathologist has to be able to identify each component of a GCT. When diagnosis is difficult, use of recent immunohistochemical markers such as OCT(octamer-binding transcription factor)3/4, Glypican 3, SALL(sal-like protein)4 may be required. OCT3/4 is helpful in the diagnosis of germinomas, Glypican 3 in the diagnosis of yolk sac tumors and SALL4 in the diagnosis of the germ cell nature of an intracranial tumor. When the germ cell nature of a pineal tumor is doubtful, the finding of an isochromosome 12p suggests the diagnosis of GCT. The final pathological report should always be confronted with the clinical data, especially the serum or cerebrospinal fluid levels of β-human chorionic gonadotropin (HCG) and alpha-fetoprotein., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

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.)

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.)

We present a case of growing teratoma syndrome of the pineal region. To our knowledge, this is the fourth case reported in the literature. A 13-year-old boy was referred for intracranial hypertension and bilateral papillary edema. CT scan showed a pineal region tumor with obstructive hydrocephalus. After CSF (cerebrospinal fluid) shunting, MRI showed that the tumor had a heterogenous signal enhancement. The tumor marker HCG (human chorionic gonadotrophin) was elevated in CSF and serum. After three cycles of chemotherapy, MRI showed an important increase in tumor size with morphologic modifications. However, HCG in CSF and serum returned to normal. Surgical resection was performed and histological examination of the whole specimen showed mature teratoma. On postoperative MRI, there was a small area of signal enhancement of the left thalamus. Radiotherapy was given. The child was in complete remission 15 months after the diagnosis. Growing teratoma syndrome is a mixed germ cell tumor with a secreting portion that responds to chemotherapy and a non secreting portion of mature teratoma that continues to grow under chemotherapy. The treatment should include chemotherapy for the malignant secreting portion and surgery for the mature teratoma.

The now nine years old girl with growth retardation, started to be ill with otitis and then diabetes insipidus of central origin at 1974. A treatment with lysin-vasopressin is prescribed. The PNEG in May 1976 shows a little, pea like, suspicious, supra-sellar nodule who is not surgically explored since they was no ophtalmologic symptoms and because a normal CT scan. Corticoid and thyroid substitutive therapy is added until September 1977 when the general status becomes impaired and vomiting starts. Also because some visual loss, a new neuroradiological study is performed showing a supra-sellar tumour and a fourth ventricle mass. The CT scan asserts the double intracranial expansive process and a posterior fossa craniotomy is done with subtotal resection of a vermian tumour and Torkildsen drainage. The histology is : Immature Dysembryoma (seminoma type) or germinoma. The follow-up was good under hormonal care. X Rays Therapy over the posterior fossa, the suprasellar region, the brain and the spinal channel was instaured. Four months later, the CT scan shows normal sized ventricles and no tumour mass at all. This case gives the authors opportunity for comments and to study the concerned literature.