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PINEAL TUMORS

The Pineal Gland, a small structure lying in the very center of the Brain, is rather peculiar since its precise function has never been fully understood. It has a role in the production of "Melatonin", a hormone, the lack of which has been associated with "jet-lag". While rare, Pineal Gland Tumors and Pineal Region Tumors account for less than 1.5% of all human Brain tumors.

Figure 1: Diagram of Brain (Sagittal View) illustrating the location of the Pineal Gland, the Superior & Inferior Colliculus and the Aqueduct of Sylvius (Cerebral Aqueduct)

SYMPTOMS

The symptoms caused by these tumors, result from tumor invasion or compression of the various nearby anatomical structures and are influenced by the different pathological entities that comprise the group called "Pineal Region Tumors".
  1. Obstructive Hydrocephalus.

    Because of its critical, central, anatomical location, these tumors can obstruct normal Cerebrospinal Fluid (CSF) pathways by compressing the Cerebral Aqueduct (or Aqueduct of Sylvius), a small channel connecting the Third to the Fourth Ventricle. This results in a condition known as "Hydrocephalus" ("water on the Brain"). Since CSF is constantly being produced, any obstruction to its flow pattern or rate of reabsorbtion will result in a "ballooning" of the Ventricular System and increased intracranial pressure. The patient will experience headaches, vomiting, lethargy and altered mental status.

  2. Posterior Mid-Brain Compression

    Compression of the adjacent Brain structure, the Superior Colliculus, may cause serious visual disturbance, the most common of which is Paralysis of Vertical Gaze. Compression injury to this mid-Brain area can result in a more extensive Neuro-ophthalmological problem known as Parinaud's Syndrome (Paralysis of upward gaze; pseudo-Argyll Robertson pupils; Convergence-Retraction Nystagmus and Eyelid Retraction also known as "Collier's Sign".

  3. Invasion of Brain (Thalamus and/or Hypothalamus)

    Direct invasion of the Thalamus can cause loss of sensation to half the body, weakness of half the body, and intermittent pain. Invasion of the vital centers of the Hypothalamus can cause disturbances in body temperature and water regulation, sleepiness, and weight gain. Other symptoms include memory problems, poor coordination and tremors.

DIAGNOSIS

The diagnosis of a Pineal Region Tumor is best established by MRI scanning since it has better resolution of Brain and tumor compared to CT scan.

Blood Tests for "Tumor Markers"

There are certain helpful additional investigations that can be done once a Pineal Region Tumor is identified. Blood tests for "tumor markers" can be used to identify certain types of tumors. The most useful and specific markers for Pineal Region Tumors are beta human chorionic gonadotropin (▀-HCG) and the alpha-fetoprotein (AFP). ▀-HCG is produced by Choriocarcinoma and Geminomas and can be detected in the blood and Cerebrospinal Fluid. Elevated AFP suggests a Malignant Germ Cell Tumor, most often Edodermal Sinus Tumor. Elevation of both "markers" can be found in Embryonal Cell Carcinomas, Malignant Teratomas, or Mixed Germ Cell Tumors.

PINEAL TUMOR PATHOLOGY

  1. Pineal Parenchymal Tumors (Pineoblastoma and Pineocytoma)

    Tumors of the Pineal Parenchymal Cells are the second largest group of Pineal Region Tumors, accounting for 30% of all tumors in this location. There are two types of tumors in this group, Pineoblastoma and Pineocytoma, differing only by their histological appearance, level of differentiation and degree of malignancy.

  2. Germ Cell Tumors (Pure Germinoma, Embryonal Cell Carcinoma, Endodermal Sinus Tumor, Choriocarcinoma, Immature Teratoma and Mature Teratoma)

    Germ Cell Tumors account for more than half of all Pineal Region Tumors. Pure Germinomas account for 65% of all intracranial Germ Cell Tumors. Germinomas are poorly circumscribed and often "seed" the Ventricular system. The Embryonal Cell Carcinoma, the least frequently reported intracranial Germ Cell Tumor, is usually highly malignant as are the rare and highly invasive Endodermal Sinus Tumors. Teratomas can be either "immature" or "mature" and represent the expression of embryonic differentiation in Germ Cell Tumors. These well circumscribed tumors are usually round or lobulated and multicystic. They produce symptoms by compressing the surrounding structures.

  3. Tumors of Supportive Tissues & Adjacent Structures (Astrocytomas, Ependymomas, Meningiomas, Hemangiopericytomas)

    Each of these tumors produce symptoms related to their size (mass) by compressing surrounding anatomical structures (as described above) and/or by virtue of their capacity to invade the surrounding brain substance ( as can occur with Astrocytomas and Ependymomas).

  4. Metastatic Tumors

    While quite rare, metastatic malignancies can affect this region producing symptoms consequent to compression, as well as invasion.

  5. Nonneoplastic Mass Lesions (Pineal Cysts, Arachnoid Cysts, Cysticercus Cysts, Vein of Galen Malformation.

Figure 2A (Left): MRI (Gadolinium Enhanced-Sagittal View) 39 year old Female with a Pineal Cyst. Serial MRI Scans have demonstrated slow, progressive increased size of the Pineal Cyst which is filled with Cerebrospinal Fluid (CSF) (Up-curved Arrow) over a period of several years. This view demonstrates the relationship of the Vein of Galen which lies immediately below the Splenium of the Corpus Callosum (Vertical Arrow); the Straight Sinus (Down-curved Arrow) indicates the plane of the Tentorium; the Cerebellum lies immediately below the Tentorium.

Figure 2B (Center): MRI (Gadolinium Enhanced-Coronal View-Same patient) The Pineal Cyst (Curved Arrow) has not caused Hydrocephalus as yet.

Figure 2C: MRI (Gadolinium Enhanced-Transaxial View-Same patient) The Pineal Cyst (Curved Arrow) lies directly posterior to the "slit-like" Third Ventricle (Horizontal Arrow).

All of these pathological entities produce symptoms by compressing surrounding anatomical structures. All are amenable to surgical management.

TREATMENT

Historical Perspective

The surgical management of Pineal & Pineal Region Tumors lagged well behind surgical treatment of other Brain problems, because of the central location within the Cranial Cavity and the vulnerability of the many vital Brain centers in this location. For decades the Pineal Region was considered to be "No Man's Land".

One of the surgeons who founded Neurosurgical Consultants was among the first Neurosurgeons to employ Microneurosurgical techniques to successfully remove various types of tumors and pathological structures in the Pineal Region. Dr. Martin Lazar first reported the resection of a Third Ventricular Ependymoma, Pineal Region Meningioma as well as Tumors of the Anterior Superior Cerebellum at the 1973 Annual Meeting of the American Association of Neurological Surgeons titled: "To Galen and Back: Surgical Excision of Pineal Region Tumors". At that time, and in subsequent scientific presentation, he described a Microneurosurgical Occipital Transtentorial direct approach, which was adapted from a concept introduced in an earlier neurosurgical era by Dr. James L. Poppen. Subsequent publications by Dr. Lazar established this technique (See Figure 2) and the possibility for Neurosurgeons to access this region successfully. Other Neurosurgical authors have subsequently described their experiences with this and other surgical approaches to this region.

For further information, please consult Direct Surgical Management of Masses in the Region of the Vein of Galen in Surgical Neurology 2:17-21,1974 (Lazar, M.L., and Clark, W.K.); Thrombosed Aneurysm of the Vein of Galen in Neurosurgery 7:274-278, 1980 (Six, E.G., Cowley, A.R., Kelly, D.L. and Laster, D.W. with Critique and Comments by M.L. Lazar); Pineal Region Meningioma: Report of Two cases and Literature Review in Neurosurgery 2:369-375, 1983 (Piatti, J.H. and Campbell, G.A with Critique and Comments by M.L. Lazar)

Figure 3: Original Surgical Diagrams reproduced from Direct Surgical Management of Masses in the Region of the Vein of Galen in Surgical Neurology 2:17-21, 1974 (Lazar, M.L., and Clark, W.K.)

A-C: A Right Occipital Craniotomy with bone removal to expose the "Confluens" of Sinuses, Transverse Sinus and superior Sagittal Sinus following which the Dura is opened.

D: The Tentorium and Falx are directly exposed. The Tentorium will be opened along the indicated line 1 centimeter Lateral to the Straight Sinus. Care must be taken to preserve the Inferior Cerebral Veins.

E: After opening the Tentorium, the Vein of Galen; Basal Vein of Rosenthal; Splenium of the Corpus Callosum; Posterior 3rd Ventricle; Anterior Superior Vermis of the Cerebellum (including the Anterior Quadrangular Lobe, Central Lobe & Lingula) and the "Tumor" are all accessible through this surgical approach.

Surgery

Prior to the introduction of Microneurosurgical techniques and limited access procedures, the TREATMENT of ALL Pineal Region Tumors was largely confined to "Shunting of the Ventricles" to control the Hydrocephalus followed by Radiation Therapy. Mortality rates were high with many of the tumors not responding to Radiation since they were actually BENIGN Lesions made worse by the treatment.

Modern treatment of Pineal Region Tumors involves:

  • Control of Hydrocephalus
  • COMPLETE SURGICAL REMOVAL of benign encapsulated tumors of the Pineal Region
  • Histological identification of nonresectable followed by Radiation and Chemotherapy
Stereotactic biopsy (which has low morbidity and mortality rates and gives satisfactory biopsy samples) of Pineal Region Tumors is an alternative to direct surgical exposure and may be preferable for certain types of tumors that are clearly not surgically resectable and can be treated with other techniques.

Endoscopic surgery can also be accomplished and offers some advantage over Stereotactic biopsies, in that the Neurosurgeon can actually inspect the lesion and surrounding tissue prior to taking samples.

Stereotactic Image Guided Endoscopic Techniques (which offers increased accuracy) are now available in some centers.

Radiation Therapy

More than 70% of Pineal Region Tumors are responsive to Radiation Therapy within 3 to 6 months of treatment. Germinomas are particularly responsive to Radio-therapy.

Combined Therapy

In many instances, Ventricular Shunting followed by Radiation Therapy, is the treatment of choice. However, this should almost always only FOLLOW BIOPSY CONFIRMATION of the Tumor. Chemotherapy

Chemotherapy treatment is effective for some Pineal Region Tumors with the choice of chemotherapeutic agents being dependent upon the type of tumor.



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This page last edited on 2/19

All content ©2016 by Neurosurgical Consultants, P.A.
Author, Martin L. Lazar, MD, FACS
All Rights Reserved. See Usage Notices.