History

Signs and symptoms associated with pineal tumors are typically secondary to compression of the midbrain, with associated neurologic dysfunction.^{[46, 10]}Further, large growth may lead to obstruction of CSF structures, specifically the aqueduct of Sylvius, leading to hydrocephalus and signs and symptoms of increased intracranial pressure (ICP), such as headaches and lethargy.^[10] If left untreated, hydrocephalus may lead to obtundation and death. Compression of the superior colliculi may lead to gaze palsy and Parinaud syndrome. The rapidity of onset of signs and symptoms depends on the speed of tumor growth.

Children with pineal region tumors can present with endocrine dysfunction. Pseudoprecocious puberty is the most common endocrine disturbance. However, the mechanism by which pineal tumors causes precocious puberty remains unclear. Hypotheses include production of gonadotropins by the tumor, and invasion of the median eminence with subsequent loss of inhibition of sexual or gonadotrophic function.^[10]Diabetes insipidus and hypogonadism have also been observed.^[47]

Physical Examination

Common signs associated with pineal tumors include the following:

Ataxia
Papilledema
Loss of upward gaze
Tremor
Altered pupillary reflexes
Hyperactive deep tendon reflexes

As mentioned previously, compression of the superior colliculi may result in Parinaud syndrome, an ocular gaze disorder. This syndrome is seen in up to 75% of patients with pineal tumors.^[48]Further compression of the periaqueductal gray region may cause mydriasis, convergence spasm, pupillary inequality, and convergence or refractory nystagmus. Impairment of downward gaze becomes more pronounced with tumors involving the ventral midbrain. Patients also can present with motor impairment such as ataxia and dysmetria resulting from compromise of cerebellar efferent fibers within the superior cerebellar peduncle.

Differential Diagnoses

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Media Gallery

Gadolinium-enhanced MRI of a 33-year-old woman who presented with visual loss, amenorrhea, and diabetes insipidus. MRI shows germinomatous invasion of the pineal gland (large arrowhead), optic chiasm (long arrow), pituitary stalk (small arrowhead), and the floor of the third ventricle (short arrow).
Noncontrast MRI of a pineocytoma in a 40-year-old man presenting with acute hydrocephalus. At surgery, the high signal area (arrow) turned out to be acute hemorrhage.
MRI of a 21-year-old man with a germinoma in the pineal region. This T1-weighted noncontrast sagittal scan shows isointense tumor, which has obstructed the aqueduct of Sylvius (arrow) to cause hydrocephalus.
MRI of a 21-year-old man with a germinoma in the pineal region. This T2-weighted noncontrast axial scan shows the tumor as hyperintense to brain matter but hypointense to cerebrospinal fluid (CSF).
MRI of a 21-year-old man with a germinoma in the pineal region. Homogenous gadolinium enhancement of the tumor is shown on this T1-weighted contrast-enhancing sagittal scan.
Sagittal MRI of a heterogeneous mixed germ cell tumor of the pineal region in a 21-year-old man who presented with hydrocephalus. After pathologic examination following complete surgical resection, the tumor was found to have multiple components, including endodermal sinus tumor, embryonal cell carcinoma, immature teratoma, and mature teratoma.
Gross tissue specimens were obtained from a 21-year-old man who presented with hydrocephalus. After pathologic examination following complete surgical resection, the tumor was found to have multiple components, including endodermal sinus tumor, embryonal cell carcinoma, immature teratoma, and mature teratoma. Gross tissue specimens reflect heterogeneity of various germ cell components.
T1-weighted contrast-enhancing sagittal MRI from a 41-year-old man with a pineocytoma. The tumor enhances homogeneously with gadolinium, except for a cystic portion.
Micrograph from a mature teratoma of the pineal region that consists of well-differentiated tissue from all 3 germinal layers. This image demonstrates nonkeratinizing squamous cell epithelium alternating with areas of ciliated columnar epithelium.
This micrograph shows osteoid bone with surrounding periosteal tissue and mesenchymal stroma occurring within a mature teratoma of the pineal region.
This micrograph features cartilaginous tissue observed within a mature teratoma of the pineal region.
Immature teratoma of the pineal region with highly cellular primitive elements resembling fetal neural tube structure.
Endodermal sinus tumor with a characteristic Schiller-Duval body.
Pineoblastoma composed of highly cellular, poorly differentiated cells that form patternless sheets.
Pineocytoma consisting of benign, well-differentiated cells forming rosettes.
MRI of a 44-year-old woman 10 years after resection of a mixed pineal cell tumor. The tumor has recurred in the pineal region (arrow) and has seeded the fourth ventricle (arrowheads).
The right side of this image shows 3 operative approaches to the pineal region. Appropriate patient positioning for each approach is on the left. Number 1 is the supracerebellar-infratentorial approach, number 2 is the occipital-transtentorial approach, and number 3 is the parietal-interhemispheric approach.
The left drawing is a sagittal view of a patient with a pineal region tumor. The right drawing shows a sagittal view of the supracerebellar/infratentorial approach to the pineal region.
Biopsy specimen from an intracranial germ cell tumor. arge tumor cells with large nuclei; prominent nucleoli; and abundant, clear cytoplasm (rich in glycogen) are noted among reactive inflammatory cells.
View during a infratentorial supracerebellar approach from the operative microscope. Courtesy of Elsevier [Kennedy BC, Bruce JN. Surgical approaches to the pineal region. Neurosurg Clin N Am. 2011 Jul;22(3):367-80. PMID: 21801985.].
Pre and postoperative MRI from a patient with a pineal tumor resected via supracerebellar infratentorial approach. Courtesy of Elsevier [Kennedy BC, Bruce JN. Surgical approaches to the pineal region. Neurosurg Clin N Am. 2011 Jul;22(3):367-80. PMID: 21801985.].
Demographics and imaging findings of pineal tumors.
Markers of pineal tumors.
Management of pineal tumor.

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Author

Jeffrey N Bruce, MD Edgar M Housepian Professor of Neurological Surgery Research, Vice-Chairman and Professor of Neurological Surgery, Director of Columbia Brain Tumor Center, Director of Bartoli Brain Tumor Laboratory, Department of Neurosurgery, Columbia University Vagelos College of Physicians and Surgeons

Jeffrey N Bruce, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, American Society of Clinical Oncology, Congress of Neurological Surgeons, New York Academy of Sciences, North American Skull Base Society, Pituitary Society, Society for Neuro-Oncology, Society of Neurological Surgeons

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Theracle, Inc. <br/>Received grant/research funds from NIH for other.

Coauthor(s)

Colin P Sperring, BS MD Candidate, Columbia University Vagelos College of Physicians and Surgeonsw

Disclosure: Nothing to disclose.

Michael G Argenziano, BA MD Candidate, Columbia University Vagelos College of Physicians and Surgeons

Disclosure: Nothing to disclose.

Nina Teresa Yoh, MD Resident Physician, Department of Neurological Surgery, Columbia University Irving Medical Center, New York-Presbyterian Hospital

Nina Teresa Yoh, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, Congress of Neurological Surgeons, Gold Humanism Honor Society, Neurocritical Care Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Brian H Kopell, MD Associate Professor, Department of Neurosurgery, Icahn School of Medicine at Mount Sinai

Brian H Kopell, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, American Society for Stereotactic and Functional Neurosurgery, Congress of Neurological Surgeons, International Parkinson and Movement Disorder Society, North American Neuromodulation Society

Disclosure: Received consulting fee from Medtronic for consulting; Received consulting fee from Abbott Neuromodulation for consulting; Received Consulting Fee from ClearPoint Neuro.

Additional Contributors

Michael G Nosko, MD, PhD Associate Professor of Surgery, Chief, Division of Neurosurgery, Medical Director, Neuroscience Unit, Medical Director, Neurosurgical Intensive Care Unit, Director, Neurovascular Surgery, Rutgers Robert Wood Johnson Medical School

Michael G Nosko, MD, PhD is a member of the following medical societies: Academy of Medicine of New Jersey, Congress of Neurological Surgeons, Canadian Neurological Sciences Federation, Alpha Omega Alpha, American Association of Neurological Surgeons, American College of Surgeons, American Heart Association, American Medical Association, New York Academy of Sciences, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Richard CE Anderson, MD Assistant Professor of Neurosurgery and Pediatric Neurosurgery, Columbia University Medical Center, Columbia University College of Physicians and Surgeons; Director, Pediatric Neurosurgery, St Joseph's Children's Hospital

Richard CE Anderson, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, Congress of Neurological Surgeons, American Association of Neurological Surgeons, Phi Beta Kappa

Disclosure: Nothing to disclose.

Alfred T Ogden, MD Assistant Professor, Department of Neurological Surgery, Columbia University Medical Center

Alfred T Ogden, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

Benjamin C Kennedy, MD Assistant Professor of Neurosurgery, Perelman School of Medicine at the University of Pennsylvania; Director of Epilepsy and Functional Neurosurgery, The Children’s Hospital of Philadelphia

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Andrew T Parsa, MD, PhD, and Chris E Mandigo, MD.

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