Last Updated: March 27, 2020
- Midline intracranial tumor arising from multipotential germ cells
- Contains tissue from all three germ cell types: ectoderm, endoderm, and mesoderm
- Fat, calcification, teeth, soft tissue, sebaceum, and cysts
- Mature: well-differentiated, WHO grade 1, often with cystic tumor component
- Immature: intermediate differentiation
- Malignant: malignant degeneration of immature teratoma, may contain somatic tumors
- Arises during fetal development due to aberrant formation of the primitive streak
- Mean patient age at diagnosis 15 years; may be detected on fetal ultrasound
- Male gender predilection (4:1)
- Laboratory findings: increased serum carcinoembryonic antigen (CEA) ± α-fetoprotein
- Common presenting signs/symptoms: macrocephaly/hydrocephalus, Parinaud’s syndrome
- Treatment: surgical resection
- Prognosis: majority are lethal in utero or during 1st week of life; patients with malignant teratomas have poor 5-year survival (<20%)
- Midline intracranial mass
- Pineal region, sellar/suprasellar, basal ganglia, and spine
- Mass effect on tectum, optic chiasm, and hypothalamus common
- Contains calcifications, solid and fluid/cystic components and fat
- Size variable, may be large in neonates (holocranial mass)
- Midline intracranial mass
- Heterogeneous and containing very low-density fat, hyperdense calcification (teeth), intermediate density soft tissue, and low-density cysts
- Soft tissue may enhance on contrast-enhanced CT
- T1WI: Heterogeneous hyperintensity due to fatty components and calcification
- T2WI: Iso- to hyperintense soft tissue, cysts/fluid; variable hyperintense peritumoral edema
- T2*GRE: Hypointense signal blooming in areas of calcification
- DWI: Diffusion restriction due to hypercellular solid components
- T1WI+C: Soft tissue components enhance, nonenhancing fatty or calcified portions
- MRI without and with IV contrast including fat-suppressed sequences; CT to detect calcification
For more information, please see the corresponding chapter in Radiopaedia.
Contributor: Rachel Seltman, MD
Kralik SF, et al. Diffusion imaging for tumor grading of supratentorial brain tumors in the first year of life. AJNR Am J Neuroradiol. 2014;35:815-823.
Liang L, et al. MRI of intracranial germ-cell tumours. Neuroradiology. 2002;44:382-388.
Liu Z, et al. Imaging characteristics of primary intracranial teratoma. Acta Radiol. 2014;55:874-881.
Noudel R, et al. Intracranial teratomas in children: the role and timing of surgical removal. J Neurosurg Pediatr. 2008;2:331-338.
Osborn AG, Salzman KL, Jhaveri MD. Diagnostic Imaging (3rd ed). Philadelphia, PA: Elsevier, 2016.
Sawamura Y. WHO histological classification of tumors of the central nervous system: germ cell tumors (WHO, 1993). Intracranial Germ Cell Tumors. 1998:3-4.
Zygourakis CC, et al. Management of central nervous system teratoma. J Clin Neurosci. 2015;22:98-104.
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