These tumors begin in the brain in the pineal gland but can spread to the spinal cord. A biphasic tumor in posterior cranial fossa and the pineal. Lesions of the pineal region rsna publications online. Tumors of the pineal region are rare and can be challenging to differentiate by imaging. Principles of treatment of the pineal region tumors pdf. Tumors of the pineal region account for less than 2% of all intracranial neoplasms 1. Pineal region tumors are primary central nervous system cns tumors. Tumors of the pineal region are relatively rare neoplasms that comprise 1% or less of all intracranial neoplasms,14. The classification, presentation, and general approach to lesions involving the pineal gland will be presented here, focusing on the management of pineal parenchymal tumors. They happen most often to children and adults younger than 40. Pineal region tumors can spread to other areas in the cns through cerebrospinal fluid csf.
They can have different grades of differentiation, from well. When they become symptomatic, some form of management must be contemplated. Pineal region tumors arise from stem cells near the pineal gland. Papillary tumor of the pineal region ptpr was classified as a distinct entity by the world health organization in 2007. This region is located deep in the middle of the brain. While several reports of computed tomography ct of third ventricular neoplasms have included an occasional pineal tumor 2, 3, few have.
The tumor originated out of both astrocytes and pinealocytes, best to our knowledge, has not been described. Papillary tumors of the pineal region ptpr were first described by a. Jeffrey bruce esteemed panelist at another free cancercare teleconference dr. Most children are aged 1020 years at presentation, with the average age at presentation being years. The pineal gland, a small, pineconeshaped organ deep within the brain, is responsible for producing melatonin. This gland is a small structure deep within the brain. Lipomas of the pineal region are even rarer and are reported occasionally in the literature. Papillary tumors of the pineal region are located on the pineal gland which is located in the center of the brain. These tumors may cause problems by pressing against other parts of the brain. Pineal parenchymal tumors comprise a group of related tumors ranging from the relatively benign to the highly malignant. A pineal region tumor is an uncommon deepseated, heterogeneous group of mass lesions of the brain, and the management strategy of any types of these tumors remains controversial. The most common is a germinoma, which is a homogeneous mass with signal intensity and attenuation similar to those of gray matter.
Evaluation of pineal region tumors in children the ispn. P ineal region tumors account for less than 5% of brain tumors in adults and around 10% in children. Pineal parenchymal tumors radiology reference article. Neoplasm principles of treatment of the pineal region tumors alexander n. This is in distinction to tentorial meningiomas which depress the cerebral veins. A pineal tumor is a tumor of the pineal gland in your brain. Pineal region tumors are grouped in four grades and subtypes based on their characteristics.
Tumours in the pineal region is a rare entity and in general considered to have a poor outcome. Pineal parenchymal tumors 1427% of pineal region tumors term pinealomas is obsolete. Epidemiological characteristics vary on the basis of tumor type. The incidence of pineal region tumors is higher in children, in whom 38% of intracranial tumors occur in the pineal region. Biopsy of pineal region tumors is now feasible and is recommended for treatment planning. Incidence, presentation, and different types of pineal region tumors pineal region tumors comprise 2. Pineal region neoplasms occur more frequently in children, accounting for 3%8% of intracranial neoplasms in the pediatric population. Normal pineal region anatomy the pineal gland is usually approximately 8 mm long and 4 mm wide. These tumors represent about 1% of all brain tumors but account for 3% to 8% of the intracranial tumors that occur in children. Pineal region tumors are presently classified into 5 categories.
Results of irradiation of tumors in the region of the pineal body. Most pineal region masses are malignant germ cell neoplasms that occur in young male patients. The treatment for the pineal region tumors depends on tumor histology. The high frequency of mixed tumors in this region further contributes to diagnostic inaccuracies of the procedure. This gland secretes a substance called melatonin that affects your sleepwake cycles. Biphasic tumors of the central nervous system are rarely described and mainly consisted out of the glial and mesenchymal component. The mystique of the even nowadays poorly understood function of the pineal gland, the everlasting beauty of its anatomy, and the unbeatable elegance of the surgical approaches to this region have fascinated generations of neurosurgeons. The optimal treatment for patients with a pineal tumor depends upon the tumor subtype and may include surgical removal, radiation therapy andor chemotherapy. French 18thcentury scientist rene descartes thought that the pineal gland was the core of the soul, noting that it was the only unpaired organ in the body, and located in the center of the brain.
Papillary tumor of the pineal region ptpr was recently recognized as a neoplasm in the world health organization who 2007 classification, arising from specialized ependymocytes in the subcommissural organ, which is located in the pineal region. The clinical presentation of children with pineal region lesions is variable and is often related to mass effect on the. This is possibly because of the fact that they may give rise to clinical symptoms rather infrequently. Tumors in this region have diverse origins, including tumors arising from pineal parenchymal cells i. The authors believe that the preferred treatment for pineal region tumors in children requires definitive surgery with a histological diagnosis and that a conservative approach consisting of shunting and radiation therapy no longer seems to be appropriate. Teratomas are multilocular heterogeneous masses containing lipid areas. Nov 01, 2010 tumors of the pineal region are classified into those arising from the pineal parenchyma, germ cell neoplasms, metastasis, and lesions arising from adjacent structures eg, astrocytoma, meningioma. The pineal gland hangs on the brain behind its very center. Tumors of the pineal region account for pdf 2mb sizes 0 downloads 3 views.
The pineal gland is located on roof of the diencephalon. Ct of pineal region tumors american journal of neuroradiology. Epidemiology of pineal region tumors in children the. Gamma knife radiosurgery gkrs for pineal region tumors. Pineal region tumors weill cornell brain and spine center. Gcts, however nongerminomatous germ cell tumors nggcts tend to have more heterogenous imaging characteristics compared to germinomas. We studied a series of nine biopsyproven pineal gland tumors that demonstrated remarkable heterogeneity in both histopathologic and.
Pa can arise anywhere in the cns, although it most frequently occurs in the cerebellum. With the galenic venous system present in the close proximity of the pineal region, preoperative stereotactic angiography has been suggested as a prerequisite for a safe stereotactic biopsy of pineal region lesions. At least 17 different types of tumors may occur in this region, and many are benign. An overview of the current surgical options for pineal.
The management of germ cell tumors is histology dependent and different protocols are emerging for patients with pure germinomas and nongerminoma germ cell tumors. The pineal gland is a small organ located at the back of the base of the brain, near the top of the brainstem. Preenhancement germinomas revealed characteristically highdensity areas. Pineal region tumors are derived from cells located in and around the pineal gland. The gland consists of pineal parenchymal cells and glial cells that can form neoplasms. Pineal tumors arise in the region of the pineal gland. We analysed a series of patients with pineal region tumours and found them to have on an average a longer life expectancy and a lesser rate of recurrence as compared to patients with cerebral tumours outwith pineal region. This is the largest reported series of histologically verified pineal region tumors studied with ct. Nowadays, germinomas can be cured by radiotherapy and chemotherapy without surgical resection but the other pineal region. It is the purpose of this communication to present results of our experience in treating patients with these neoplasms.
These tumors tend to occur in young adults between 20 and 40 years old. Pineal region neoplasms can also arise from germ cells and adjacent structures. Tumors of the pineal region are rare, but they occur more frequently in children than in adults and are most likely to be detected during. Pineal tumors can happen at any age, but they tend to happen in children and young adults. The principle cell of the pineal gland is the pineal parenchymal cell or.
Mimics of nonneoplastic pineal region tumor include the following. Parinauds syndrome is a pathognomonic sign and is present in 5070% of patients with pineal region tumors 30. Parinauds syndrome includes upward gaze palsy with impaired convergence, nystagmus retractorius, eyelid retraction colliers sign, and lightnear dissociation pseudo argyllrobertson pupil, where pupils are dilated. We present a case of a brain tumor consisted out of pilocytic astrocytoma pa and pineocytoma as components situated in the pineal. Pdf on jan 1, 2019, nir shimony and others published pineal region tumors find, read and cite all the research you need on researchgate.
Germ cell tumors gcts, the most frequent tumor type found in the pineal region, are discussed elsewhere. Tumors of the pineal region are rare and their optimal management remains controversial. Pineal region tumors form in the pineal region of the brain. Principles of treatment of the pineal region tumors.
Pineal region tumors represent less than 1% of all primary brain tumors. Pineal region tumors arise in or around the pineal gland and are relatively uncommon. Pineal region masses include germ cell tumors gcts, pineal parenchymal cell tumors, glioma, meningioma, metastases, and nonneoplastic masses such as pineal cysts, lipoma, epidermoid, vascular malformations. The tumors were classified as germcell tumors, glial tumors, pineal parenchymal tumors, and meningiomas.
Papillary tumor of the pineal region pubmed central pmc. About 10% to 20% of the tumors, particularly pineoblastoma, have the potential to spread through the cerebrospinal fluid. Treatment of tumors in the pineal region remains one of the great intellectual and technical challenges to neurological surgeons. Protocol for management of patients with pineal region tumours v1. Germ cell tumors and pineal parenchymal neoplasms do not have pathognomonic imaging findings, but imaging in combination with laboratory evaluation helps. Neuroendocrine, immunohistochemical, and ultrastructural study of pineal region tumors.
Its responsible for producing several hormones, including melatonin, which regulates sleepwake cycles as well as some reproductive functions. There are multiple types of pineal gland tumors, most of which are not cancerous but can nonetheless cause extreme distress. In this editorial,we propose a simplified and stratified management scheme for tumors in this region. He thought that the gland controlled the movement of the various body humor. Intrinsic pineal tissue masses also tend to cause upward displacement of the internal cerebral veins. The sites of origin of germ cell tumors in the cns are unique, that is, extraaxial locations in proximity to the pineal gland and infundibulum. During a prior era when surgical approaches to the pineal region were associated with. Teratomas of the pineal region are more common in males and in child hoodearly adulthood, and are classified as extraaxial teratomas. Jeffrey bruce discusses cutting edge brain tumor treatment at aans 20 dr. Tumors of the pineal region account for tumors of the pineal region include pineal parenchymal neoplasms, germ cell neoplasms, and tumors arising from adjacent structures, including meningiomas, astrocytomas, and ependymomas 1. In fact, the pineal gland is the third eye of the brain. The computed tomographic ct features of pineal region tumors were analyzed in 60 histologically proven tumors. The pineal region is a heterogeneous area that includes the pineal gland and several parapineal structures.
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