Common primary pineal region tumors include germ cell tumors: germinomas (most common), choriocarcinomas, yolk-sac tumors, and 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, ptosis, and loss of pupillary light and accommodation reflexes by compressing the pretectum rostral to the superior colliculi (Parinaud's syndrome). These tumors may cause precocious puberty, especially in boys, probably because the hypothalamus is compressed.
CSF β-human chorionic gonadotropin or α-fetoprotein may be elevated, depending on the tumor type. Elevated levels suggest the diagnosis; levels may be measured to monitor response to treatment.
Prognosis and treatment 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.
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