Teratoma of the central nervous system

Teratoma of the central nervous system (CNS teratoma) is a rare germ cell tumor that arises within the brain or spinal cord. Teratomas are composed of tissues derived from all three embryonic germ layers — ectoderm, mesoderm, and endoderm — and may contain differentiated structures such as hair, teeth, bone, cartilage, and glandular tissue. CNS teratomas are classified as mature (benign), immature (potentially malignant), or teratoma with malignant transformation. They most commonly occur in the midline structures of the brain, particularly the pineal region and the suprasellar area, though they can also arise in the posterior fossa or spinal cord. Symptoms depend on the tumor's location and size. Pineal region teratomas frequently cause obstructive hydrocephalus, leading to headaches, nausea, vomiting, and visual disturbances such as Parinaud syndrome (impaired upward gaze). Suprasellar teratomas may present with endocrine dysfunction, including diabetes insipidus, growth hormone deficiency, and precocious or delayed puberty. Other symptoms can include seizures, focal neurological deficits, and signs of increased intracranial pressure. In neonates and infants, CNS teratomas may present with macrocephaly and developmental delay. CNS teratomas occur predominantly in children and young adults, with a male predominance, particularly for pineal region tumors. Diagnosis involves neuroimaging (MRI/CT), measurement of serum and cerebrospinal fluid tumor markers (alpha-fetoprotein and beta-hCG), and histopathological examination. Treatment typically involves surgical resection, which is often curative for mature teratomas. Immature teratomas or those with malignant components may require adjuvant chemotherapy and/or radiotherapy. The prognosis for mature teratomas is generally favorable following complete surgical excision, while immature teratomas and those with malignant transformation carry a more guarded prognosis.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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