Germ Cell Tumors of the Central Nervous System


Germinoma

Definition

  • Radiosensitive neoplasm of young individuals arising in the pineal or suprasellar region with histopathologic and immunohistochemical features similar to testicular seminoma and ovarian dysgerminoma

Clinical Features

Epidemiology

  • Germinomas account for about 3% of pediatric intracranial tumors

  • 65% of all CNS germ cell tumors

  • Twice as common in East Asia and Japan as in the United States

  • Male predominance

Presentation

  • Males in late teens or early 20s in the pineal gland region—most common

  • Headaches caused by progressive hydrocephalus and intracranial hypertension from pineal region tumors obstructing the cerebral aqueduct

  • Parinaud syndrome (paralysis of upward gaze and convergence) may be seen with tumors that compress or invade the tectal plate

  • Suprasellar region less common: symptoms include visual field defects, diabetes insipidus, pituitary failure, and delayed growth and sexual maturation (tumors that disrupt the hypothalamic/pituitary axis)

Prognosis and Treatment

  • Germinomas are radiosensitive: 10-year survival rate of 85% or greater

  • Prognosis not as good with mixed germ cell tumors

Imaging Characteristics

  • Germinomas are hyperdense, enhancing lesions of the pineal or suprasellar region

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