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Teratoma

Last Updated: March 27, 2020

Open Table of Contents: Teratoma

Figure 1: This complex pineal-region teratoma demonstrates areas of solid and cystic change and calcification on CT (top left) and areas of hyperintense fat signal intensity on the T1-weighted MR image (top right). Heterogeneous enhancement is a hallmark finding that illustrates the complexity of teratomas on T1 after contrast administration (bottom).

Figure 1: This complex pineal-region teratoma demonstrates areas of solid and cystic change and calcification on CT (top left) and areas of hyperintense fat signal intensity on the T1-weighted MR image (top right). Heterogeneous enhancement is a hallmark finding that illustrates the complexity of teratomas on T1 after contrast administration (bottom).

Basic Description

  • Midline intracranial tumor arising from multipotential germ cells

Pathology

  • Contains tissue from all three germ cell types: ectoderm, endoderm, and mesoderm
    • Fat, calcification, teeth, soft tissue, sebaceum, and cysts
  • Three types
    • Mature: well-differentiated, WHO grade 1, often with cystic tumor component
    • Immature: intermediate differentiation
    • Malignant: malignant degeneration of immature teratoma, may contain somatic tumors

Clinical Features

  • 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%)

Imaging Features

  • General
    • 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)
  • CT
    • 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
  • MRI
    • 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

Imaging Recommendations

  • 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

DOI: https://doi.org/10.18791/nsatlas.v1.03.01.40

References

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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