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BACKGROUND: Different treatment options have been proposed for obstructive hydrocephalus associated with pineal lesions. We discuss the obstructive hydrocephalus management associated with pineal region tumors and cysts in Helsinki Neurosurgery. METHODS: In this article, hydrocephalus treatment by tumor-cyst removal (n = 40), shunt surgery (n = 25), and endoscopic ventriculostomies (n = 3) is evaluated in 68 patients with obstructive hydrocephalus among 136 patients with pineal region tumor and cyst. Multivariate statistical analysis was followed by univariate and multivariate regression models of last functional status, last tumor-free imaging, and disease-specific mortality of the study population. RESULTS: Preoperative hydrocephalus was linked to higher World Health Organization tumor grades, poor functional status, higher mortality, and incomplete resection of pineal region cysts and tumors. Preoperative hydrocephalus remained a predictor of poor last functional status after multivariate regression. Pineal lesion removal with the posterior third ventricle opening as primary hydrocephalus treatment resulted in better last functional status, fewer postoperative shunts, fewer hydrocephalus related procedures, and fewer postoperative infections than in the shunt-treatment group. Multivariate regression analysis linked higher World Health Organization tumor & nbsp;grade, poor immediate functional status, postoperative complications, and incomplete surgical resection as independent predictors of disease mortality in patients with hydrocephalus. Same variables (except immediate modified Rankin Scale score) and higher number of shunt surgeries became independent predictors of poor last functional status at multivariate analysis. Incomplete resection was the only independent predictor of tumor-free magnetic resonance imaging at the last evaluation. CONCLUSIONS: Direct removal of pineal lesions with the opening of the posterior third ventricle could represent effective and reliable management of the associated obstructive hydrocephalus. Further research is required to generalize our inferences.

OBJECTIVE: The midline supracerebellar infratentorial (SCIT) approach and its paramedian development are commonly used for dealing with pineal lesions. Comparative clinical studies are lacking, however. We aim to establish the better performance of the paramedian SCIT approach in terms of clinical safety in surgically treated pineal cysts and pineal region tumors. Procedural functionality and effectiveness have been also analyzed. METHODS: A comparative analysis of clinical, radiologic, pathologic, and surgical features, and outcome was performed between 55 midline and 57 paramedian SCIT approaches that were exclusively performed in 112 patients (57 pineal cysts and 55 tumors of the pineal region) operated in sitting position by a single surgeon. Information was retrieved from hospital records and microsurgical videos. RESULTS: The paramedian SCIT approach linked with fewer postoperative complications (odds ratio [OR]: 0.40) and fewer approach-related complications (OR: 0.28) than the midline SCIT approach. The SCIT paramedian approach was achieved in a shorter time, by a smaller bone flap, and with fewer complex procedural steps than the midline approach. The SCIT paramedian approach did not require the opening of the falx cerebelli, midline cerebellar retraction, section of the midline cerebellar draining veins, nor wide opening of the dura. Gross total resection, size of the lesion, microsurgical time for removal, histopathological diagnosis and postoperative outcome were statistically similar in both groups. CONCLUSIONS: The SCIT approach represents a safer and more functional approach for the removal of cysts and tumors of the pineal region than the classic midline approach, while maintaining the same effectiveness.

Pineal parenchymal tumors are categorized into WHO Grade I pineocytoma, WHO Grade II or III pineal parenchyma tumor of intermediate differentiation (PPTID), and WHO Grade IV pineoblastoma. Aggressive surgery to achieve gross total resection (GTR) may be challenging in the pineal region due to the risk of neurologic injury. There is currently no consensus from phase III prospective data to guide adjuvant therapy in PPTID, but focal radiotherapy should be considered in this setting to improve local control. Papillary tumors of the pineal region (PTPR) is another rare neuroectodermal tumor with limited published literature and no established guidelines with respect to adjuvant therapy after surgical resection; however, focal radiotherapy should also be considered to improve local control. In this chapter, we present general principles of treatment planning for these two disease entities of the pineal region.

Tumors arising in the pineal region present a number of challenges when planning for effective removal. This report describes the successful resection of a falcotentorial meningioma occurring in a 56-year-old female using a supracerebellar infratentorial approach. In order to excise the pineal region mass, a unique combination of instrumentation was used, including a microscope, endoscope, and abdominal laparoscope. This technique afforded us passage to the pineal region, which allowed for enhanced visualization and maneuverability and was more amenable to decreasing the physical stress of the operating surgeon. This article is the first to detail the use of an abdominal laparoscope to remove a pineal tumor of this size for near-total resection. The various surgical approaches and tools traditionally used to remove pineal tumors are discussed, and the particular advantages and disadvantages of our hybrid approach are reviewed.

International audience; Overall, tumors of primary central nervous system (CNS) are quite common in adults with an incidence rate close to 30 new cases/100,000 inhabitants per year. Significant clinical and biological advances have been accomplished in the most common adult primary CNS tumors (i.e., diffuse gliomas). However, most CNS tumor subtypes are rare with an incidence rate below the threshold defining rare disease of 6.0 new cases/100,000 inhabitants per year. Close to 150 entities of primary CNS tumors have now been identified by the novel integrated histomolecular classification published by the World Health Organization (WHO) and its updates by the c-IMPACT NOW consortium (the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy). While these entities can be better classified into smaller groups either by their histomolecular features and/or by their location, assessing their treatment by clinical trials and improving the survival of patients remain challenging. Despite these tumors are rare, research, and advances remain slower compared to diffuse gliomas for instance. In some cases (i.e., ependymoma, medulloblastoma) the understanding is high because single or few driver mutations have been defined. The European Union has launched European Reference Networks (ERNs) dedicated to support advances on the clinical side of rare diseases including rare cancers. The ERN for rare solid adult tumors is termed EURACAN. Within EURACAN, Domain 10 brings together the European patient advocacy groups (ePAGs) and physicians dedicated to improving outcomes in rare primary CNS tumors and also aims at supporting research, care and teaching in the field. In this review, we discuss the relevant biological and clinical characteristics, clinical management of patients, and research directions for the following types of rare primary CNS tumors: medulloblastoma, pineal region tumors, glioneuronal and rare glial tumors, ependymal tumors, grade III meningioma and mesenchymal tumors, primary central nervous system lymphoma, germ cell tumors, spinal cord tumors and rare pituitary tumors.

A combination of modern methods of high-tech microneurosurgery, neuroanesthesia and neuroreanimation allows a successful realization of the program for the surgical treatment of pineal tumors. Such operations belong to the highest difficulty category and carry a high surgical risk.

Background: Diffuse midline glioma (DMG) is an invasive astrocytic tumor arisen from midline structures, such as the pons and thalamus. Five cases of DMG in the pineal region have been reported, but the clinical course was poor; there was no case of survival for more than 2 years. Case Description: We report the case of a 12-year-old boy with DMG in the pineal region who is living a normal daily life for more than 6 years following multimodal treatment. He complained of a headache accompanied by vomiting that had gradually worsened 1 month previously, and initial magnetic resonance imaging revealed a pineal tumor. Germinoma was initially suspected; however, a combination of chemotherapy using carboplatin and etoposide was ineffective. The first surgery was performed through the left occipital transtentorial approach (OTA); the diagnosis was DMG. After 60 Gy radiotherapy concomitant with temozolomide (TMZ), the tumor enlarged. Second surgery was performed through bilateral OTAs, and 90% of the tumor was removed. In addition, stereotactic radiotherapy (30 Gy, six fractions) was administered, and the local equivalent dose in 2 Gy/fraction reached 97.5 Gy. Maintenance chemotherapy using TMZ and bevacizumab was continued for 2 years. After finishing chemotherapy, the enhancing lesion enlarged again, and bevacizumab monotherapy was effective. Now, at 6 years after diagnosis, the patient leads an ordinary life as a student. Conclusion: Maximum resection and high-dose radiotherapy followed by bevacizumab may have been effective in the present case.

Intracranial teratomas are rare germ cell neoplasms that contain tissues derived from all three germ cell layers and most commonly occurring during childhood. This is the first report of pineal region mixed mature teratoma and germinoma in two fraternal brothers of fraternal triplets.We report the case of a mixed mature teratoma and germinoma of the pineal region in two brothers of fraternal triplets. Older brother was initially diagnosed at the age of 11 years with the pure teratoma of the pineal region but the review of the pathology 3 years after initial surgery revealed the mixed mature teratoma with 5% germinomatous component. The younger brother was diagnosed at the age of 13 years with the mixed mature teratoma with 10% germinomatous component tumor of the pineal region. Younger brother has been treated with adjuvant chemo-radiotherapy and older brother was treated without adjuvant therapy. Both brothers had no recurrence.Pineal mature teratomas have a good prognosis, in contrast to their immature or mixed counterparts. A rigorous histological examination of the tumor samples is mandatory, in order to not omit a mixed contingent within the tumor.

The pineal region is a complex area of the brain, including many different tissue elements that can give rise to a wide variety of tumor processes. Pineal region tumors represent 2.5–8.5% of all pediatric intracranial tumors, and 1% of those in adults. Other lesions located in this region, such as arteriovenous malformations, vein of Galen aneurysmal malformations, or cavernomas, are even rarer and are challenges for diagnostics and management.

Large pineal lesions displacing the splenium of the corpus callosum may be approached through posterior interhemispheric trajectory with concomitant splenium section. Transcallosal approach provides direct route to the pineal lesion with the advantage to visualize the superior, anterior, and posterior borders of the lesion. Transcallosal approach is frequently associated with neuropsychological complications and a risk of damaging the deep venous system. Although replaced by other approaches to pineal region lesions, transcallosal approach is still being used, predominantly in pediatric pineal region lesions.

The complexity of pineal region lesions requires a fine-tuned approach, the therapy depending on the nature of the lesions and the range of mixed preoperative tests. Oncological treatment—chemo- and radiotherapy—in case of malignant tumors usually entails a good response. One-third of the lesions are benign, surgery being usually curative in their case. This chapter highlights the important aspects of the therapeutic approach based on the authors’ practical experience, from anatomy of the pineal region, laboratory diagnosis, pathology, clinical and imaging features, and anesthetic methods to surgical approaches, illustrated with case presentations.

Radiation therapy is an important part of the multidisciplinary treatment of pineal tumors. Target volume and total dose administered depend on the histological type and extension of disease. Radiation treatment can be performed using highly conformal techniques, different by dose distribution, dose to normal structures and organs at risk, dose amount outside the target volume, and treatment time and cost.

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.

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.

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.

To describe the use of the NICO Myriad, a new side-cutting aspiration device for the resection of tumors, in a developing country. The 11-, 13-, and 15-ga handpieces were used to resect tumors exposed via craniotomies, and the 19-ga handpiece was used down the side channel of a Storz Oi endoscope to resect tumors exposed endoscopically. The Myriad was used to resect 23 tumors, including spinal cord tumors, posterior fosssa tumors and pineal tumors, and the cysts associated with two craniopharyngiomas. No complications were associated with the Myriad. Handpieces that were re-sterilized in Steranios after the initial use could each be used two to four times thereafter. The Myriad is the first effective tumor removal device that can be introduced down the side channel of most endoscopes, greatly expanding the spectrum of tumors that can be treated endoscopically. Its minimal diameter allows better visibility in small, deep sites such as the pineal region than is usually available when ultrasonic aspirators are used. The cost of the device, and particularly the handpieces, will limit their utility in developing countries until re-usable handpieces are developed.

Manuscript submitted May 23, 2011. Accepted September 25, 2011. Kenneth Grant Jamieson is celebrated as one of Australia's top neurosurgeons. His most notable contributions to neurosurgery included novel treatments of aneurysms and pineal tumors and studies of head injury. Jamieson was also an innovator for the development of new neurosurgical instruments and renowned for his teaching abilities, prolificacy, and mentorship. This preeminent neurosurgeon's life was cut short at the age of 51. Our current understanding and knowledge of treatments of various neurosurgical diseases is based on pioneers such as Kenneth Grant Jamieson.

Pineal tumors are rare neoplasms that are categorized into pineoblastomas, pineocytomas, and pineal parenchymal tumors of intermediate differentiation. Pineal anlage tumors are primary pineal tumors with neuroepithelial and ectomesenchymal differentiation and without endodermal differentiation. The authors review the literature and report the case of a 5-month-old boy with a pineal anlage tumor. This is only the sixth case of a pineal anlage tumor reported in the English-language literature adding to the understanding of this tumor's presentation, immunomorphological and molecular characteristics, embryological origin, radiological appearance, treatment outcome, and prognosis.

Meningiomas are uncommon tumors in children and either more rarely encountered in the pineal region. We report two cases of meningioma of the pineal region in children. One of these cases was a five years-old girl and the other a one year-old boy. No specific clinical presentation or tomographic examinations findings was identified before treatment, suggestive of a diagnosis of menigioma. The clinical and laboratory features were very similar to the most common tumors of the pineal region. Prior to the surgery, the histology of these tumors was not suspected. Both patients underwent direct surgery and complete removal was achieved by a suboccipital transtentorial approach. The tumors originated from velum interpositum in both cases. At the follow up, one case presented with recurrence six years later, and she underwent a reoperation with total resection without morbidity. Long-term follow up presented no other recurrences. Meningiomas são tumores poucos frequentes em crianças, e mais raramente encontrados na região da pineal. Relatamos dois casos de meningioma da região da pineal em crianças, uma menina de cinco anos e um menino de um ano de idade. Não foi identificada nenhuma forma de apresentação clinica ou caracteristica tomográfica, antes do tratamento, que sugerisse o diagnóstico de meningioma. As características clinicas e laboratoriais encontradas foram similares às de tumores mais frequentes da região da pineal. Ambos os pacientes foram submetidos ao tratamento cirúrgico e a remoção completa foi obtida por abordagem suboccipital transtentorial. Durante o seguimento, um dos pacientes foi reoperado por recorrencia do tumor seis anos após o tratamento inicial. Atualmente, os pacientes encontram-se livres de recorrência tumoral.

Long-standing debate continues about the management and biopsy of pineal tumors because of their complex microsurgical anatomy and deep location. Inspired by the concept of biopsy under direct visualization in the absence of hydrocephalus, we explored the effectiveness of neuroendoscope outside of its traditional territory using a new minimally invasive technique, computer-assisted cisternal endoscopy (CACE), for the biopsy of pineal tumors.Five cadaver heads were dissected to expose the pineal region through the posterior fossa. In the other 5 heads, a rigid endoscope-wand combination was introduced in the supracerebellar space lateral to the arachnoid of the superior cerebellar cistern in midline. Endoscopic exposure of the pineal gland was correlated with the real-time image of the localizing wand. After the wand was removed, arachnoid was further dissected from the deep veins and the pineal gland, and a four-quadrant biopsy was obtained.The combination of technologies of frameless guided stereotaxy and neuroendoscopy enhanced our ability to navigate the ventriculoscope in narrow spaces (e.g., posterior fossa cisterns). Compared with transventricular and conventional stereotactic trajectories, application of CACE in supracerebellar infratentorial trajectory offered the shortest route to the pineal region, anatomical orientation, no violation of eloquent neurovascular structures, and adequate visibility to deep veins and arteries.CACE may be used to approach pineal lesions outside the cerebral ventricular system for biopsy or debulking. Continuous computer updates on the endoscope position allows its safe navigation in narrow spaces (e.g., cerebrospinal fluid cistern). Its success will await future surgical trials.

There are three goals in the treatment of pineal tumors: diverting cerebrospinal fluid (CSF) in the case of hydrocephalus, obtaining tissue for diagnosis, and obtaining CSF to identify biological markers. Neuroendoscopy can accomplish all these goals. Two routes can be used to treat pineal tumors. In the transventricular transforaminal approach, the pineal mass is exposed from its anterior aspect. The other route is posterior from a supracerebellar infratentorial approach that resembles the standard surgical approach. It is crucial to recognize that the aqueduct may be compromised and a third ventriculostomy may need to be performed.

We have applied the neuronavigation system to endoscopic biopsy and third ventriculostomy in the management of patients with a pineal tumor with hydrocephalus. With the guidance of neuronavigation, the two optimal sites of burr hole and trajectories were planned preoperatively, and the advancing endoscopic device was monitored in real time during the procedure. In our five patients, the diameters of the tumors were 2-3 cm, and the mean systemic accuracy of registration with neuronavigation was 1.2 mm. The biopsy and third ventriculostomy were performed successfully via the respective optimal burr hole and the trajectory determined using preoperative neuronavigation. There were no procedure-related complications, and none of the patients needed another procedure for CSF diversion during the follow-up periods. We present our technique which includes the application of the neuronavigation system to the biopsy and third ventriculostomy in pineal tumor with associated hydrocephalus. This technique can be performed using a simple rigid endoscope via the determined optimal entries and trajectories. The optimal preoperative planning and the intraoperative guidance by neuronavigation are thought to be able to give more chances to minimize the brain injury related to movements or deviation of endoscopic device.

Radiotherapy plays a vital role in the vast majority of tumors that arise from the pineal region. While most pineal tumors are quite rare, refinement of radiation techniques and doses over the past several decades, along with concomitant improvements in surgical techniques and chemotherapeutic regimens, have led to improvements in patient outcomes.

Background: Papillary pineocytoma is an extremely rare tumor usually with a poor outcome. Case report: We report a case of a 10-year-old-girl with pineal gland tumor and obstructuve hydrocephalus diangnosed using MRI. The child was successful treated by insertion of a ventriculoperitoneal shunt and consecutive tumor resection by supracerebellar-infratentorial approach. Histopathological examination showed a papillary structure of the pineocytoma. As such, tumors are considered to be aggressive the child was subjected to radio- and chemotherapy. Conclusion: At six year follow-up after surgery, the patient is symptom-free and the MRI shows no tumor recurrence.

Resumen Revisamos el tratamiento y los resultados de una serie de 21 pacientes con lesiones de region pineal. De los 21 casos en diecinueve se realizo cirugia abierta. Nuestra serie incluye: 5 germinomas, 1 teratoma atipico, 2 teratomas maduros, 1 tumor mixto, 3 pineoblastomas, 1 pineocitoma, 2 gliomas, 1 cordoma, 2 quistes aracnoideos y una metastasis. Los otros dos tumores fueron catalogados como germinomas. El estudio preoperatorio debe incluir RM craneoespinal con y sin contraste, citologia de LCR y valoracion de marcadores tumorales en suero y LCR. Nosotros pensamos que el manejo correcto de estas lesiones requiere abordaje quirurgico para diagnostico histologico. Dicho abordaje vendra determinado por las imagenes de la RM preoperatoria y la extension tumoral. El tratamiento postoperatorio debe basarse en la histologia y la extension de la lesion. Los procesos benignos deben tratarse unicamente con cirugia. La radioterapia craneoespinal y quimioterapia deben ser administradas en aquellos pacientes con lesiones altamente malignas, diseminacion meningea, metastasis extraneural o citologia positiva. En nuestra serie la mortalidad operatoria es nula y la morbilidad minima.

In recent years, the use of the endoscope is a valid alternative to the classical microscopical approaches for the treatment of the pineal tumors. It can be used for endoscopic tumor biopsy (ETB) as well as endoscopic-assisted complete tumor removal in some cases. At the same time, if there is coexisting hydrocephalus, this can be treated endoscopically with endoscopic third ventriculostomy (ETV).

Pineal tumor management in pediatric patients must be based on close co-operation between oncologists, surgeons, radiation oncologists, neurologists, ophthalmologists, and endocrinologists. Radiation therapy (RT) remains critical in most situations and should be assessed as soon as the diagnosis is made, in order to optimize the radiation technique. This paper will focus on RT modalities, indications, as well as modalities in main pediatric pineal tumors (germ cell tumors and pineal parenchyma tumors). RT modalities are presently being debated and new RT techniques (intensity-modulated RT, proton therapy etc.) that are now available for pineal lesions need to be evaluated. Radiation strategies are also controversial for germ cell tumors: cranio-spinal radiation versus chemotherapy followed by focal radiation, which also requires discussion.

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.

Pineal region tumors can be difficult to biopsy, given the critical structures in the location of the pineal gland. Modern computerized imaging techniques like computed tomography and magnetic resonance imaging used in association with tumor markers and the age at presentation may enable treatment without biopsy using a rational treatment algorithm.

Pineal tumors comprise 0.4 - 1.0% of intracranial space-occupying lesions in adults. Papillary tumor of pineal region (PTPR) is a very rare entity. It has been newly described in WHO 2007 classification of brain tumors. Only a few case reports are available in the literature. We report a case of a 60 year-old female presenting with headache, giddiness and reduced vision. Imaging studies showed a pineal mass with areas of hemorrhage. All ventricles were normal. There was a past history of a pineal gland tumor excised 2 years ago. This case is being reported for its rarity and aggressiveness in the form of recurrence. Limited/available immunohistochemistry workup has been done.

CRX (OTX3) is a transcription factor of the OTX homeobox family, whose expression and function is essential for the development and differentiation of retinal and pineal cells. Among human cancers, CRX seems to be selectively expressed only by retinoblastomas and pineal tumors. In our immunohistochemical analysis, performed on 89 primary and metastatic central nervous system tumors, we found that CRX is strongly expressed by normal pineal tissue as well as by pineal parenchymal tumors. Other than pineal tumors, we observed CRX positivity not only in a few medulloblastomas but also in atypical teratoid/rhabdoid tumors and in small cell lung carcinoma metastasis, in which photoreceptor differentiation has not been reported so far. In conclusion, CRX could represent a potential routine immunohistochemical marker in surgical neuropathology for the differential diagnosis of tumors of the pineal region. However, owing to the significant percentage of negative cells in some pineal tumors of our series, CRX negativity does not definitively rule out the diagnosis of a pineal parenchymal tumor, particularly in case of small biopsy specimens.

Background and Objective Surgical treatment for pineal tumors is technically challenging—weighing the risks and benefits of microsurgical resection for the patient with a pineal tumor versus settling for an endoscopic third ventriculostomy and biopsy is sometimes difficult. Traditional microsurgical resection for pineal region tumors has typically required large open craniotomies and involvement or retraction of neural tissue with significant mortality and morbidity. With the advancement of high-resolution fiber optics, a fully endoscopic, supracerebellar, infratentorial approach, without any cerebellar retraction or manipulation of neural tissue, is introduced for the gross total resection of pineal region tumors. Conclusion As an endoscopic modification of the open craniotomy procedure, this technique combines the advantages and benefits of both open microsurgical resection and minimally invasive endoscopic surgeries.

Objectives Pineal lesions can present as a heterogeneous collection of benign and malignant disease conditions. Pineal lesions include germ cell tumours, neoplasms arising from the pineal parenchyma, as well as other pineal region masses. Methods A variety of cases of pineal lesions are presented. The important clinical features and typical imaging findings of each pineal lesion are described with emphasis on their morphological appearance and signal intensity characteristics on magnetic resonance imaging (MRI). Conclusion Knowledge of the imaging characteristics and clinical features of varying pineal lesions can assist in narrowing the differential diagnosis for more accurate and rational therapeutic planning. Teaching Points • Pineal parenchymal tumours show an “explosion” of normal pineal calcifications towards the periphery. • Pineoblastomas often have restricted diffusion, with apparent diffusion coefficient (ADC) values lower than germinomas. • Pineal teratomas and pineal lipomas display fat signal characteristics and fat saturation on MRI. • Pineal lesions in patients with known malignancy should raise suspicion of metastatic involvement. • Pineal cysts and arachnoid cysts show MRI signal characteristics similar to cerebrospinal fluid (CSF).

The pineal gland has been studied through philosophy and science for thousands of years. Its role in human physiology was not well understood until the scientific community first started to report on pineal pathology in the eighteenth century. Throughout the nineteenth and twentieth centuries, reports on pineal tumors and the emergence of comparative anatomy allowed more complete understanding of pineal function. Neurosurgical methods of treating pineal pathology first emerged in the early twentieth century. In the latter half of the twentieth century, the emergence of microsurgical technique allowed for excellent outcomes with minimal morbidity and mortality.

Pineal region tumors represent 0.4% to 1.0% of intracranial tumors in American literature. Obtaining a tissue diagnosis is the cornerstone of the rational management of pineal lesions. The initial surgical decision involves choosing between a stereotactic biopsy and open microsurgical procedures. Open resection facilitates the maximal removal of tumor volume and has diagnostic accuracy and improved prognosis. Stereotactic biopsy is less invasive and has a lower risk of complications. A review of all major series reporting stereotactic biopsy for pineal region lesions reveals a mean diagnostic yield of 94%, with a morbidity of 1.3% and a mortality of 8.1%.

Pineal tumors are relatively rare central nervous system lesions with a predilection for the pediatric population. For the vast majority of these lesions, surgical resection is a critical step in effective treatment. This article discusses current strategies for preoperative evaluation, operative management, and postoperative care of the pediatric patient with a newly diagnosed pineal region tumor.

Endoscopic resection of pineal tumors using an endoscope with a mounted rigid suction that allows bimanual handling of the tumor for resection. This contrasts to the established method of biopsy of pineal tumors through intraventricular approach.Two patients, one with a cystic lesion in the pineal region and one with a large pineal tumor, were operated in sitting position through a subtorcular approach. Endoscope was held in the left hand with suction tip extending beyond the tip through its instrument channel. Regular microsurgical instrumentation/CUSA/Nico Aspirator was used with the right hand for dissection, cutting, and removing the tumor under endoscopic vision.Surgeon comfort was superior to when microscope is used in sitting position. Complete resection was achieved in all cases.The two-handed endoscopic technique using a mounted suction on the endoscope as described is a safe and effective strategy for resecting pineal region tumors.

Epidemiology, Histologic Classification and Clinical Course of Brain Tumors.- Imaging Modalities in Brain Tumors.- Molecular Abnormalities in Gliomas.- Low-Grade Gliomas.- High-Grade Gliomas.- Pineal Tumors.- Embryonal Tumors, Tumors of Cranial Nerves.- Meningeal Tumors.- Lymphomas and Hemopoietic Neoplasms.- Masses of the Sellar and Junxtasellar Region.- Brain Metastasis.- Scinitgraphy in Brain Tumors.

T UMORS Of the pineal body are rare. According to Cushing's 1 figures, 0.7 per cent of his 2,000 intracranial tumors were pineal in origin. In spite of their rarity, however, certain clinical manifestations of growths in this region are of more than usual interest. Among these features arc the disturbances concerned with pupillary activity, movements of the eyeballs and other ocular disorders. The paper here presented is devoted chiefly to a clinical study of these oculomotor and other related disturbances. The material from which this information has been gathered consisted of 16 cases in which a diagnosis of pineal tumor was made at the Lahey Clinic during the years 1933 to 1944 inclusive. In 11 of the 16 patients the lesion was verified histologically either from tissue removed at operation or at autopsy. In the other 5 patients a presumed and almost certain diagnosis was made on the basis of the clinical evidence together with the finding of the characteristic filling defect in the posterior portion of the third ventricle as seen in the ventriculogram. These diagnostic features were supported by the demonstration of internal hydrocephalus and the complete relief of symptoms and signs following a decompression and roentgen therapy. In spite of the fact that it was not possible to verify the tumor histologically in these 5 patients, we feel that the clinical picture, the ventriculogram and the course following decompression and radiation therapy all support the diagnosis of pineal tumor with sufficient certainty to include them in this survey. The surgical removal of a pineal tumor is an extremely difficult task and entails considerable risk. On the other hand, as Horrax and Daniels 5 have pointed out, subtemporal decompression followed by radiation therapy offers a comparatively safe method of treatment which has proved highly satisfactory in several cases. For this reason it is highly important to obtain all available evidence bearing upon the diagnosis of a growth in the pineal region. The oculomotor and pupillary disturbances furnish considerable information along these lines. Before taking up in detail the various ocular disorders that may occur with pineal tumors, it may be well to note briefly certain general features shown by patients harboring these lesions. By recalling the strategic position of the epiphysis just above the corpora quadrigemina (Fig. 1), one can predict with a good deal of accuracy the most frequent signs and symptoms resulting from a tumor of this structure: 1. Increased intracranial pressure, often acute, producing headache

Approximately 18,400 primary brain tumors were diagnosed in the United States during 2004, and, of these, approximately 12,690 resulted in death [1]. Pineal tumors make up only 0.4% to 1% of all brain tumors [2].

MR images of pineal region tumors were analyzed in 26 patients with histologically proved tumors: seven germ-cell tumors, six astrocytomas, five teratomas, three pineoblastomas, two meningiomas, one dermoid, one epidermoid, and one metastasis. In an attempt to identify specific MR characteristics of these lesions, Gd-DTPA was administered to six patients. CSF and blood serum were assayed for alpha-fetoprotein (AFP) and human chorionic gonadotropin-beta subunit (HCG-beta) in 18 patients. MR findings were correlated with age, sex, the presence of biochemical tumor markers, and surgical outcome. We found that the most important factors in the determination of tumor type were the patient's age and the tumor markers. Increased levels of both HCG-beta and AFP were specific for patients with malignant teratomas and undifferentiated germ-cell tumors. HCG-beta alone was elevated in the patient with choriocarcinoma; only AFP was elevated in the patient with an endodermal sinus tumor. Tumor markers were not present in other patients in this series. The tumor size and the presence of fat were also helpful in determining tumor type. Hemorrhage was rare, seen only in the patient with a choriocarcinoma. Gd-DTPA did not enhance diagnostic specificity but aided in the detection of tumor seeding through CSF. We conclude that, although MR is sensitive in the detection of pineal region tumors and provides superb anatomic detail, MR signal characteristics are usually nonspecific. Correlation with the patient's age and the tumor markers significantly improves diagnostic accuracy.

Tumors of the pineal region are rare and relatively few centers around the world have published substantial numbers of carefully studied cases. This review gives a historical account of our understanding of the normal pineal and the evolution of the classification of tumors and other mass lesions of the pineal region in human beings. Based on our experience over the past 30 years, a working classification is proposed and recent advances in the neuropathology of these lesions are discussed.