Most pineal region tumors are germ cell tumors.
Common primary pineal region tumors include germ cell tumors:
Germinomas (most common)
Choriocarcinomas
Yolk-sac tumors
Teratomas
Less common primary pineal tumors include pineocytomas and the rare malignant pineoblastomas.
Pineal region tumors tend to occur during childhood but can occur at any age.
These tumors may increase intracranial pressure by compressing the aqueduct of Sylvius. They may also cause paresis of upward gaze and loss of pupillary light and accommodation reflexes by compressing the pretectum rostral to the superior colliculi (Parinaud syndrome). These tumors may cause precocious puberty, especially in boys, as the hypothalamus is compressed ventrally.
Diagnosis of pineal region tumors is by MRI and tissue biopsy. Lumbar puncture is usually contraindicated because herniation is a risk.
Prognosis for and treatment of pineal region tumors depend on tumor histology. Radiation therapy, chemotherapy, radiosurgery, and surgery are used alone or in combination. Germinomas are very sensitive to radiation therapy and are often cured.
(See also Overview of Intracranial Tumors.)
